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1631

GENITOURINARY IMAGING
Radiologic-Pathologic Correla-
tion of Primary Retroperitoneal
Neoplasms
Khalid Al-Dasuqi, MD
Lina Irshaid, MD Primary neoplasms that originate in the soft tissue of the retroperi-
Mahan Mathur, MD toneum are rare, but they are often malignant and can grow to a
substantial size at clinical presentation. Although imaging findings
Abbreviations: ASPS = alveolar soft-part sar- can be nonspecific, knowledge of some distinguishing imaging
coma, FDG = fluorodeoxyglucose, MPNST = features and clinical and epidemiologic considerations can aid the
malignant peripheral nerve sheath tumor, RMS =
rhabdomyosarcoma, WHO = World Health
radiologist in narrowing the differential diagnosis or, in some cases,
Organization providing a specific diagnosis. Some of the more important findings
RadioGraphics 2020; 40:1631–1657
at cross-sectional imaging that can facilitate this assessment include
tumor size and location (eg, presacral, paravertebral, or in the organ
https://doi.org/10.1148/rg.2020200015
of Zuckerkandl); tissue composition (eg, fat, fibrous tissue, cystic
Content Codes: components, or myxoid matrix), including assessment of the signal
From the Department of Radiology and Biomed- intensity of the lesion at T2-weighted MRI; degree of vasculariza-
ical Imaging (K.A.D., M.M.) and Department
of Pathology (L.I.),Yale School of Medicine, 333
tion; and relationship to adjacent structures (ie, invasion of vascular
Cedar St, PO Box 208042, Room TE-2, New structures such as the inferior vena cava). This assessment is further
Haven, CT 06520. Recipient of a Certificate of enhanced by an understanding of the gross and microscopic histo-
Merit award for an education exhibit at the 2019
RSNA Annual Meeting. Received February 14, logic appearances of these neoplasms. Primary solid retroperitoneal
2020; revision requested March 19 and received neoplasms are grouped and presented according to their primary
April 16; accepted April 17. For this journal-
based SA-CME activity, the authors, editor, and
soft-tissue element (eg, adipocytic, smooth muscle, fibroblastic,
reviewers have disclosed no relevant relationships. neurogenic, or skeletal muscle). Brief discussions of primary cystic
Address correspondence to M.M. (e-mail: retroperitoneal neoplasms and some miscellaneous neoplasms that
[email protected]).
do not fit into the aforementioned categories follow. An imaging
©
RSNA, 2020 algorithm to ensure a systematic approach to diagnosis of these le-
sions is also provided, which will allow the radiologist to provide
SA-CME LEARNING OBJECTIVES more accurate interpretations for their referring providers, thus en-
After completing this journal-based SA-CME suring optimal patient treatment.
activity, participants will be able to:
„ Describe the fascial planes and spaces
Online DICOM image stacks and supplemental material are available
of the retroperitoneal compartments. for this article.
„ Characterize solid and cystic retroperi- ©
RSNA, 2020 • radiographics.rsna.org
toneal neoplasms based on their imaging
features.
„ Correlate imaging features with gross
and microscopic histologic findings.
Introduction
See rsna.org/learning-center-rg.
Primary malignant tumors that originate in the soft tissue of the
retroperitoneum (ie, outside the solid retroperitoneal organs such
as the kidneys, adrenal glands, and portions of the small and large
bowel) are rare. In one review (1) of 25 647 malignant neoplasms
from a tumor registry, retroperitoneal tumors accounted for 0.16%
of all tumors. Nevertheless, 79%-90% of all retroperitoneal tumors
are malignant, and they are associated with relatively high mortal-
ity rates, owing partly to the nonspecific symptoms, or lack thereof,
in patients at presentation (2). However, these numbers are derived
from observational studies that date back to 1950, when all cases of
retroperitoneal lymphoma were historically considered to arise from
the retroperitoneal soft tissue. Today, our understanding of the origin
An earlier incorrect version of of retroperitoneal lymphoma is more nuanced, so the actual preva-
this article appeared in print. lence of primary malignant retroperitoneal tumors may be lower
The online version is correct. than previously reported.
1632 October Special Issue 2020 radiographics.rsna.org

by different fascia. The most anterior compart-

TEACHING POINTS ment, known as the anterior pararenal space, is
„ The presence of incomplete fat suppression at MRI, thick or
bounded anteriorly by the posterior layer of the
irregular capsules or septa, internal nodular enhancement, or
necrosis in a retroperitoneal fat-containing mass should raise
parietal peritoneum, posteriorly by the anterior
suspicion for a lipomatous sarcoma, and biopsy and/or surgi- renal fascia (also known as the Gerota fascia),
cal resection are warranted in those cases. and laterally by the lateroconal fascia. The most
„ Myxoid liposarcomas are often predominantly nonfatty, with posterior compartment, known as the posterior
a cystic appearance at CT and MRI due to the extracellular pararenal space, is bounded anteriorly by the
myxoid matrix (hypoattenuating compared with muscle at posterior renal fascia (also known as the Zucker-
CT, high signal intensity at T2-weighted MRI, and low signal
kandl fascia) and posteriorly by the transversalis
intensity at T1-weighted MRI). However, unlike cystic lesions,
myxoid liposarcomas demonstrate varying amounts of con- fascia. Medially, this space is limited by the
trast material enhancement. While they generally contain a fusion of the posterior renal and transversalis
paucity of fat, the presence of a small amount of fat in lacy fascia with the muscular fascia, while anterolat-
septa or nodular components is pathognomonic for myxoid erally, this is contiguous with the properitoneal
liposarcomas.
fat. The perirenal space is bounded anteriorly by
„ A large non–fat-containing retroperitoneal mass with involve-
the anterior renal fascia and posteriorly by the
ment of a contiguous vessel and varying internal necrosis
should raise the possibility of a leiomyosarcoma.
posterior renal fascia (Fig 1a) (3).
„ At T2-weighted MRI, diffuse high signal intensity (a “light-
In addition to delineating the retroperitoneal
bulb” appearance) has often been described in paraganglio- compartments, the aforementioned fascial layers
mas. However, a more complex appearance is frequently ob- have been shown to represent potentially expansile
served because of the signal heterogeneity that is associated planes that allow communication of neoplastic and
with hemorrhage and/or necrosis. nonneoplastic processes between these compart-
„ At CT, lymphatic malformations often appear as a uniformly ments (4). The retromesenteric plane corresponds
cystic mass and exhibit attenuation that is near that of wa- to the anterior renal fascia, the retrorenal plane
ter. The walls and/or septa are rarely perceptible, and septal
enhancement is difficult to appreciate at CT. They classically
corresponds to the posterior renal fascia, and the
can involve more than one retroperitoneal compartment and lateroconal plane corresponds to the lateroconal
can appear as an elongated and insinuating mass, which is an fascia (Fig 1a) (5). Inferiorly, these form the com-
important imaging clue. bined interfascial plane, which courses anteriorly
to the psoas muscle, allowing communication to
the pelvis (Fig 1b) (5). Some authors (6) have
CT and MRI are the primary imaging mo- proposed an additional interfascial plane, termed
dalities for the diagnosis and follow-up of ret- the subfascial plane, which is located between the
roperitoneal lesions. Determining the anatomic posterior pararenal space and the transversalis fas-
extent and key imaging features of primary cia. Perinephric bridging septa serve as potential
retroperitoneal neoplasms is crucial for providing channels that allow disease processes to commu-
an accurate diagnosis and is important in select- nicate from the perirenal space to the interfascial
ing the appropriate patient treatment. This can be planes (Fig 1c) (7).
facilitated by an understanding of the gross and
microscopic histologic features of the different Localization of Retroperitoneal
lesions. Histologic subtyping also is important in Tumors
prognostication and determination of the appro- Before examining specific imaging features of the
priate treatment regimen in patients with primary primary retroperitoneal neoplasm, the radiologist
retroperitoneal neoplasms. must ensure that the mass arises in the retroperi-
After we review the abdominal retroperito- toneal soft tissues as opposed to the peritoneum
neal anatomy and discuss how to recognize the or an organ that resides in the retroperitoneum.
retroperitoneal origin of a mass, we elaborate on This is critically important because a mass con-
several types of common and uncommon pri- taining macroscopic fat arising in the peritoneal
mary retroperitoneal neoplasms, highlighting key cavity seldom is a liposarcoma, while a fat-con-
epidemiologic, clinical, imaging, and histologic taining mass arising from a retroperitoneal organ
features that allow the radiologist to provide an (such as the kidneys) often is a benign finding.
accurate diagnosis. A summary of these fea- First, the radiologist can start by assessing how
tures can be found in the Table. An algorithmic normal structures are displaced by the identi-
approach to evaluating these lesions at MRI is fied mass (8). This is especially useful because
presented online. (Fig E1). most retroperitoneal tumors are large at patient
presentation, and displacement of adjacent struc-
Abdominal Retroperitoneal Anatomy tures is common. Masses displacing the retroperi-
The retroperitoneum is divided into three com- toneal organs anteriorly (eg, the kidneys, ascend-
partments, the borders of which are delineated ing colon, descending colon, and pancreas) are
RG • Volume 40 Number 6 Al-Dasuqi et al 1633

Key Imaging, Epidemiologic, Clinical, and Histologic Features of Primary Retroperitoneal Neoplasms

Key Epidemiologic, Clinical,

Category of Tumor Key Radiologic Features and Histologic Features
Adipocytic
Lipoma Fat-containing mass with no/trace inter- Rare in the retroperitoneum
nal complexity Even with no internal complexity, the mass
should be considered a well-differentiated
liposarcoma
Well-differentiated Predominantly fat containing, with thick Most common primary retroperitoneal sarcoma
liposarcoma septa and soft-tissue nodularity (usu- No potential to metastasize, but poor long-term
ally ≥1 cm) survival due to high risk of local recurrence
and potential for degeneration to a dediffer-
entiated liposarcoma
Dedifferentiated Fat containing, with increasing internal Potential to metastasize
liposarcoma complexity Can arise in a preexisting or current well-differ-
entiated liposarcoma
Myxoid liposar- Paucity of fat, often present in septa or as Propensity to metastasize to unusual soft-tissue
coma small nodules and bone locations
Variable myxoid tissue (T2-hyperintense
with variable enhancement)
Pleomorphic lipo- Trace of fat to no visible fat Aggressive tumor
sarcoma Heterogeneous appearance with necrosis May metastasize early to the lungs in 75% of
and/or hemorrhage patients
Smooth muscle
Leiomyoma Similar appearance to that of smooth Uncommon
muscle Most often diagnosed in women of reproduc-
Hypointense at T2-weighted MRI, with tive age, usually hormonally driven
variable enhancement
Leiomyosarcoma Heterogeneous mass with involvement Second most common primary retroperitoneal
of a contiguous vessel and variable sarcoma
necrosis Classified as extravascular, intravascular, or a
combination of both
Fibroblastic
Solitary fibrous Heterogeneous appearance Most common location in the abdomen is the
tumor Often highly vascular with prominent retroperitoneum
collateral vessels at CT Malignancy rate, 20%–40%
Flow voids at MRI Small percentage are associated with paraneo-
plastic syndromes, most commonly Doege-
Potter syndrome (hypoglycemia due to exces-
sive production of insulin-like growth)
Myxofibrosar- Imaging appearance is dependent on Common extremity sarcoma in elderly patients,
coma histologic grade: low-grade tumors but retroperitoneal location is rare
show abundant myxoid matrix, while High rate of local recurrence regardless of
high-grade lesions are more heteroge- grade; however, high-grade tumors are more
neous; may show “tail” sign at contrast likely to metastasize
material–enhanced T1-weighted MRI
Neurogenic
Neurofibroma Target sign: peripheral signal hyperinten- Subtypes: localized, diffuse, plexiform
sity with central signal hypointensity Neurofibromatosis 1 is associated with the pres-
ence of multiple neurofibromas or a plexi-
form neurofibroma
(Table continues)

more likely to be primary retroperitoneal tumors. “beak” sign, the “phantom organ” sign, the
In addition, displacement of the abdominal aorta “embedded organ” sign, and the “prominent
and/or the inferior vena cava and their branches feeding artery” sign (8). The beak sign is present
can be suggestive of a retroperitoneal mass. when the deformed edges of the organ adjacent
Certain radiologic signs can also be used to the index mass appear beak-shaped, suggest-
to confirm the lesion’s location, including the ing that the mass originates from the organ, while
1634 October Special Issue 2020 radiographics.rsna.org

Schwannoma Difficult to distinguish from a neurofi- Mostly sporadic

broma Small subset associated with neurofibromatosis
May show fascicular sign (numerous in- Rate of successful surgical excision generally
ternal ringlike structures at T2-weighted higher than that of neurofibromas
MRI) Malignant degeneration rare
Malignant pe- Difficult to distinguish from neurofibroma Approximately 50% arise de novo
ripheral nerve and schwannoma The rest are seen in neurofibromatosis 1, with
sheath tumor Clues include larger size, ill-defined a small subset occurring in patients with a
borders, internal heterogeneity with remote history of radiation therapy
necrosis and/or hemorrhage, and new
or worsening neurologic deficit
Ganglioneuroma Paravertebral mass Benign tumors arise from paravertebral sympa-
Heterogeneous with a variable amount of thetic ganglia
myxoid stroma Manifest most commonly in children, adoles-
“Whorled” appearance at T2-weighted cents, and young adults
MRI Rarely secrete sufficient amounts of catechol-
amines to cause sympathetic symptoms (eg,
flushing)
Ganglioneuroblas- Heterogeneous Malignant tumor
toma Calcifications, necrosis, and hemorrhage Commonly arises in adrenal medulla
more common than ganglioneuroma Primarily affects children aged 2–4 years
Avid radiotracer uptake at iodine 123 (123I)
metaiodobenzylguanidine (MIBG) MRI
in approximately 70% of cases
Paraganglioma Classically hyperintense at T2-weighted Most common location in abdomen is the origin
MRI, with hypervascularity of the inferior mesenteric artery near the aor-
More complex appearance is frequently tic bifurcation (organ of Zuckerkandl)
observed because of hemorrhage and/ Associated with multiple endocrine neoplasia
or necrosis syndromes and Von Hippel–Lindau syndrome
Gallium 68 (68Ga) tetraazacyclodo-
decane tetraacetic acid-octreotate
(DOTATATE) PET/CT is superior for
localizing and staging
Skeletal muscle: Heterogeneous, nonspecific infiltrative ap- Most common soft-tissue sarcoma in children
rhabdomyosar- pearance, with regions of necrosis aged 15 years and younger
coma Most commonly seen in the head or neck
Retroperitoneal location seen in 7%–19% of
patients
Miscellaneous
Undifferentiated Heterogeneous nonspecific appearance; Formerly known as malignant fibrous histiocy-
pleomorphic calcifications seen in 5%–20% of cases toma
sarcoma Diagnosis of exclusion
Alveolar soft-part Heterogeneous hypervascular mass; ne- Manifests as a painless slowly growing tumor but
sarcoma crosis and hemorrhage may be present frequently metastasizes
Cystic
Lymphatic malfor- Involves more than one retroperitoneal Considered a form of slowly growing vascular
mation compartment malformation
Insinuates between structures Most common in the head and neck; only 5% in
Fluid-fluid levels may be seen the abdomen
Chylous content may be detectable with Treatment is percutaneous sclerotherapy and/or
chemical-shift MRI surgery
Tailgut cyst/ret- Uni- or multilocular cystic masses in the Congenital lesions arising from the embryonic
rorectal cystic presacral space, with variably thick hindgut
hamartoma septa and occasional calcifications Treatment is surgical excision owing to risk of
infection and malignant degeneration
Cystic teratoma Presence of fat-fluid levels and calcifica- Germ cell neoplasms
tions (coarse or toothlike) Retroperitoneal location is rare in adults
RG • Volume 40 Number 6 Al-Dasuqi et al 1635

Figure 1. Normal retroperitoneal anatomy. (a) Axial illustration at the level of the kidneys shows the normal tricom-
partmental retroperitoneal spaces. The anterior pararenal space (shaded blue) is delineated anteriorly by the posterior
layer of the parietal peritoneum (dashed blue line), posteriorly by the anterior renal fascia (dashed black circle), and
laterally by the lateroconal fascia (dashed red circle). The posterior pararenal space (shaded green) is delineated ante-
riorly by the posterior renal fascia (dashed white circle) and posteriorly by the transversalis fascia (dotted green line).
The perirenal space (shaded orange) is delineated anteriorly by the anterior renal fascia and posteriorly by the poste-
rior renal fascia. The respective fascial layers can expand, giving rise to interfascial planes that allow communication
across the retroperitoneal compartments. LCP = lateroconal plane, RMP = retromesenteric plane, RRP = retrorenal plane.
(b) Sagittal illustration shows the retromesenteric plane (shaded blue, small solid arrow) and the retrorenal plane (shaded
green, dashed arrow), which give rise to the combined interfascial plane (shaded red, thick solid arrow), which allows
communication to the retroperitoneal portions of the pelvis. (c) Axial illustration of the right kidney shows the presence
of bridging septa (white arrows) that allow communication from the perirenal space to the retromesenteric (shaded light
blue) and retrorenal (shaded green) planes, respectively. Perirenal lymphatic vessels (thick solid black arrow), arteries
(dashed black arrow), and veins (thin solid black arrow) are also shown. (Figures 1a and 1b adapted and reprinted, with
permission, from reference 3. Figure 1c adapted and reprinted, with permission, from reference 7.)

are characterized on the basis of their location

as (a) superficial lipomas, when they occur in
the subcutaneous soft tissues, or (b) deep-seated
lipomas, when they are deep to the superficial
fascia. While lipomas are the most common soft-
tissue tumor (accounting for 50% of all soft-tis-
the embedded organ sign is seen when the organ sue masses), retroperitoneal lipomas are rare, and
adjacent to the tumor is embedded in it, rather thus, any primarily fat-containing retroperitoneal
than being compressed, with a crescent shape mass should be considered a well-differentiated
(8). These signs are typically absent in primary liposarcoma rather than a lipoma, until it is
retroperitoneal tumors and are more suggestive proven otherwise (10).
that the mass arises from an organ in the retro- The median age at presentation of patients
peritoneal cavity. with lipomas is approximately 53 years (11), ac-
cording to a recent study consisting of 66 patients
Adipocytic Tumors with a pathologically proven diagnosis of lipoma.
Patients are often asymptomatic, and the lipoma
Lipomas only comes to clinical attention once the lesion
has grown large enough to cause mass effect on
Epidemiologic and Clinical Features.—Lipomas adjacent organs and structures (12). Diagnosis
are benign mesenchymal tumors consisting of of a retroperitoneal lipoma on the basis of biopsy
encapsulated mature adipose tissue (9). Lipomas alone should be made with caution because the
1636 October Special Issue 2020 radiographics.rsna.org

Figure 2. Lipoma in a 34-year-old woman who presented with a long-standing history of left lower quadrant pain,
frequent bowel movements, and urinary urgency. (a, b) Axial contrast-enhanced CT image (a) and contrast-enhanced
T1-weighted fat-saturated MR image (b) show a lipomatous mass (* in a) occupying the retroperitoneal space along the
left pelvic sidewall, with a few minimally enhancing thin septa (arrow in b). (c) At gross pathologic evaluation, the mass
is thinly encapsulated, soft, and yellow, with homogeneously yellow lobulated cut surfaces (*) without grossly identifi-
able fibrosis, necrosis, calcification, or hemorrhage. (d) Medium-power photomicrograph shows mature adipocytes, with
minimal size variation and without nuclear atypia. (Hematoxylin-eosin stain; original magnification, 40.) Adipocytes are
divided into lobules by fibrous bands (arrow) with intermixed small- and medium-sized vessels (arrowheads).

tissue may be derived from lipoma-like areas of at MRI, thick or irregular capsules or septa,
an otherwise under-sampled well-differentiated internal nodular enhancement, or necrosis in a
liposarcoma (12). retroperitoneal fat-containing mass should raise
suspicion for a lipomatous sarcoma, and biopsy
Radiologic Features.—MRI is the modality of and/or surgical resection are warranted in those
choice for evaluation of fat-containing lesions ow- cases (13).
ing to its superior soft-tissue resolution. Lipomas
most often show homogeneous signal intensity Pathologic and Molecular Features.—At gross
at MRI that is isointense compared with sub- pathologic evaluation, lipomas are well-circum-
cutaneous fat during all sequences, and chemi- scribed and encapsulated tumors that display
cal fat saturation is key in confirming the pure homogeneous lobulated cut surfaces, often with-
lipomatous nature of the lesions. Thin septa may out fibrosis, necrosis, calcification, or hemorrhage
occasionally be present in lipomas, and they may (Fig 2c). Microscopic features include mature
rarely demonstrate minimal enhancement with adipocytes, with minimal size variation and with-
administration of intravenous gadolinium con- out nuclear atypia. Adipocytes are divided into
trast material (13). lobules by fibrous bands with intermixed small-
At CT, lipomas typically appear as well-de- and medium-sized vessels (Fig 2d).
fined encapsulated masses with similar attenu-
ation to that of subcutaneous fat (−10 to −100 Liposarcoma
HU) (Fig 2a, 2b) (14). Malignant lipomatous
lesions are difficult to exclude with US alone, Epidemiologic and Clinical Features.—Lipo-
and follow-up MRI should be recommended. sarcoma is a malignant adipose tissue tumor
The presence of incomplete fat suppression (9). It accounts for approximately 15% of all
RG • Volume 40 Number 6 Al-Dasuqi et al 1637

soft-tissue tumors (9), with 10%–20% of lipo- Radiologic Features.—Liposarcomas can have
sarcomas arising in the retroperitoneum (15). a variable imaging appearance, and the amount
Liposarcoma is the most common primary ret- of intratumoral fat is generally inversely propor-
roperitoneal sarcoma, accounting for 35% of all tional to the grade of the tumor. Well-differen-
malignant retroperitoneal soft-tissue tumors in tiated liposarcomas are usually predominantly
adult patients (16). They often manifest as large fatty at CT and MRI and may demonstrate
encapsulated fat-containing masses that displace thick septa or soft-tissue–attenuating nodular-
adjacent structures, and average patients typi- ity that could exhibit mild to marked enhance-
cally present during their fifth through seventh ment after contrast material administration (Fig
decades of life (15–17). 3a, 3b) (12). Other imaging features that have
Liposarcomas are histologically divided into been shown to favor the diagnosis of well-dif-
four subtypes including (a) well-differentiated ferentiated liposarcoma over benign lipomatous
liposarcoma (also called atypical lipomatous tu- tumors include a lesion size of 10 cm or larger
mor when found in the extremities), (b) myxoid and the presence of a soft-tissue–attenuating
liposarcoma, (c) pleomorphic liposarcoma, and nodule measuring greater than or equal to 1
(d) dedifferentiated liposarcoma (9). Among cm. The latter feature specifically predicted a
these, well-differentiated liposarcomas are the diagnosis of well-differentiated liposarcoma over
most common, followed by the dedifferentiated lipoma in all cases in one study (23,24). While
subtype, with myxoid and pleomorphic liposar- primary retroperitoneal teratomas can also
comas rarely seen in the retroperitoneum (15). contain macroscopic fat, these are rare and may
In one study (15) of 177 patients with retroperi- demonstrate fat-fluid levels and/or calcifications
toneal liposarcomas, well-differentiated tumors (often teethlike).
were found in 56% of patients, while dediffer- Because dedifferentiated liposarcomas are be-
entiated lesions were present in 37% of patients. lieved to arise in a preexisting well-differentiated
Well-differentiated liposarcomas are considered liposarcoma, the classic imaging appearance of a
intermediate-grade tumors, according to the dedifferentiated liposarcoma is a bimorphic mass
World Health Organization (WHO) classification with clear demarcation between the predomi-
system, while the other subtypes are considered nantly fatty tissue and the nonfatty solid tissue
high-grade tumors (9). (Fig E2a; a full DICOM image stack is available
Well-differentiated retroperitoneal liposar- online). Calcifications are seen in approximately
comas have no potential to metastasize, but the one-fifth of cases (25). Myxoid liposarcomas
long-term mortality rate can reach 22%–33% are often predominantly nonfatty, with a cystic
owing to a high risk of recurrence and a po- appearance at CT and MRI due to the extra-
tential for malignant degeneration to dedif- cellular myxoid matrix (hypoattenuating com-
ferentiated liposarcoma (17–19). The high risk pared with muscle at CT, high signal intensity
of recurrence is due, in part, to the difficulty of at T2-weighted MRI, and low signal intensity
achieving tumor-free resection margins, be- at T1-weighted MRI) (Fig 4a, 4b) (26). How-
cause tumors are often inseparable from adja- ever, unlike cystic lesions, myxoid liposarcomas
cent structures (15). In one study (19) of 168 demonstrate varying amounts of contrast mate-
patients with retroperitoneal liposarcoma, half rial enhancement (Fig 4c). While they generally
of the patients required resection of at least one contain a paucity of fat, the presence of a small
contiguous organ. Recurrence of well-differenti- amount of fat in lacy septa or nodular compo-
ated liposarcoma is associated with a higher risk nents is pathognomonic for myxoid liposarco-
of dedifferentiation (20). mas. Pleomorphic liposarcomas have a variable
Dedifferentiated liposarcomas are high-grade heterogeneous imaging appearance at CT and
sarcomas that are believed to either arise de novo MRI (Fig 5a–5c), often manifesting as large well-
in a preexisting well-differentiated liposarcoma defined soft-tissue masses, with areas of necrosis
(approximately 90%) or form in a recurrent well- and hemorrhage (12).
differentiated liposarcoma (approximately 10%)
(18). Dedifferentiated liposarcomas are clinically Pathologic and Molecular Features.—Well-dif-
more aggressive than well-differentiated types, ferentiated liposarcomas are grossly well-circum-
with a higher propensity to recur after resection scribed and lobulated tumors with variably firm
and the presence of distant metastases in approx- cut surfaces (Fig 3c) (12). At microscopic evalu-
imately one-fifth of cases (20). Myxoid liposarco- ation, these tumors show lobules of relatively
mas have a propensity to metastasize to unusual mature adipocytes of varying sizes and thickened
soft-tissue and bone locations, while pleomorphic fibrous bands containing atypical stromal cell
liposarcomas can metastasize early to the lungs in nuclei (Fig 3d) (27). Lipoblasts may be identi-
up to 75% of patients (21,22). fied but are not required for the diagnosis (28).
1638 October Special Issue 2020 radiographics.rsna.org

Figure 3. Well-differentiated liposarcoma in a 55-year-old woman who had increasing abdominal firm-
ness, nausea, early satiety, and constipation. (a) Coronal CT image without contrast material shows a large
heterogeneous well-defined predominantly fat-containing mass that displaces the left kidney medially.
Internally, the mass contains a few thick septa (dashed arrow) and soft-tissue nodular components (solid
arrow). (b) Axial contrast-enhanced fat-suppressed T1-weighted MR image shows a fat-containing mass
(*) with thin enhancing septa (arrow). (c) Photograph of the specimen at gross pathologic examination
reveals a lobulated lipomatous tumor adhering to and surrounding the kidney (arrow). The tumor displays
a yellow and fatty (*) to tan-gray (**) cut surface with bands of connective tissue (arrowhead) dividing the
tumor into nodules. (d) Medium-power photomicrograph shows lipoblasts with multiple intracytoplasmic
vacuoles that indent the enlarged and hyperchromatic nucleus (arrows) and thickened fibrous bands (*),
with atypical stromal cell nuclei. (Hematoxylin-eosin stain; original magnification, 200.)

Well-differentiated liposarcomas are character- dedifferentiated liposarcomas show a (usually

ized by MDM2 gene amplification, which can be abrupt) transition from a well-differentiated li-
detected by means of fluorescence in situ hybrid- posarcoma component to a high-grade sarcoma
ization, polymerase chain reaction, and compara- component (Fig E2c) (27,28). However, in
tive genomic hybridization methods (29). many instances, particularly in biopsy samples,
The gross appearance of dedifferentiated these transition areas are not identified, and
liposarcomas varies, depending on the cellular morphologic overlap between dedifferentiated
composition of the tumor. Well-differentiated liposarcomas and undifferentiated pleomor-
liposarcoma components often appear tan- phic sarcomas may complicate the distinction
yellow, lobulated, and greasy; however, dedif- between the two (28). If MDM2 amplification is
ferentiated components are typically variegated, detected, a diagnosis of dedifferentiated liposar-
with firm or fleshy cut surfaces (Fig E2b). Thor- coma can be made (27,28). Moreover, poorly
ough gross sampling of all regions is critical for differentiated retroperitoneal tumors are much
accurate tumor classification. Microscopically, more likely to be dedifferentiated liposarcomas
RG • Volume 40 Number 6 Al-Dasuqi et al 1639

Figure 4. Myxoid liposarcoma in a 68-year-old man who presented with a 3-month history of worsen-
ing abdominal pain. (a, b) Axial T2-weighted MR images acquired without (a) and with (b) fat sup-
pression reveal a hyperintense mass (* in a) without fat suppression. Lacelike signal hypointensity is
seen (arrowhead in b). (c) Axial fat-suppressed contrast-enhanced T1-weighted MR image shows patchy
areas of enhancement (arrow). (d) Photograph at gross pathologic examination shows a bivalved well-
circumscribed mass with a tan-yellow gelatinous smooth shiny cut surface (*) and areas of hemorrhage
(**) covering 30% of the total surface area. The tumor appears completely encased by a thin fibrous
pseudocapsule (arrow). (e) Low-power photomicrograph shows abundant myxoid stroma that contains
spindled tumor cells and delicate arborizing “chicken-wire” capillary vasculature (arrows). (Hematoxylin-
eosin stain; original magnification, 100.)

than they are to be undifferentiated pleomor- lipoblasts, and delicate arborizing “chicken-wire”
phic sarcomas (27,28). capillary vasculature (Fig 4e) (9). More than 90%
At gross pathologic examination, myxoid liposar- of cases harbor t(12,16)(q13;p11) translocation,
comas are well-circumscribed masses with lobu- involving the DDIT3 and FUS genes (30).
lated gelatinous cut surfaces. High-grade tumors At gross pathologic evaluation, pleomorphic
exhibit variably firm gray foci, with areas of necrosis liposarcomas often manifest as multinodular
and/or hemorrhage (Fig 4d). At microscopic evalu- masses with tan-white to yellow and firm to
ation , myxoid liposarcomas show an abundant fleshy cut surfaces (Fig 5d). Internally, areas of
myxoid stroma that contains spindled tumor cells, necrosis may be seen. Microscopic features are
1640 October Special Issue 2020 radiographics.rsna.org

Figure 5. Pleomorphic liposarcoma in a 65-year-old woman with an 18-month history of vague abdominal pain.
(a, b) Coronal T2-weighted MR images acquired without (a) and with (b) fat suppression reveal a fat-containing mass (* in
a) with a hyperintense soft-tissue component (arrow in b). (c) Axial fat-suppressed contrast-enhanced T1-weighted MR im-
age shows enhancement of the soft-tissue component (arrow). (d) Photograph of the mass at gross pathologic examination
shows a multinodular tan-white to yellow mass that contains firm (*) to fleshy (arrowhead) cut surfaces. Occasional areas of
necrosis (arrow) are identified. (e) Medium-power photomicrograph reveals numerous uni- (*) and multivacuolated (arrows)
lipoblasts demonstrating large pleomorphic nuclei scalloped by lipid vacuoles and surrounded by pleomorphic spindle cells.
(Hematoxylin-eosin stain; original magnification, 200.)

variable but unified by the presence of univacu- Smooth Muscle Tumors

olated or multivacuolated lipoblasts, which must
be seen to make the diagnosis (Fig 5e) (28). Leiomyoma
Lipoblasts are defined as cells with hyperchro-
matic nuclei that are scalloped by lipid vacuoles. Epidemiologic and Clinical Features.—Leio-
Lipoblasts are often admixed with large high- myoma is a benign tumor of smooth muscle
grade pleomorphic cells (28). In a case series of cells (9). Primary retroperitoneal leiomyomas
57 cases of pleomorphic liposarcomas, Hornick are rare and are believed to arise from remnants
et al (31) noted considerable variability in the of the müllerian or wolffian ducts (32). Most
quantity of lipoblasts in the examined tumors, primary retroperitoneal leiomyomas are seen
ranging from less than 1% to 80%. Significant in women, but a small percentage of cases have
variability was also noted between separate areas been described in men (33,34). Up to 40% of
in the same tumor. This histologic finding may retroperitoneal leiomyomas are associated with
explain the intralesional variability in the imag- uterine leiomyomas (32). Pelvic retroperitoneal
ing appearance of pleomorphic liposarcomas, as leiomyomas are sometimes referred to as parasitic
noted in Figure 5. leiomyomas when they are close to the uterus. In
RG • Volume 40 Number 6 Al-Dasuqi et al 1641

patients with a parasitic leiomyoma, the tumor and 23% of patients with intravascular tumors,
is believed to originate from the uterus but, over with the lungs, liver, and peritoneum being the
time, it becomes adherent to adjacent structures, most common sites (39). Prognosis is typically
developing an extrauterine blood supply and dependent on achieving complete excision with
subsequently losing its original attachment to the wide negative margins (40).
uterus (32,35). Retroperitoneal leiomyomas may
manifest with vague symptoms including pelvic Radiologic Features.—Leiomyosarcomas usually
and/or back pain and discomfort. Presenting manifest as large soft-tissue masses, with internal
symptoms may also be secondary to compression heterogeneity and heterogeneous enhancement
of adjacent structures, including urinary urgency usually secondary to necrosis and hemorrhage
and constipation (35). (Fig 6a). The mean tumor size for lesions with
and without vascular involvement has been
Radiologic Features.—Retroperitoneal leiomyo- reported as 10.4 cm and 11.3 cm, respectively
mas are similar in attenuation to uterine myo- (41). Calcifications are not commonly found, and
metrial smooth muscle at CT (Fig E3a, E3b; adipose tissue is absent (39). Purely intravascu-
a full DICOM image stack is available online). lar lesions appear as heterogeneously enhancing
Calcifications are not commonly present. At T2- expansile masses. They commonly exhibit low to
weighted MRI, they typically show signal hypoin- intermediate signal intensity at T1-weighted MRI
tensity and variable enhancement with adminis- and intermediate to high signal intensity at T2-
tration of contrast material (35). weighted MRI. They also occasionally demon-
strate fluid-fluid levels secondary to hemorrhage
Pathologic and Molecular Features.—At gross (26). A large non–fat-containing retroperitoneal
pathologic examination, leiomyomas typically mass with involvement of a contiguous vessel and
manifest as well-circumscribed masses with varying internal necrosis should raise the possi-
tan whorled cut surfaces (Fig E3c). Histologic bility of a leiomyosarcoma (Fig 6b) (26).
features include intersecting fascicles of uniform
spindle cells, with blunt-ended nuclei and eosino- Pathologic and Molecular Features.—At gross
philic cytoplasms (34). Nuclear pleomorphism, pathologic evaluation, leiomyosarcomas appear
mitotic activity, and necrosis are typically absent as large masses with fleshy tan-white cut surfaces.
(34). In female patients, retroperitoneal leiomyo- Hemorrhage and/or necrosis occur frequently (Fig
mas are hormonally sensitive and frequently stain 6c). Microscopically, leiomyosarcomas show inter-
positive for estrogen and progesterone receptors secting fascicles of spindled cells, with abundant
(34), as shown in Figure E3d. mitotic activity and nuclear atypia (Fig 6d, 6e) (34).

Leiomyosarcoma Fibroblastic Tumors

Epidemiologic and Clinical Features.—Leio- Solitary Fibrous Tumors

myosarcoma is a malignant tumor of smooth
muscle cells (9), with a retroperitoneal location Epidemiologic and Clinical Features.—Solitary
in 12%–69% of cases (36,37). It is the second fibrous tumors, previously known as localized
most common primary malignant retroperito- mesothelioma, are rare mesenchymal tumors that
neal sarcoma, accounting for 28% of cases (26). arise from fibroblast-like cells in the connective
Leiomyosarcomas are typically diagnosed dur- tissue (9). They are on the same spectrum of tu-
ing the fifth to sixth decades of life, more often mors as hemangiopericytomas, and the two terms
in women than in men (38). Leiomyosarcoma is are often used interchangeably (42). Approxi-
believed to originate from the large blood ves- mately one-third of solitary fibrous tumors are
sels in the retroperitoneum (eg, the inferior vena intraabdominal, and the most common intraab-
cava) and/or their tributaries (39). Nevertheless, dominal site is the retroperitoneum. The majority
leiomyosarcomas most commonly manifest as ex- of patients are diagnosed in the fifth to seventh
travascular tumors (62% of cases) and are rarely decades of life, but the tumor can arise at any age
completely intravascular (5%) (38). In 33% of (42). Most solitary fibrous tumors are benign,
cases, leiomyosarcomas have both an intravascu- with an estimated malignancy rate of 20%–40%
lar and an extravascular component (38). Similar (42). Retroperitoneal solitary fibrous tumors are
to liposarcomas, leiomyosarcomas are often large usually initially asymptomatic and can grow large
when patients present, and symptoms are usu- before causing symptoms. A small percentage of
ally related to compression of adjacent structures retroperitoneal solitary fibrous tumors are associ-
(26,39). Metastases are seen at the time of diag- ated with paraneoplastic syndromes, most com-
nosis in 9% of patients with extravascular tumors monly Doege-Potter syndrome, which occurs in
1642 October Special Issue 2020 radiographics.rsna.org

Figure 6. Leiomyosarcoma in two patients.

(a) Coronal contrast-enhanced CT image in a
66-year-old man with a 3-month history of ab-
dominal pain, decreased appetite, and weight
loss shows a large heterogeneous right peri-
renal mass (arrow), with regions of necrosis
(*) and without invasion of the right kidney.
(b) Axial fat-saturated contrast-enhanced
T1-weighted image in a 68-year-old woman
shows a left retroperitoneal leiomyosarcoma
(*) invading the inferior vena cava (arrowhead). (c) Photograph at gross pathologic examination of the lesion in a shows that the
tumor is inferior to the kidney and entirely extrarenal. It displays a tan-white whorled stellate appearance (*). (d) Low-power photo-
micrograph of a specimen from the patient in a shows intersecting fascicles of spindled cells (arrows) with abundant mitotic activity
and nuclear atypia. (Hematoxylin-eosin stain; original magnification, 100.) (e) Photomicrographs of a specimen from the patient in
a show that these cells are immunoreactive for desmin (top left) and smooth muscle actin (top right) and are negative for anaplastic
lymphoma kinase (bottom left) and S100 (bottom right), supporting the diagnosis of leiomyosarcoma. (Desmin, smooth muscle
actin, anaplastic lymphoma kinase, and S100 stains, respectively; original magnification, 100.)

patients with hypoglycemia secondary to excessive signal intensity at T1-weighted and T2-weighted
production of insulin-like growth factor 2 from MRI is variable and is dependent on cellular-
tumor cells (41). ity and the abundance of myxoid stroma and/
or fibrous tissue (43). Solitary fibrous tumors
Radiologic Features.—Although their imaging are typically hypointense to isointense compared
appearance is nonspecific, most solitary fibrous with muscle at T1-weighted MRI and hypoin-
tumors show heterogeneity and are highly tense at T2-weighted MRI (44). Avid contrast
vascular at contrast-enhanced imaging, with material enhancement is usually noted, and flow
prominent collateral vessels (Fig E4a) (43,44). voids (manifesting as hypointense foci at T1-
They may rarely exhibit areas of cystic degen- and T2-weighted MRI) may be present owing to
eration, necrosis, and calcifications (43). The prominent intralesional vessels (44).
RG • Volume 40 Number 6 Al-Dasuqi et al 1643

Pathologic and Molecular Features.—Solitary hanced T1-weighted MRI was 79%–90% specific
fibrous tumors manifest as well-circumscribed for myxofibrosarcoma. This infiltrative appearance
firm masses with tan fleshy lobulated cut surfaces has also been shown to be associated with a higher
(Fig E4b) (45). Microscopically, these tumors are rate of recurrence after surgical resection (50).
composed of round to spindled cells with a pat-
ternless arrangement around ectatic “staghorn” Pathologic and Molecular Features.—At gross
vessels (Fig E4c) (46). Tumor cells are charac- pathologic examination, myxofibrosarcomas are
teristically positive for the STAT6 gene, and most typically multinodular growths with tan-white
cases harbor 12q intrachromosomal rearrange- gelatinous cut surfaces. Areas of necrosis are
ments, resulting in NAB2-STAT6 gene fusion commonly seen in high-grade tumors (Fig E5b).
(Fig E4d) (47). At microscopic evaluation, myxofibrosarcomas
characteristically show a multinodular growth
Myxofibrosarcoma pattern, with incomplete fibrous septa, myxoid
stroma–containing pleomorphic tumor cells,
Epidemiologic and Clinical Features.—Myxofi- frequent atypical mitotic figures, and surrounding
brosarcoma, previously known as a myxoid variant curvilinear thin-walled blood vessels (Fig E5c).
of malignant fibrous histiocytoma, is one of the The tumor grade, reflected by the degree of cellu-
most common malignant soft-tissue tumors in larity and atypia, is predictive of the prognosis and
elderly patients (9,48) and shows a slight predi- risk of metastasis (28).
lection for male patients (48). Low-grade lesions
tend to be hypocellular and contain an abundant Neurogenic Tumors
myxoid matrix, while high-grade lesions are hy-
percellular, with prominent cellular pleomorphism Neurofibromas
and atypical mitotic figures, and contain areas of
hemorrhage and necrosis, with little myxoid ma- Epidemiologic and Clinical Features.—Neurofi-
trix (<10% of the tumor area) (49). Despite being bromas are benign nerve sheath tumors that are
one of the most common sarcomas of the extremi- composed of benign neoplastic Schwann cells in-
ties in elderly patients, myxofibrosarcomas arising termixed with fibroblasts, collagen, and a myxoid
in the retroperitoneum are rare (48). Myxofibro- matrix (51). They are often unencapsulated and
sarcomas are associated with a relatively high rate interspersed with nerve fibers (51). Neurofibromas
of local recurrence after surgical resection when can occur in the cutaneous or deep soft tissues,
compared with other sarcomas, owing to the infil- and they account for 1% of all retroperitoneal
trative nature of the tumor, regardless of the tumor tumors (52). The main subtypes are (a) localized,
grade. A high tumor grade correlates positively (b) diffuse, and (c) plexiform (51). Solitary local-
with the likelihood of metastatic spread (48). ized neurofibromas are often sporadic and are not
associated with neurofibromatosis type 1, whereas
Radiologic Features.—The imaging appearance multiple neurofibromas or plexiform neurofibro-
of myxofibrosarcoma is highly dependent on the mas are nearly always associated with neurofibro-
histologic tumor grade (49). Low-grade lesions matosis type 1 (51).
are hypoattenuating compared with muscle at CT The age of onset in patients with solitary
and show high T2 and low T1 signal intensity at neurofibromas is 20–30 years, but neurofibro-
MRI owing to the abundant myxoid matrix (49). mas in patients with neurofibromatosis type 1
On the other hand, high-grade lesions tend to have often present at an earlier age (53). Malignant
similar attenuation to that of muscle at CT and ex- degeneration of neurofibromas is more common
hibit intermediate signal intensity at T1-weighted with plexiform neurofibromas or in patients with
MRI that is secondary to hypercellularity, which neurofibromatosis type 1 (54). Neurofibromas
is also associated with more avid contrast material are frequently discovered incidentally when they
enhancement (49). They can also show regions of arise in the retroperitoneum because they are
hemorrhage and/or necrosis (Fig E5a; a full DI- more likely to be asymptomatic (53). Solitary
COM image stack is available online.). A tail-like localized neurofibromas are usually managed
pattern is often described (50) in myxofibrosar- conservatively, with observation, unless they are
coma at T2-weighted and fat-suppressed contrast- associated with pain or a neurologic deficit, or if
enhanced T1-weighted MRI that is secondary to there is clinical concern for malignant potential.
the curvilinear tumoral fascial projections extend- The goal of surgery is to attempt resection with
ing from the epicenter of the mass. In a study (50) preservation of nerve function (53).
of 96 patients with predominantly myxoid neo-
plasms, including myxofibrosarcoma, the presence Radiologic Features.—Localized neurofibromas
of this “tail sign” at fat-suppressed contrast-en- typically manifest as well-defined minimally
1644 October Special Issue 2020 radiographics.rsna.org

Figure 7. Neurofibroma in a 54-year-old woman with an incidentally discovered left retroperitoneal mass. (a) Axial
contrast-enhanced CT image shows a well-defined low-attenuation retroperitoneal mass (arrow) with the claw sign,
which is seen in association with the displaced left psoas muscle. (b, c) Axial fat-saturated T2-weighted MR image (b)
shows a homogeneously hyperintense mass (arrow), and axial fat-saturated contrast-enhanced T1-weighted MR
image (c) shows diffuse enhancement (arrowhead in c). (d) Axial T2-weighted MR image with fat suppression in an
18-year-old woman with neurofibroma shows the classic target sign, as evidenced by the characteristic central area
of low signal intensity surrounded by a rim of high signal intensity (arrowhead). (e) Photograph of gross pathologic
specimen shows that the mass seen in a–c is well circumscribed with a solid pale yellow and homogeneous cut sur-
face. (f) High-power photomicrograph shows a moderately cellular proliferation of loosely arranged ovoid to spindle
cells with small wavy nuclei (arrows) in a myxedematous stromal matrix. (Hematoxylin-eosin stain; original magnifi-
cation, 400.) The lesional cells have benign cytologic features and lack substantial mitotic activity.

enhancing hypoattenuating masses (20–40 HU) can variably demonstrate a target sign, which
at CT (Fig 7a) (53). They often show signal consists of a central focus of low signal intensity
isointensity to hypointensity compared with that is presumed to reflect the centrally dense
muscle at T1-weighted MRI and signal hyper- fibrocollagenous tissue, surrounded by peripheral
intensity at fluid-sensitive MRI, with variable hyperintensity, which is thought to be secondary
enhancement (Fig 7b, 7c) (53). Neurofibromas to the peripheral myxoid matrix at T2-weighted
RG • Volume 40 Number 6 Al-Dasuqi et al 1645

MRI (Fig 7d) (55). Plexiform neurofibromas ringlike structures internally (59). As schwannomas
tend to manifest as large infiltrative multilobu- age, they undergo cystic degeneration (56). This is
lated masses or a conglomerate of masses along more commonly seen in retroperitoneal schwan-
the nerve fibers (56). Retroperitoneal plexiform nomas as they grow to be larger than 5 cm (Fig 8a,
neurofibromas tend to be bilateral, symmetric, 8b). Long-standing schwannomas can also show
and parallel to the psoas muscle, which can be calcifications and hemorrhage (26,32).
an important imaging clue to differentiate them
from other retroperitoneal sarcomas (56). Pathologic and Molecular Features.—At gross
pathologic evaluation, schwannomas are well-
Pathologic and Molecular Features.—At gross circumscribed encapsulated masses that display
pathologic evaluation, most neurofibromas mani- tan-yellow cut surfaces and grow eccentrically to
fest as well-circumscribed unencapsulated masses the tumor from which they originate (51). Large
with pale-yellow homogeneous cut surfaces (Fig long-standing tumors may undergo cystic de-
7e). Plexiform neurofibromas tend to be larger, generation and may show hemorrhage, necrosis,
multinodular, and elongated (51). Microscopi- and calcification (Fig 8c) (51). Characteristic
cally, neurofibromas exhibit hypocellular to mod- microscopic features include admixed cellular
erately cellular proliferations of spindle cells with “Antoni A” and loose hypocellular “Antoni B”
small wavy nuclei in a myxoedematous stromal zones. Antoni A zones commonly exhibit Verocay
matrix (Fig 7f) (51). Tumor cells are cytologically body formation, which is defined as rows of pali-
benign and lack substantial mitotic activity. Scat- sading cells separated by a hypocellular hyalin-
tered mast cells are commonly seen (51). ized area (Fig 8d, 8e) (51). Schwannomas are
strongly and diffusely S100 positive (51).
Schwannomas
Malignant Peripheral Nerve Sheath
Epidemiologic and Clinical Features.—Schwan- Tumors
nomas, also known as neurolemmas, are another
type of benign nerve sheath tumor that account Epidemiologic and Clinical Features.—Malig-
for 4% of all retroperitoneal tumors (56). As nant peripheral nerve sheath tumors (MPNSTs)
opposed to neurofibromas, they are exclusively are malignant soft-tissue sarcomas that arise
made of benign neoplastic Schwann cells and are from peripheral nerves either de novo (ap-
separable from the nerve fibers (51). Thus, they proximately 50%) or in a preexisting plexiform
tend to grow eccentrically from peripheral nerve neurofibroma (54). They account for up to
fibers (51). Schwannomas can occur at any age 10% of soft-tissue sarcomas (56). The age at
but are more common between the second and onset in patients with neurofibromatosis type
fifth decades of life (51). Most cases are sporadic, 1 is typically 20–30 years, and patients without
with the exception of a small fraction of cases neurofibromatosis type 1 tend to be older than
that can be associated with neurofibromatosis 60 years at diagnosis (60). A small subset of
type 2 (51). Malignant degeneration is rare in MPNSTs occur in patients who have undergone
schwannomas (32). They are also less likely to prior radiation therapy (often for breast cancer
produce symptoms compared with neurofibro- or lymphoma), with an average lag time of 15
mas, and the rate of successful surgical resection years (54,61). Presenting symptoms are usu-
is generally higher (57). ally related to the nerve or nerves involved and
are similar to symptoms of neurofibroma (53).
Radiologic Features.—Schwannomas are often A sudden increase in size of a known neurofi-
indistinguishable from neurofibromas at imag- broma is also suggestive of an MPNST (62).
ing (58). They often appear as round or fusiform Long-term prognosis is poor because of the po-
hypoattenuating masses at CT (26). The typical tential of local recurrence and metastasis (54).
signal intensity characteristics of schwannomas at
MRI are similar to those of neurofibromas, with Radiologic Features.—MPNSTs show het-
intermediate signal intensity at T1-weighted MRI erogeneous attenuation at CT, with a higher
(iso- to hypointense to muscle) and signal hyperin- likelihood of central necrosis and/or hemorrhage
tensity at T2-weighted MRI (56). Variable enhance- compared with benign neurogenic tumors (53).
ment is seen at CT and MRI (26). In addition, The MRI appearance is also heterogeneous,
schwannomas can show the target sign, although with possible regions of necrosis and/or hemor-
it is seen less frequently compared with neurofi- rhage (Fig 9a–9c). Among the key features that
bromas (58). The fascicular sign is another typical differentiate MPNST from neurofibroma in-
imaging appearance seen in schwannomas at T2- clude large tumor size, peripheral enhancement,
weighted MRI, where they demonstrate numerous perilesional edema, intralesional cysts, and
1646 October Special Issue 2020 radiographics.rsna.org

Figure 8. Schwannoma with cystic degeneration in a 41-year-old woman with a calcified abdominal mass seen on a radiograph of
the lumbar spine obtained for sciatica (not shown). (a) Axial contrast-enhanced CT image shows a low-attenuation mass with linear
and curvilinear calcifications (arrow). (b) Axial fat-saturated T2-weighted MR image shows the mass to be heterogeneously hyperin-
tense, with an internal cystic formation (*). (c) Photograph from gross pathologic examination shows a well-circumscribed tumor with
a tan-yellow solid and cystic cut surface (arrow) that is 60% white fibrous tissue and 40% yellow fatty tissue. In addition, there is a
central hemorrhagic cystic component (*). (d) Low-power photomicrograph shows a central cyst (*) with surrounding smaller cysts
(arrow). (Hematoxylin-eosin stain; original magnification, 40.) (e) High-power photomicrograph shows Verocay bodies made of pali-
sading cells (arrow) that are separated by a hypocellular hyalinized area (*). (Hematoxylin-eosin stain; original magnification, 200.)

ill-defined borders (62,63). Fused fluorine 18 paravertebral sympathetic ganglia and account
(18F)-fluorodeoxyglucose (FDG) PET/CT can for 0.7%–1.6% of all primary retroperitoneal
be useful for evaluation of malignant degenera- tumors (64). Internally, these tumors are com-
tion of neurofibroma to MPNST (63). posed of mature primitive neural crest cells (65).
They are typically asymptomatic and manifest
Pathologic and Molecular Features.—MPNSTs in children, adolescents, and young adults, with
often manifest as large fusiform masses with a slight female predominance (65). Ganglioneu-
fleshy variegated cut surfaces that display hemor- romas can arise from maturing neuroblastomas
rhage and/or necrosis (Fig 9d). Histologically, or ganglioneuroblastomas (66). They rarely
these tumors are variably cellular and composed secrete sufficient amounts of catecholamines to
of fascicles of spindle cells with tapered or wavy cause sympathetic symptoms such as flushing.
nuclei, indistinct cytoplasmic borders, and brisk Complete surgical resection is curative in most
mitotic activity (Fig 9e) (51). In cases originat- patients (66).
ing from a benign nerve sheath tumor, most
commonly a neurofibroma, transition areas of Radiologic Features.—A ganglioneuroma typi-
increased cellularity may be seen (51). cally manifests as a vertically oriented paraver-
tebral mass in the retroperitoneum or posterior
Ganglioneuromas mediastinum. In the retroperitoneum, ganglio-
neuromas can arise from the sympathetic chain
Epidemiologic and Clinical Features.—Ganglio- (more common) or the adrenal medulla (56,66).
neuromas are benign tumors that arise from the At CT, they manifest as a mildly hypoattenuating
RG • Volume 40 Number 6 Al-Dasuqi et al 1647

Figure 9. MPNST in a 12-year-old girl with a his-

tory of neurofibromatosis type 1. (a–c) Coronal
T2-weighted (a) and axial nonenhanced (b) and
contrast-enhanced (c) T1-weighted MR images
show an enhancing T2-hyperintense, T1-isoin-
tense retroperitoneal mass with central necrosis (*
in c). (d) Photograph of the sectioned specimen
shows a lobulated well-circumscribed mass with
a variegated cut surface, including tan-white to
brown areas of viable tumor (*), yellow areas of
necrosis (arrowhead), and cystic hemorrhagic foci
(arrow). (e) Medium-power photomicrograph
shows a highly cellular proliferation of spindle cells
with tapered and wavy nuclei, indistinct cytoplas-
mic borders, and brisk mitotic activity (some atyp-
ical) (arrowheads), arranged in fascicles. (Hema-
toxylin-eosin stain; original magnification, 200.)

well-defined homogeneous mass with variable en- intensity in the lesion at T2-weighted MRI (67).
hancement (56). Calcifications can be seen in up The appearance at contrast-enhanced imaging
to 25% of cases and are often punctate or speck- is variable; however, a lack of early enhancement
led, while central necrosis and hemorrhage are has been observed, with a gradual increase during
rarely encountered (56,66). Heterogeneous signal the more delayed phases (67). This has been pos-
intensity is seen at both T1-weighted and T2- tulated to occur because of the abundant myxoid
weighted MRI, with the degree of hyperintensity matrix, which could result in delayed progressive
at T2-weighted MRI positively correlating with accumulation of contrast material in the extracel-
the signal intensity of the myxoid stroma found lular space (68).
in situ (67). A whorled appearance has also been
described at T2-weighted MRI and manifests as Pathologic and Molecular Features.—At gross
internal curvilinear bands of low signal intensity pathologic evaluation, these tumors are well-
(Fig 10a, 10b) (67). These are thought to repre- defined, with gelatinous tan-gray cut surfaces
sent the interlacing areas of Schwann cells and (Fig 10c). Microscopically, interlacing areas of
collagen fibers (Fig 10d) in a background myxoid spindled Schwann cells with bland pointed nuclei
stroma that represent the surrounding high signal and ganglion cells intermixed with collagenous
1648 October Special Issue 2020 radiographics.rsna.org

Figure 10. Ganglioneuroma in a 39-year-old man with a left suprarenal mass that was found
incidentally at US (not shown). (a) Axial T2-weighted image shows a hyperintense mass (due
to a myxoid stroma), with a curvilinear band of signal hypointensity (arrowhead), correspond-
ing to the whorled appearance that has been described in these masses. (b) Axial fat-saturated
contrast-enhanced T1-weighted MR image shows predominantly peripheral enhancement (ar-
rowhead). (c) Photograph of the gross pathologic specimen shows a well-defined mass with
a gelatinous tan-brown focally hemorrhagic cut surface. (d) Medium-power photomicrograph
shows spindled Schwann cells (*) with bland pointed nuclei and ganglion cells (arrowheads)
intermixed in a collagenous stroma. (Hematoxylin-eosin stain; original magnification, 100.)

stroma are often seen in a background of myxoid Radiologic Features.—The CT and MRI appear-
stroma (Fig 10d). The lack of neuroblasts is key in ances of ganglioneuroblastomas vary from solid
excluding an underlying malignant neoplasm, such to cystic or a combination of both solid and cystic
as ganglioneuroblastoma or neuroblastoma (51). components (66). Enhancement after administra-
tion of contrast material is also variable. (Fig E6a;
Ganglioneuroblastomas a full DICOM image stack is available online.)
Calcifications and necrosis or hemorrhage are
Epidemiologic and Clinical Features.—Ganglio- more commonly present than they are with gan-
neuroblastomas are rare malignant tumors that glioneuromas (65). Although calcification may be
arise from the sympathetic chain and are com- difficult to detect at MRI, the presence of necro-
posed of both mature and immature ganglion sis and hemorrhage manifests as regions of signal
cells (66). Unlike ganglioneuromas, they are most hyperintensity at T1- and T2-weighted MRI. Avid
commonly seen arising from the adrenal medulla, radiotracer uptake at 123I MIBG imaging is seen
followed by the paraspinal sympathetic chain in in approximately 70% of cases (65).
the retroperitoneum, and to a lesser extent, the
posterior mediastinum (65). Ganglioneuroblasto- Pathologic and Molecular Features.—The gross
mas primarily affect children, with a mean age of and microscopic appearances of ganglioneuroblas-
onset of 2–4 years (66). The malignant potential of tomas are variable, depending on the subtype and
ganglioneuroblastomas is dependent on the degree degree of differentiation (Fig E6b, E6c). At gross
of immaturity of the neural crest cells. The prog- pathologic evaluation, these tumors often exhibit
nosis is generally more favorable compared with areas of hemorrhage and/or necrosis. Microscopic
that of neuroblastomas (65). features include neuroblasts, mature ganglion
RG • Volume 40 Number 6 Al-Dasuqi et al 1649

cells, and Schwannian stroma. Patients who have of a hypertensive crisis after the use of intrave-
molecular aberrations in MYC, ALK, or ATRX nous contrast material in patients with known
genes tend to have a worse prognosis (69–71). catecholamine hypersecreting tumors have since
been refuted in subsequent studies (76). At T2-
Paragangliomas weighted MRI, diffuse high signal intensity (a
“lightbulb” appearance) has often been described
Epidemiologic and Clinical Features.—Para- in paragangliomas. However, a more complex
gangliomas, also known as extra-adrenal pheo- appearance is frequently observed because of
chromocytomas, are rare tumors of chromaffin the signal heterogeneity that is associated with
cell origin that arise from the extra-adrenal hemorrhage and/or necrosis (Fig 11b, 11c) (26).
paraganglion cells in the sympathetic or parasym- T1-weighted MRI typically shows intermediate
pathetic chains (9). Sympathetic paragangliomas to low signal intensity. Areas of signal hyperin-
secrete catecholamine, and approximately 40% tensity at T1-weighted MRI may be secondary
of patients with them present with clinical find- to hemorrhage (39). Signal intensity voids at T1-
ings related to excess catecholamine, including and T2-weighted MRI can create the typical “salt
hypertension and tachycardia (26). Parasym- and pepper” appearance (64).
pathetic paragangliomas are located in the base If tumor localization is not achieved or if
of the skull and neck and are associated with there is a high likelihood for distant metastases,
the branches of the glossopharyngeal and vagal functional nuclear imaging with 123I MIBG, in-
nerves, whereas sympathetic paragangliomas dium 111 (111In) pentetreotide, and/or PET are
arise anywhere along the sympathetic chain, often performed (73). Until recently, 18F-FDG
most commonly in the abdomen (approximately PET imaging has been shown to be the most
75% of all sympathetic paragangliomas) (72). sensitive modality for diagnosis of metastatic
Therefore, retroperitoneal paragangliomas are sites of disease (32). However, recent evidence
almost exclusively sympathetic paragangliomas has shown that 68Ga-DOTATATE PET/CT is
(73). In the abdomen, the most common loca- superior to other functional and structural imag-
tion for primary paragangliomas is in the organ of ing modalities for localization and staging of
Zuckerkandl, which is located at the origin of the paragangliomas (77).
inferior mesenteric artery near the aortic bifurca-
tion (26). Pathologic and Molecular Features.—At gross
Paragangliomas are associated with several pathologic examination, these tumors are typi-
genetic disorders in approximately 30%–40% cally well circumscribed with myxoid and gelati-
of cases, including multiple endocrine neoplasia nous cut surfaces (Fig 11d). Microscopic features
syndromes and von Hippel–Lindau syndrome include the characteristic zellballen growth pat-
(39,72). The mean age at presentation of pa- tern, with nests of tumor cells surrounded by fi-
tients with paraganglioma is during the second brovascular septa (Fig 11e) (78). The tumor cells
to fourth decades of life (39). The diagnosis can show eosinophilic granular cytoplasms, central
be made clinically on the basis of detection of nuclei with nucleoli, and intranuclear pseudoin-
elevated metanephrine or vanillylmandelic acid clusions (Fig 11f) (78). A substantial subset of
levels in the urine (73). The risk of malignancy in patients with paragangliomas harbor mutations in
sympathetic paragangliomas is estimated to be in succinate dehydrogenase (79).
the range of 30%–40% (32). Treatment usually
consists of surgical resection with postoperative Skeletal Muscle Tumors
radiation (74,75). Preoperative medical treatment
with α-adrenergic blockers is crucial to preven- Epidemiologic and Clinical Features
tion of life-threatening complications secondary Rhabdomyosarcoma (RMS) is a malignant tumor
to intraoperative catecholamine release (75). arising from primitive mesenchymal cells that
show skeletal muscle differentiation (9). It is the
Radiologic Features.—Benign and malignant most common soft-tissue sarcoma in children
paragangliomas are difficult to distinguish on the aged 15 years and younger. Nevertheless, RMS is
basis of imaging unless tumors appear locally rare in adults (80). RMS is divided into four ma-
aggressive or metastases are present at the time of jor subtypes: (a) embryonal RMS (greater than
diagnosis (56). At CT, paragangliomas are typi- 50% of all cases), (b) alveolar RMS, (c) pleomor-
cally heterogeneous, and necrosis, hemorrhage, phic RMS, and (d) sclerosing and spindle-cell
and/or calcifications can often be seen (56). Avid RMS (9). The most common site of involvement
contrast material enhancement is often noted is the head and neck (approximately 35% of all
because of hypervascularity, especially peripher- cases), followed by the genitourinary system (81).
ally (Fig 11a) (26). Early concerns about the risk Approximately 7%–19% of RMSs arise in the
1650 October Special Issue 2020 radiographics.rsna.org

Figure 11. Paraganglioma in a 47-year-old woman who presented with weight loss, abdominal pain, and sciatica. (a) Axial contrast-
enhanced CT image shows a large mass at the level of the aortic bifurcation, with central necrosis (*) and avid peripheral enhance-
ment (arrow). (b, c) Axial T2-weighted (b) and contrast-enhanced T1-weighted (c) MR images also show the central necrosis (*) and
the avid peripheral enhancement. (d) Photograph at gross pathologic examination shows a well-circumscribed and encapsulated
mass with a myxoid and gelatinous central component (*) and surrounding red hemorrhagic (**) and yellow necrotic (arrowhead)
foci. A large-caliber vessel (inferior mesenteric artery) is completely occluded by the tumor (arrow). (e) Low-power photomicrograph
shows the characteristic zellballen growth pattern with nests of tumor cells (*) surrounded by fibrovascular septa (arrow). (Hematox-
ylin-eosin stain; original magnification, 40.) (f) High-power photomicrograph shows the eosinophilic granular cell cytoplasm, the
central nuclei with nucleoli, and intranuclear pseudoinclusions (arrow). (Hematoxylin-eosin stain; original magnification, 400.)

retroperitoneum (82). In terms of risk stratifica- (Fig E7b). Skeletal differentiation can be defined
tion and prognostication, the retroperitoneum is by means of morphologic, immunohistochemi-
considered an unfavorable primary site (81). cal, ultrastructural, or molecular assessment. The
hallmark microscopic features of embryonal RMS
Radiologic Features includes its “marbled” appearance due to varia-
Retroperitoneal RMS can manifest as well- tions in cellularity, with perivascular tumor cell
circumscribed lesions or show an infiltrative or condensation (Fig E7c) and the various stages of
invasive appearance at the time of diagnosis (83). skeletal muscle embryogenesis commonly exhib-
At CT, they are typically heterogeneously attenu- ited by the primitive mesenchymal cells (85). The
ating, with central low attenuation that is second- rhabdomyoblasts in embryonal RMS are ovoid
ary to necrosis (83). Enhancement with contrast to spindled, contain scant to abundant eosino-
material is also heterogeneous, especially when philic cytoplasms, and are arranged in patternless
there is central necrosis (Fig E7a; a full DICOM sheets (Fig E7d) (85). Alveolar RMS is derived
image stack is available online.) They usually from the alveolar pattern that is seen histologi-
show signal hypo- to isointensity at T1-weighted cally. Alveolar RMS is typically composed of
MRI and iso- to hyperintensity at T2-weighted small blue round lymphoma-like cells (85).
MRI (84). RMS is intensely avid at18F-FDG
PET/CT (81). Miscellaneous Tumors

Pathologic and Molecular Features Undifferentiated Pleomorphic Sarcoma

At gross pathologic examination, RMS can be
well defined or poorly circumscribed and can Epidemiologic and Clinical Features.—Undifferen-
occasionally exhibit necrosis and/or hemorrhage tiated pleomorphic sarcoma replaced the outdated
RG • Volume 40 Number 6 Al-Dasuqi et al 1651

malignant fibrous histiocytoma in the 2002 WHO the overall median survival rate was 11 years in
classification (86). Undifferentiated pleomorphic patients without metastasis and 3 years in patients
sarcomas are commonly seen in patients during with metastasis.
their fifth to seventh decades of life, with a higher
incidence in men (87). They most frequently oc- Radiologic Features.—The imaging appearance
cur in the extremities, followed by the retroperito- of ASPS reflects its rich hypervascularity. At CT,
neum, and are rarely seen in the peritoneal cavity it typically manifests as a well-defined lesion
of the abdomen (87). Undifferentiated pleomor- that is iso- to hypoattenuating compared with
phic sarcoma is a clinically aggressive tumor, with muscle, with marked hypervascularity with the
an estimated local recurrence rate of 19%–31%, administration of contrast material (Fig E9a; a
and metastases are seen in 31%–35% of cases full DICOM image stack is available online) (92).
(87). At T1-weighted MRI, they are usually isointense
compared with muscle, although they occasion-
Radiologic Features.—Undifferentiated pleomor- ally may show mild hyperintensity due to hemor-
phic sarcoma has a nonspecific imaging appear- rhage (92). A key imaging clue is the presence
ance and commonly manifests as a well-defined of flow voids at T1- and T2-weighted MRI (93).
heterogeneous mass with areas of avid contrast One recent study (93) reported that the presence
material enhancement at CT and MRI (Fig E8a) of greater than five peritumoral and intratumoral
(88). They usually show attenuation similar to that flow voids in a deep soft-tissue solid enhancing
of muscle at CT, intermediate signal intensity at lesion is diagnostic of ASPS, with sensitivity of
T1-weighted MRI, and signal hyperintensity at 96% and specificity of 91%. Central necrosis has
T2-weighted MRI (53). The presence of hemor- also been reported and is especially seen in larger
rhage can result in areas of signal hyperintensity lesions (93).
at T1-weighted MRI. Calcifications are seen in Occasionally, ASPS and other hypervascular
5%–20% of cases (53). tumors such as solitary fibrous tumors may be
confused with hemangiomas, given their intense
Pathologic and Molecular Features.—Undiffer- contrast material enhancement. Some of the
entiated pleomorphic sarcoma is a diagnosis made distinguishing clues that suggest hemangiomas
after the exclusion of differentiated pleomorphic include their lobulated appearance, high signal
sarcoma (eg, liposarcoma, leiomyosarcoma), and intensity at T2-weighted MRI, gradual pooling
thus, extensive tumor sampling is critical for accu- of contrast material, and the presence of intrale-
rate tumor classification (86). At gross pathologic sional fat and phleboliths, which may be calcified
examination, these tumors display heterogeneous at CT (92). Confirmation can be obtained with
firm to fleshy cut surfaces that commonly show tagged technetium 99m red blood cell scintig-
hemorrhage and/or necrosis (Fig E8b). Micro- raphy, if findings at cross-sectional imaging are
scopically, undifferentiated pleomorphic sarcomas indeterminate (94).
are hypercellular and are composed of markedly
atypical cells, without evidence of lineage-specific Pathologic and Molecular Features.—At gross
differentiation (Fig E8c) (28). pathologic examination, ASPS demonstrates
rubbery tan cut surfaces with hemorrhage and/or
Alveolar Soft-part Sarcoma necrosis (Fig E9b). At histologic evaluation, these
tumors display a nested architecture, with lobules
Epidemiologic Features.—Alveolar soft-part of tumor cells separated by delicate fibrovascular
sarcoma (ASPS) is a rare malignant soft-tissue septa (95). At cytologic evaluation, tumor cells
tumor that accounts for up to 1% of all soft-tissue are discohesive and polygonal, with prominent
sarcomas (89,90). The median age at diagnosis is nucleoli and an eosinophilic granular cytoplasm
22–26 years with a higher incidence in women, (Fig E9c). Most cases show intracytoplasmic crys-
especially in younger patients (89,90). ASPS most talline inclusions that can be highlighted by using
commonly occurs in the extremities in adults, periodic acid Schiff diastase stain (Fig E9d). ASPS
whereas the head and neck are the primary sites is characterized by t(X:17) translocation, resulting
in children (91). Approximately 3%–8% of ASPSs in fusion of the ASPSCR1 and TFE3 genes (95).
occur in the retroperitoneum (89,90). ASPS typi-
cally manifests as a painless slow-growing tumor. Cystic Tumors
Despite its early indolent course, many ASPSs Primary retroperitoneal cystic neoplasms are
metastasize later in the disease course. Portera et uncommon, with a reported incidence of one in
al (90) reported a metastatic rate of up to 65%. 100 000 in adults (96). They can be broadly cat-
The prognosis is highly dependent on the pres- egorized as cysts of epithelial origin (eg, primary
ence of metastatic disease (89). In one study (89), mucinous cystadeonoma), mesothelial origin (eg,
1652 October Special Issue 2020 radiographics.rsna.org

cystic mesothelioma), or germ cell origin (eg, cys- however, a well-defined muscle layer and nerve
tic teratoma) (26). Additional miscellaneous enti- plexus are characteristically absent (97).
ties include tailgut cysts, lymphatic malformations,
epidermoid cysts and pseudomyxoma retroperito- Lymphatic Malformation
nei (26). On rare occasions, solid retroperitoneal
masses (such as paragangliomas) may manifest as Epidemiologic and Clinical Features.—Lymphatic
cystic lesions (66). malformation (also known as cystic lymphan-
gioma), is a benign cystic mass comprising dilated
Tailgut Cyst lymphatic channels, which forms secondary to
failure of normal lymphatic channel communi-
Epidemiologic and Clinical Features.—Tailgut cation or drainage (100). They are considered a
cysts (also known as retrorectal cystic hamar- form of slow-flowing vascular malformation (101).
tomas) are uncommon congenital lesions that Lymphatic malformations can occur in patients of
arise from the embryonic hindgut (97). In one any age, but most of them manifest in early child-
literature review (97) of 53 cases of tailgut cysts, hood. They are most commonly found in the head
the average age of patients at presentation was 35 or neck and are rarely encountered in the abdo-
years (range, 4 days to 73 years), and they occur men (101). They can be asymptomatic or may
more often in women (3:1 female-to-male ratio). be associated with vague abdominal discomfort.
Approximately half of all lesions are detected Treatment typically includes percutaneous sclero-
incidentally in patients with reported symptoms therapy and/or surgery (102,103).
including low back or rectal pain, rectal fullness,
and pain during defecation (97). Surgical excision Radiologic Features.—Because most lymphatic
is usually recommended because of the risks of malformations manifest in patients during child-
malignant degeneration and infection associated hood, initial imaging assessment is often per-
with biopsy (98). Although malignant degenera- formed with US, which often shows a multilocu-
tion is uncommon, one literature review (98) lated anechoic mass with thin septa. Vascularity
spanning all reported cases from 1932 to 2011 can occasionally be seen in the septa (101). At
reported a malignancy rate of 14%, with some CT, lymphatic malformations often appear as a
subtypes including mucinous adenocarcinoma, uniformly cystic mass and exhibit attenuation that
carcinoid tumors, and squamous carcinoma. is near that of water. The walls and/or septa are
rarely perceptible, and septal enhancement is dif-
Radiologic Features.—Tailgut cysts are charac- ficult to appreciate at CT. They classically can in-
teristically located in the presacral or retrorectal volve more than one retroperitoneal compartment
space, with occasional extension into the ischio- and can appear as an elongated and insinuating
anal fossa (99). At CT, the mass typically mani- mass, which is an important imaging clue (100).
fests as a well-defined unilocular or multilocular Uncomplicated lymphatic malformations usually
hypoattenuating cystic lesion with variably thick appear as multilocular multiseptated fluid-filled
septa and occasional calcifications (26,99). The masses with classic fluid high signal intensity at
MRI appearance mirrors the CT findings, with T2-weighted MRI and enhancing thin septa with
the cystic content appearing T2 hyperintense and administration of contrast material (Fig 13a, 13b).
T1 hypointense (Fig 12a, 12b), although signal in- The presence of fluid-fluid levels at T2-weighted
tensity may be variable in the presence of internal MRI may indicate hemorrhage. Signal hyperin-
hemorrhagic and/or proteinaceous debris. If septa tensity at T1-weighted MRI can also be suggestive
are present, they typically show mild enhance- of the presence of proteinaceous or hemorrhagic
ment. Imaging findings that suggest superimposed debris secondary to superinfection or hemorrhage
infection or malignancy include indistinct mar- (102). One study (104) suggested that the detec-
gins, nodular wall thickening with enhancement, tion of intracystic lipid through chemical shift
intracystic enhancing nodules, superior extension MRI may be valuable for detection of lymphatic
above S3, and/or lymphadenopathy (99). malformations with chylous content.

Pathologic and Molecular Features.—Tailgut Pathologic and Molecular Features.—Lym-

cysts are typically thin-walled multiloculated cysts phatic malformations have a variable appearance
that contain clear serous or opaque mucoid fluid at gross pathologic evaluation. These lesions are
(Fig 12c). Histologically, tailgut cysts are lined by typically smooth-walled and uni- or multilocu-
gastrointestinal tract mucosa that is most com- lated and contain clear serous fluid (Fig 13c).
monly stratified squamous epithelium. (Fig 12d) Microscopic features include cystic walls com-
(97). The underlying fibroconnective tissue may posed of fibrous tissue and lined by a monolayer
contain disorganized smooth muscle bundles; of endothelial cells (Fig 13d).
RG • Volume 40 Number 6 Al-Dasuqi et al 1653

Figure 12. Tailgut cyst in a 51-year-old woman who presented with rectal discom-
fort. (a, b) Axial fat-suppressed T2-weighted (a) and sagittal fat-suppressed contrast-en-
hanced T1-weighted (b) MR images show a large cystic presacral mass without internal
nodularity or septa (*). (c) Photograph at gross pathologic examination shows a cyst
with a tan smooth glistening surface. (d) Medium-power photomicrograph shows a tail-
gut cyst lined by mucinous columnar epithelium (arrow) and containing serous luminal
fluid. (Hematoxylin-eosin stain; original magnification, 100.)

Algorithmic Approach liposarcomas do not metastasize, the presence of

After localizing the tumor to the retroperitoneum, distant disease is suggestive of a more aggressive
the radiologist should categorize the lesion as higher-grade tumor.
predominantly solid or cystic (Fig E1), with the If no macroscopic lipid is detected, the rela-
caveat that certain solid masses may have internal tionship of the lesion to adjacent vessels should
cystic components, which often represent necrosis be assessed. If there is evidence of vessel invasion,
and/or hemorrhage. If the mass is determined to then the possibility of leiomyosarcoma should
have a predominantly solid appearance, it should be strongly considered. However, because the
be scrupulously examined for the presence of majority of leiomyosarcomas are classified as
macroscopic lipid because it is highly suggestive of extravascular, vessel invasion is not always visible
liposarcoma. Primary retroperitoneal lipomas are radiologically. If no vessel invasion is seen, the
uncommon and are often evaluated as well-differ- predominant signal intensity of the mass at T2-
entiated liposarcomas, particularly in the presence weighted MRI should be assessed. The presence
of thickened septa and soft-tissue nodular compo- of a target sign of peripheral signal hyperintensity
nents larger than or equal to 1 cm. In rare cases, and central hypointensity at T2-weighted MRI is
a primary retroperitoneal teratoma manifests as suggestive of a neurogenic origin. A predominant
a fat- and calcium-containing mass, possibly with T2-hyperintense mass with brisk hypervascular
a fat-fluid level. Differentiating among the differ- enhancement can be seen in paragangliomas,
ent histologic categories of liposarcoma can be while a paucity of enhancement is suggestive of
challenging, and a paucity of macroscopic lipid is an underlying myxoid matrix that can be seen
often seen with myxoid and pleomorphic liposar- with a schwannoma, neurofibroma, myxoid
comas, with the former also exhibiting a myxoid liposarcoma with no detectable fat, or low-grade
matrix (T2-hyperintense components with a low myxofibrosarcoma. Signal hypointensity at T2-
level of enhancement). Because well-differentiated weighted MRI is uncommon but should raise
1654 October Special Issue 2020 radiographics.rsna.org

Figure 13. Lymphatic malformation in a 17-year-old adolescent girl with left-sided flank pain. (a, b) Axial T2-
weighted (a) and contrast-enhanced T1-weighted (b) images show a multiseptated cystic mass (*) with mild
septal enhancement (arrow in b). (c) Photograph at gross pathologic examination of the specimen reveals a
smooth-walled multiloculated cystic structure (*) filled with a small amount of clear serous fluid. No definitive ex-
crescences are identified, and minimal solid components are seen. (d) Low-power photomicrograph shows cystic
channels lined by a monolayer of endothelial cells (arrow), overlying fibrous tissue with lymphocytic infiltration (*).
(Hematoxylin-eosin stain; original magnification, 100.)

the possibility of a leiomyoma, particularly if the It is important to evaluate the abdominopelvic

patient is a woman with a history of fibroids. cavity carefully for any abnormalities outside of
Heterogeneous signal intensity at T2-weighted the retroperitoneum that may suggest an alternate
MRI is more common and can be seen with a diagnosis, specifically lymphoma or metastatic dis-
plethora of neoplasms that are difficult to confi- ease. Lymphoma is the most common retroperito-
dently distinguish on the basis of imaging alone. neal malignancy, accounting for up to one-third of
If the lesion is paravertebral, the possibility of a all cases (2). Retroperitoneal lymphoma is seen in
neurogenic tumor such as a ganglioneuroma or 25%–55% of cases of lymphoma, most commonly
ganglioneuroblastoma could be considered, with manifesting as well-defined para-aortic or pelvic
the former characterized by a whorled appear- masses (lymph nodes) with homogeneous en-
ance at T2-weighted MRI. hancement and no necrosis or calcification when
Cystic retroperitoneal neoplasms are challeng- imaged before treatment. The adenopathy may be
ing to distinguish on the basis of imaging alone. If discrete or may form a conglomerate nodal mass.
the mass occupies more than one retroperitoneal In some cases, lymphoma may appear infiltrative,
compartment and/or insinuates between struc- with a mantlelike growth pattern, typically around
tures without invading them, then the possibility the major vessels (52).
of a lymphatic malformation should be strongly If the patient has a history of primary malig-
considered. Cystic teratomas are rare and may nancy or if there are imaging findings suggestive of
manifest with a fat-fluid level. A tailgut cyst or a primary malignancy elsewhere (eg, a concurrent
retrorectal hamartoma should be included in the renal mass or the presence of skeletal metastatic
differential diagnosis of any cystic mass located in disease), retroperitoneal metastatic lymphadenopa-
the presacral space. thy should be strongly considered. This is typically
RG • Volume 40 Number 6 Al-Dasuqi et al 1655

seen in patients with renal cell carcinoma, cervical 11. Knebel C, Neumann J, Schwaiger BJ, et al. Differentiating
atypical lipomatous tumors from lipomas with magnetic
cancer, and prostate cancer (52). In addition, pa- resonance imaging: a comparison with MDM2 gene ampli-
tients with primary malignant testicular neoplasms fication status. BMC Cancer 2019;19(1):309.
can develop metastatic retroperitoneal disease, 12. Craig WD, Fanburg-Smith JC, Henry LR, Guerrero
R, Barton JH. Fat-containing lesions of the retroperito-
which sometimes occurs in the absence of an ap- neum: radiologic-pathologic correlation. RadioGraphics
parent gonadal primary tumor (105). 2009;29(1):261–290.
13. Gupta P, Potti TA, Wuertzer SD, Lenchik L, Pacholke
DA. Spectrum of Fat-containing Soft-Tissue Masses at
Conclusion MR Imaging: The Common, the Uncommon, the Char-
Primary retroperitoneal neoplasms are a rare and acteristic, and the Sometimes Confusing. RadioGraphics
eclectic group of tumors with a variety of imaging 2016;36(3):753–766.
14. Shaaban AM, Rezvani M, Tubay M, Elsayes KM, Woodward
features, some of which can be derived from their PJ, Menias CO. Fat-containing Retroperitoneal Lesions:
histologic appearance. Incorporating a systematic Imaging Characteristics, Localization, and Differential
approach to these neoplasms, taking into account Diagnosis. RadioGraphics 2016;36(3):710–734.
15. Singer S, Antonescu CR, Riedel E, Brennan MF. Histologic
epidemiologic and clinical features, allows the subtype and margin of resection predict pattern of recur-
radiologist to provide consistently high-quality rence and survival for retroperitoneal liposarcoma. Ann Surg
accurate interpretations for referring providers. 2003;238(3):358–370; discussion 370–371.
16. Kransdorf MJ. Malignant soft-tissue tumors in a large
Accurate interpretation is critical because it al- referral population: distribution of diagnoses by age, sex,
lows the radiologist to help direct the next step and location. AJR Am J Roentgenol 1995;164(1):129–134.
in managing patient care, whether it is conserva- 17. Goldblum JR, Folpe AL, Weiss SW, Enzinger FM, Weiss
SW. Enzinger and Weiss’s soft tissue tumors. 6th ed. Phila-
tive or surgical, and can help to guide referring delphia, Pa: Saunders/Elsevier, 2014.
providers before potential biopsy (ie, to sample 18. Johnson CN, Ha AS, Chen E, Davidson D. Lipo-
a higher-grade component of the neoplasm). An matous Soft-tissue Tumors. J Am Acad Orthop Surg
2018;26(22):779–788.
understanding of the expected gross pathologic 19. Dalal KM, Kattan MW, Antonescu CR, Brennan MF, Singer
and microscopic appearance of these lesions can S. Subtype specific prognostic nomogram for patients with
help facilitate these radiologic interpretations. primary liposarcoma of the retroperitoneum, extremity, or
trunk. Ann Surg 2006;244(3):381–391.
20. Dalal KM, Antonescu CR, Singer S. Diagnosis and
Acknowledgment.—The authors would like to thank Mark management of lipomatous tumors. J Surg Oncol
Saba, MA, senior designer and illustrator, User Experience 2008;97(4):298–313.
and Design Services, Yale University, Information Technology 21. Antonescu CR, Tschernyavsky SJ, Decuseara R, et al. Prog-
Services, for creating the illustrations in Figure 1. nostic impact of P53 status, TLS-CHOP fusion transcript
structure, and histological grade in myxoid liposarcoma:
a molecular and clinicopathologic study of 82 cases. Clin
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