Intrathoracic Giant Solitary Fibrous Tumor: Case Report

Clinics of Surgery
Case Report Volume 4
ISSN 2638-1451
Intrathoracic Giant Solitary Fibrous Tumor: Case Report
Mindaye ET1*
, Tesfaye GK2
and Aboye AG3
1
Department of surgery, Saint Paul’s Hospital Millennium Medical College, Swaziland Street 1271 Addis Ababa, Ethiopia
2
Department of surgery, Saint Paul’s Hospital Millennium Medical College, Addis Ababa, Ethiopia
3
Department of Radiology, Addis Ababa University, Addis Ababa, Ethiopia
*Corresponding author:
Esubalew Taddese Mindaye,
Department of surgery, Saint Paul’s Hospital
Millennium Medical College,
Swaziland Street 1271 Addis Ababa,
Ethiopia, E-mail: esubetad24@yahoo.com
Received: 07 Dec 2020
Accepted: 22 Dec 2020
Published: 26 Dec 2020
Copyright:
©2020 Mindaye ET et al. This is an open access article dis-
tributed under the terms of the Creative Commons Attribu-
tion License, which permits unrestricted use, distribution,
and build upon your work non-commercially.
Citation:
Mindaye ET, Intrathoracic Giant Solitary Fibrous Tumor:
Case Report. Clinics of Surgery. 2020; 4(3): 1-4.
Keywords:
Solitary Fibrous Tumor; Diaphragm, Pleura; Case report
Abbreviations:
CD: Cluster of Differentiation; CT: Computerized
Tomography; MRI: Magnetic Resonance Imag-
ing; SPHMMC – St. Paul’s Hospital Millennium
Medical College; SFTP –Solitary Fibrous Tumor
of the Pleura
1. Abstract
1.1. Introduction: Solitary Fibrous Tumor of the Pleura(SFTP)
is exceedingly rare mesenchymal tumor commonly arising from
the visceral pleura and accounts for < 5% of all pleural tumors.
Although it commonly has benign histologic characteristics, the
tumor behavior is often unpredictable and less understood due to
limited number of cases.
1.2. Case Presentation: We present a rare case of Intrathoracic
Giant SFTP in a 65 years old female who presented with a progres-
sive worsening of shortness of breath of 1year duration associated
with intermittent dry cough, low grade fever, easy fatigability and
loss of appetite. Complete enbloc resection was done and she was
discharged improved.
1.3. Discussion: Most patients with SFTP are asymptomatic and
definitive diagnosis is often made after surgical exploration and
histopathologic study. SFTP should be considered as differential
diagnosis in patients with atypical or recurrent respiratory symp-
toms despite adequate medical treatment. Complete surgical exci-
sion is the main stay of treatment and meticulous post-operative
follow up is mandatory as the risk of recurrence is higher and the
tumor behavior is still less understood.
2. Introduction
Solitary Fibrous Tumor of the Pleura (SFTP) is exceedingly rare
mesenchymal tumor and accounts for < 5% of all pleural tumors
[1, 2]. Majority of SFTP are pedunculated with benign histologic
characteristics and about 800 cases have been reported in litera-
tures [3, 4]. It most commonly arises from visceral pleura and, the
tumor’s behavior is often unpredictable and doesn’t always cor-
relate with the histologic findings and mandates long term follow
up [3, 5]. Due to rarity of the tumor, complete understanding of
its pathogenic factors, clinical features, imaging characteristics,
management options and prognostic analysis are very limited [6].
Most patient are asymptomatic especially those with small tumor;
however, patients may present with respiratory symptoms [1]. Pre-
operative diagnosis is usually challenging and definitive diagnosis
is often made after operative exploration [7].
Although Chest Computerized Tomography(CT), Magnetic Res-
onance Imaging(MRI) and Doppler Ultrasound have lower speci-
ficity, they provide important clue to identify the tumor, it’s local
extent and invasion of adjacent organ, which is crucial in guiding
surgery [7].
Complete Surgical resection is the main stay of treatment for SFTP
clinicsofsurgery.com 1

[5]. The case report has been reported in line with the SCARE
2018 criteria [8].
3. Case Presentation
Sixty-five years old female presented with a complaint of progres-
sive worsening of shortness of breath of 1year duration associated
with intermittent dry cough, low grade fever, easy fatigability and
loss of appetite. For these complaints she visited different hospi-
tals and was treated for severe pneumonia with intravenous an-
tibiotics and supported with oxygen. But her shortness of breath
progressed, saturation of oxygen deteriorated and she became de-
pendent on intranasal oxygen support. For this reason, she was re-
ferred to our tertiary care center, Saint Paul’s Hospital Millennium
Medical College, Addis Ababa, Ethiopia. She had no contact his-
tory with known pulmonary tuberculosis patients and have never
smoked cigarettes. she has no history of drug allergy, self or family
history of relevant medical or surgical illness. On presentation her
oxygen saturation was 86 % with 3 liters of intranasal oxygen and
respiratory rate was 28-34 breath/mint. She was emaciated and had
digital clubbing. There was absent air entry over her left anterior
and posterior lower two third lung field. Otherwise, there was no
remarkable finding on other systems.
Her hemoglobin was 12.6gm/dl and serum albumin was 3.4gm/
dl. Other parameters of complete blood count, renal function test
and serum electrolytes were in the normal range. Chest computer-
ized tomography (Chest CT) showed 20 cm x 13 cm x 15cm huge
heterogeneously enhancing, lobulated, left intrathoracic mass that
seem to arise from the left diaphragmatic pleura (Figure 1). It has
compressed the left basal lung and thoracic aorta. Ultrasound guid-
ed tissue biopsy from the mass showed proliferative oval to spin-
dle cells with mild to moderate nuclear pleomorphism in short and
long fascicles with diagnostic impression of benign spindle cell
lesion likely solitary fibrous tumor. Abdominal ultrasound didn’t
reveal any evidence of secondary.
Figure 1: Chest CT scan showing huge heterogeneously enhancing left
intrathoracic mass compressing the thoracic aorta and left lung
Figure 2: Intra operative picture showing huge left intrathoracic mass
compressing the left lung
Figure 3: Post-operative picture showing 20 cm x 15cm firm mass weigh-
ing 3kg.
With an impression of left intrathoracic mass likely arising from
the left diaphragm the surgical team decided to do surgical ex-
ploration and the patient was operated through left posterolateral
thoracotomy after getting informed written consent. The intraoper-
ative finding was a 20cm x 15 cm huge, highly vascular, firm, pe-
dunculated left intra thoracic mass arising from the left diaphrag-
matic pleura with about 4cm stalk (Figure 2 and 3). The left lung
was collapsed and had extensive adhesion with the mass. There
was no mediastinal lymphadenopathy. With these findings en-bloc
complete excision of the mass with the diaphragmatic stack was
done and the diaphragmatic defect was closed in 2 layers (Figure
4). Left tube thoracotomy was left and thoracotomy wound was
closed 2 in layers.
Figure 4: Intra operative picture showing diaphragmatic defect closed in
2 layers
Volume 4 Issue 3-2020 Case Report

Post procedure, she was transferred to intensive care unit and put
on oxygen support as well as epidural analgesics. Subsequently,
she had smooth recovery and was transferred to surgical ward. The
chest tube output was insignificant and minor bubbling decreased
gradually. Control chest x ray taken on her 4th post op day showed
well expanded left lung with no evidence of pneumothorax for
which the chest tube was removed.
Tissue section of the mass showed lobulated, yellow-white appear-
ance (Figure 5) and histopathologic study showed proliferative
spindle to ovoid cells with ovoid nucleus and scanty cytoplasm in
a collagenous stroma (Figure 6). There was no evidence of mitosis
and necrosis the final diagnosis being Giant solitary fibrous tumor
of the pleura.
Subsequently, the patient showed a remarkable improvement and
was discharged from the hospital in a stable condition.
Figure 5: post-operative tissue cut section showing lobulated, yel-
low-white mass
Figure 6: Histologic section showing proliferative spindle to ovoid cells
with ovoid nucleus and scanty cytoplasm
4. Discussion
Solitary fibrous tumor is slow growing, rare, mesenchymal tumor
accounting for < 2% of all soft tissue tumors [9]. About 50-70%
of them are extra pleural involving the abdomen, pelvis, head and
neck [10]. SFTP spreads locally to the chest wall, axilla and su-
praclavicular area, and distant metastasis to the liver, bone, brain
and adrenal gland is unusual [2]. Hilar and mediastinal lymphatic
metastasis occurs in <50% of patients [2]. Our patient has no im-
aging and intraoperative evidence of metastasis. When SFTP arise
from the diaphragmatic pleura both the diaphragm and peritoneum
may be involved [2] and we have resected part of the diaphragm
attached to the stalk of the mass. Although 80% of SFTP arise
from visceral pleura [2], the origin in our case was from the pari-
etal pleura which is rare. About 12% of SFTP are malignant and
are characterized by their larger size usually>8cm, arise in atypi-
cal locations like parietal and mediastinal pleura, and histological
features of increased cellularity, pleomorphism and >4 mitosis/10
HPF [11]. In this regard, although our patient’s tumor arises from
the parietal pleura and have larger size the histological features go
for benign SFTP. Benign SFTP may rapidly enlarge and transform
into the malignant form [11]. For this reason, complete resection
of all SFTP is mandatory.
Most SFTP are asymptomatic being diagnosed incidentally in 50%
of cases [11] and those who are symptomatic are usually malig-
nant and larger in size [4]. Symptoms include cough, chest pain,
dyspnea, digital clubbing and clinical findings of pleural effusion.
Rarely, SFTP may produce insulin-like growth factor 2 and cause
refractory hypoglycemia (Doege-Potter syndrome) [4]. Although
our patient was being treated for pneumonia which could be ex-
plained by obstructive pneumonitis in patients with SFTP, there
was delay to reach at diagnosis of SFTP as its rarity hinders it from
being considered into the differential diagnosis.
Although no specific computed tomographic features have been
described, SFTP typically appear as well-defined, enhancing, het-
erogeneous or homogenous mass in contact with the pleural sur-
face [4]. Like our case, definitive diagnosis is often made after sur-
gical exploration and histopathologic study. Although we couldn’t
do immunohistochemistry study in our case, SFTP are positive for
vimentine and CD34, and are negative for keratin [7].
The main stay of treatment for all SFTP is complete enbloc surgi-
cal excision with 2 cm tumor free margin [4]. Post-operative risk
of recurrence is higher especially for malignant, sessile SFTP and
usually occurs within the first 2years of follow up [4]. Although
neo-adjuvant and adjuvant treatment with chemotherapy and ra-
diotherapy is being used sporadically, its benefit is still unproved
because of limited number of patients [4]. In this regard, our pa-
tient was not initiated on adjuvant treatment. We are following
her with clinical evaluation and chest x-ray every 3 months, and
have chest CT scan annually. She has marked improvement of her
symptoms and is also happy with her treatment.
5. Conclusion
Although SFTP is rare mesenchymal tumor, its behavior is un-
predictable. Patients have no specific clinical presentation and are
usually misdiagnosed. So, SFTP should be considered as differ-

ential diagnosis in patients with atypical or recurrent respiratory
symptoms despite adequate medical treatment. Complete surgical
excision is the main stay of treatment and meticulous post-opera-
tive follow up is mandatory as the risk of recurrence is higher and
the tumor behavior is still less understood.
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