Oligodendroglioma Clinical Pathways v2 2024
Oligodendroglioma Clinical Pathways v2 2024
Oligodendroglioma
October 2024 – V2.2024
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Table of Contents
Presumptive Conditions.…………………………………………....…….………………………………………………………………………………………………………..……......…... 3
Molecular Testing…………....……………………………………………………………………………………..…..……………………………….……………………………….………... 6
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Oligodendroglioma – Presumptive Conditions
VA automatically presumes that certain disabilities were caused by military service. This is because of the unique circumstances
of a specific Veteran’s military service. If a presumed condition is diagnosed in a Veteran within a certain group, they can be
awarded disability compensation.
If the patient served on or after Sept. 11, 2001, in Afghanistan, Djibouti, Egypt, Jordan, Lebanon, Syria, Uzbekistan, or Yemen or
if you served in the *Southwest Asia theater of operations, or Somalia, on or after Aug. 2, 1990, specific conditions include:
• Brain Cancer
* The Southwest Asia theater of operations refers to Iraq, Kuwait, Saudi Arabia, the neutral zone between Iraq and Saudi Arabia,
Bahrain, Qatar, the United Arab Emirates, Oman, the Gulf of Aden, the Gulf of Oman, the Persian Gulf, the Arabian Sea, the Red
Sea, and the airspace above these locations.
For more information, please visit U.S. Department of Veterans Affairs - Presumptive Disability Benefits (va.gov)
3
Oligodendroglioma – Grade 2
No Biopsy
Clinical trial(s) always considered on pathway. For assistance finding a clinical trial, email [email protected].
a
Refer to Neuro-oncology via National TeleOncology consult if local VA Neuro-oncology unavailable
b
High Risk Features to include >40 years old and residual tumor, neurologic symptoms to include uncontrolled seizures, or atypical neuroimaging to include contrast enhancement; take into account high risk features as determined by Neuropathologist
c
MRI Surveillance at least every 4 months for first 5 years, every 6 months for years 5-10, at least annually >10 years
d
Multidisciplinary Discussion through local tumor board or National TeleOncology CNS Virtual Tumor Board
e
Imaging MRI with and without contrast; CT with contrast if MRI contraindicated
f
Pseudo Progression can occur inside the radiation field (defined as within the 80% isodose line) up to 12 weeks post-radiation
g
Adjuvant Therapy PC cycles repeat every 6 weeks; Cycle 1- CCNU (Lomustine) 90mg/m2 orally day 1 and procarbazine 60mg/m2 orally days 8-21 of a 42 day cycle; Cycle 2- CCNU 100mg/m2 orally day 1 (if no myelosuppression) and procarbazine 60mg/m2 orally days 8-21; Cycles 3-6- CCNU
110mg/m2 orally day 1 (if no myelosuppression) and procarbazine 60mg/m2 orally days 8-21; ondansetron 8mg orally prior to each dose of CCNU; CBC prior to day 1 of each cycle; if low tolerance anticipated use temozolomide 150mg/m2 days 1-5 of a 28-day cycle for cycle 1, then (if no
myelosuppression) increase dose to 200mg/m2 days 1-5 of a 28-day cycle for subsequent cycles up to cycle 12 with a CBC between day 22 and day 28; ondansetron 8mg daily before each temozolomide dose
h
PC is the procarbazine and CCNU of PCV; vincristine is omitted due to the lack of efficacy and increased toxicity
CCNU Lomustine
KPS Karnofsky Performance Status
PC Procarbazine and CCNU
4
Oligodendroglioma – Grade 3
Clinical trial(s) always considered on pathway. For assistance finding a clinical trial, email [email protected].
a
Imaging MRI with and without contrast; CT with contrast if MRI contraindicated
b
Refer to Neuro-oncology via National TeleOncology consult if local VA Neuro-oncology unavailable
c
Refer to Genetic Counseling for patient with personal history of other primary cancers particularly if diagnosed < 45 years or first or second degree relative with cancer < 55 years, clinician discretion for those >55 years
d
Life expectancy months refer to Hospice
e
Multidisciplinary Discussion through local tumor board or National TeleOncology CNS Virtual Tumor Board
f
Pseudo Progression can occur inside the radiation field (defined as within the 80% isodose line) up to 12 weeks post-radiation
g
Adjuvant Therapy PC cycles repeat every 6 weeks; Cycle 1- CCNU (Lomustine) 90mg/m2 orally day 1 and procarbazine 60mg/m2 orally days 8-21 of a 42 day cycle; Cycle 2- CCNU 100mg/m2 orally day 1 (if no myelosuppression)
and procarbazine 60mg/m2 orally days 8-21; Cycles 3-6- CCNU 110mg/m2 orally day 1 (if no myelosuppression) and procarbazine 60mg/m2 orally days 8-21; ondansetron 8mg orally prior to each dose of CCNU; CBC prior to day 1 of
each cycle; if low tolerance anticipated use temozolomide 150mg/m2 days 1-5 of a 28-day cycle for cycle 1, then (if no myelosuppression) increase dose to 200mg/m2 days 1-5 of a 28-day cycle for subsequent cycles up to cycle 12
with a CBC between day 22 and day 28; ondansetron 8mg daily before each temozolomide dose
h
PC is the procarbazine and CCNU of PCV; vincristine is omitted due to the lack of efficacy and increased toxicity
i
MRI Surveillance at least every 4 months for first 5 years, every 6 months for years 5-10, at least annually >10 years
CCNU Lomustine
KPS Karnofsky Performance Status
PC Procarbazine and CCNU
5
Oligodendroglioma – Molecular Testing
Evaluation by
Neuropathologist
IDH wildtype Order CGP using DNA and RNA (for both DNA and RNA fusion coverage),
and ATRX lost and methylation testing for possible HGAP or other rare high-grade gliomas
Yes
Microvascular
Age >55 non-midline, non-posterior fossa, and Perform IDH and IDH wildtype by molecular and [TERT mutation or EGFR
proliferation or Glioblastoma
no history of previous low grade glioma ATRX test by IHC amplification or gain of chromosome 7 with loss of chromosome 10]
necrosis?
Age and unusual Molecular testing for IDH1, IDH2, and TERT mutations, IDH wildtype and none Refer to Neuropathology and order CGP using DNA
Molecular Testing a Evaluation by IDH wildtype by IHC IDH mutation?
characteristics? and FISH for EGFR and chromosomes 7 and 10 of the above and RNA (for both DNA and RNA fusion coverage)
Neuropathologist
Age <55, midline tumor, circumscribed glioma, Order CGP using DNA and RNA IDH mutant by Follow the IDH Mutant
posterior fossa location, or unusual histology (for both DNA and RNA fusion coverage) molecular testing pathway
CDKN2A or CDKN2B
Astrocytoma WHO grade 4
homozygous deletion
IDH mutant FISH for CDKN2A and
No Type of deletion?
Clinical trial(s) always considered on pathway. For assistance finding a clinical trial, email [email protected]. and ATRX lost CDKN2B
a
All Initial Diagnoses should go through Molecular Testing pathway; recurrent tumors should have neuropathology review CDKN2A and CDKN2B Evaluation by
Astrocytoma
intact or single copy deletion Neuropathologist for grading
ATRX Alpha Thalassemia X-linked Intellectual Disability
CGP Comprehensive Genomic Profiling
IDH Isocitrate Dehydrogenase CDKN2A or CDKN2B Astrocytoma WHO
IHC Immunohistochemistry homozygous deletion grade 4 IDH mutant
MGMT-methylation Methyltransferase Methylation
CDKN2A and
WHO World Health Organization Intact
CDKN2B status?
CDKN2A and CDKN2B intact or Evaluation by
Astrocytoma
single copy deletion Neuropathologist for grading
IDH mutant and
FISH for 1p/19q co-deletion and CDKN2A/CDKN2B loss 1p/19q co-deleted?
ATRX retained
CDKN2A or CDKN2B Oligodendroglioma
homozygous deletion WHO grade 3
CDKN2A and
Co-deleted
CDKN2B status?
CDKN2A and CDKN2B intact or Evaluation by
Oligodendroglioma
single copy deletion Neuropathologist for grading
6
Oligodendroglioma – Molecular Testing Table
NPOP
Eligibility Test Category Test Type Recommended Vendors Specimen Type
Coverage
Age > 55,
Non-midline, IDH1 R132 mutation
IHC Local VA or locally contracted vendor No Tumor Tissue
Non-posterior fossa, AND ATRX
No history of previous low grade glioma
1p/19q FISH for codeletion
IDH-mutated FISH Local VA or locally contracted vendor No Tumor Tissue
CDKN2A/B homozygous loss
Molecular TERT promoter mutation testing Local VA or locally contracted vendor No Tumor Tissue
EGFR for amplification
**FISH Local VA or locally contracted vendor No Tumor Tissue
Chromosomes 7 and 10 for gain 7/loss 10
IDH-wild type
Chromosomal microarray (aka Oncoscan
**Microarray Local VA or locally contracted vendor No Tumor Tissue
FFPE)
Methylation Testing MGMT promoter methylation testing Local VA or locally contracted vendor No Tumor Tissue
Age < 55,
Midline tumor,
Circumscribed glioma,
DNA and RNA-based comprehensive
Posterior fossa location, Somatic NGS Tempus Yes Tumor Tissue
genomic profiling (CGP)
Unusual histology,
IDH-wildtype WITH loss of ATRX, OR
IDH-wildtype WITHOUT glioblastoma-defining molecular alterations
* Testing should not be ordered indiscriminately on every case as it will inappropriately exhaust tissue. Instead, follow the pathological workup recommended on the Molecular Testing page for appropriate test utilization
** Choose FISH OR microarray. In most cases FISH can be done instead of microarray. If performing microarray, then FISH is not indicated.