Tumors (Martin) Flashcards
Intracranial tumors typically arise where in the kids?
posterior fossa; infratentorial
Intracranial tumors typically arise where in adults?
cerebral hemispheres; supratentorial
If an adult presents with new onset seizures what should you suspect?
a brain tumor
What are the 4 histological parameters of gliomas?
Nuclear atypia
Mitosis
Microvascular proliferation
Necrosis
Astrocytomas are usually what type of lesions?
White matter lesions
The two (MAIN) type of astrocyte tumors?
Astrocytomas
in adults - infiltrating astrocytoma
in kids - pilocytic astrocytoma
Infiltrating astrocytoma
grade II to IV; mostly adults (80%); presents with seizures, headaches and focal neurological deficits;
IDH-mutant is good prognosis
IDH-wild type is a bad prognosis
confirmatory test with IDH1 R132H mutant protein (90%)
Which marker in infiltrating astrocytoma indicative of a bad prognosis?
IDH-wild type
What is the confirmatory test for infiltrating astrocytoma?
IDH1 R132H mutant protein (90%)
Pilocytic astrocytoma
well circumscribed cystic with a mural nodule tumor of astrocytes in kids located in the cerebellum; IAA 1549 BRAF gene; resection is the only treatment
Characteristics of a pilocytic astrocytoma?
has a biphasic pattern; hair-like cells with bipolar processes; Rosenthal fibers and will be GFAP+
Name of tumor that has a biphasic pattern; hair-like cells with bipolar processes; Rosenthal fibers and will be GFAP+?
pilocytic astrocytoma
What is the treatment for pilocytic astrocytoma?
resection only
What is the most common primary brain neoplasm?
Glioblastoma (GBM)
What are the two types of glioblastomas (GBM)?
Primary - older patients; IDH - wild type and TERT & EGFR mutations
Secondary - younger patients; IDH1 and TP53 mutation (R132H had better prognosis than IDH - wild type)
Tumor that appears to have hypodense central necrosis on imaging?
Glioblastomas (GBM)
What are the 3 histological hallmarks of glioblastomas?
- Necrosis - serpentine pattern of necrosis
- Pseudo-palisading
- Vascular/endothelial proliferation
A tumor with these 3 histological hallmarks:
1. Necrosis - serpentine pattern of necrosis
2. Pseudo-palisading
3. Vascular/endothelial proliferation
Glioblastomas (GBM)
Tumor of oligodendrocytes?
Oligodendroglioma
Oligodendroglioma
white matter tumor in older patients; neurological complaints for several years; will see calcifications, “fried eggs” and “chicken wire”
IDH1 & IDH2 - favorable prognosis (90%)
1P19Q loss - favorable prognosis
anaplastic - poor prognosis
What are the genetics associated with favorable prognosis in Oligodendrogliomas?
IDH1 & IDH2 - favorable prognosis (90%)
1P19Q loss - favorable prognosis
What is indicative of a poor prognosis in Oligodendrogliomas?
anaplastic - poor prognosis
Tumor that has shows calcifications, “fried eggs” and “chicken wire”?
Oligodendroglioma
Tumor of ependymal cells?
Ependymoma; seen in kids and discretely around the 4th ventricle
Ependymoma
Tumor of ependymal cells seen in kids and discretely around the 4th ventricle; will see ependymal rosettes (true rosettes - more diagnostic); there are 3 types:
1. Anaplastic
2. Mxyopapillary
3. Subependymoma
What is very diagnostic of an ependymoma?
true rosettes - tubular structure with central canal; more diagnostic than perivascular rosettes
What are the 3 types of ependymomas?
- Anaplastic (less common)
- Mxyopapillary - film terminale
- Subependymoma - lateral or 4th ventricle (causes obstructive hydrocephalus)
Which type of ependymoma is typically known to cause obstructing hydrocephalus?
Subependymoma - because it is located around the lateral or 4th ventricle - can cause obstructive hydrocephalus
Which type of ependhymoma is typically known to case lower back pain?
Mxyopapillary - because it is located near the film terminal of the spinal cord
What are the two types of choroid plexus tumors?
- Choroid plexus papilloma - children in lateral ventricles
- Colloid cyst - young adults attached to roof of 3rd ventricle (obstruct foramen of mono)
Gangliogliomas typically affects which part of the cerebral?
temporal lobes; mixed with mature neuronal and glial cells; present as seizures and medical refractory epilepsy
What is the most common pediatric brain tumor?
Medulloblastoma
What are the 4 types of medulloblastoma?
- WNT (BEST prognosis) - older kids with chromosome 6 B-catenin mutation (100% 5 yr survival)
- SHH - infants and young adults with MYCN mutation
- Group 3 (WORST prognosis) - MYC and 17(I17q) mutation
- Group 4 I17q with NO MYC mutation - poor prognosis but better than group 3
Which type of medulloblastoma has the worst prognosis and what’s its genetics?
Group 3 (WORST prognosis) - MYC and 17(I17q) mutation
Which type of medulloblastoma has the best prognosis and what’s its genetics?
WNT (BEST prognosis) - older kids with chromosome 6 B-catenin mutation; 100% 5 yr survival
Hallmarks of medulloblastomas
located at the midline (cerebellum) and will be “drop Metz” throughout the CSF because it is gray friable (tends to fall apart); will also see “cell wrapping”
Which intracranial tumors are located at the midline?
medulloblastoma (cerebellum) and germ cell tumors (pineal)
Atypical teratoid/Rhabdoid tumors
located in the posterior fossa; kids under 3 yrs (HIGHLY aggressive - survival <1 yr); will have +EMA and Vimentin and “rhabdoid cells
Primary CNS lymphoma
seen in immunosuppressed (AIDs) individuals or as a latent infection with EBV; all in B-cell origin so CD20+; will see “hooping”
What is the most common type of pineal tumor?
Germinoma; male predominance; seen in the first two decades of life and seen in Japanese patients (10%)
Tumor of the meninges?
Meningioma
Meningioma
benign tumor of the meninges that compresses the brain (does NOT invade brain parenchyma) and has the potential to penetrate the bone; NF2 mutation on chromosome 22q22; can see calcified psammoma bodies; can be seen in pregnancy and regress after delivery
Which tumor has been seen to produce symptoms during pregnancy and then have a relief of symptoms after delivery?
Meningioma
Mutation of Meningiomas?
NF2 mutation on chromosome 22q22
Neuroblastoma
common extracranial solid tumor of childhood (18 months of age); arise in the adrenal medulla produce catecholamines - “blueberry muffin baby”; presents with large abdominal masses, fever, and possible weight loss; spreads via blood to liver, lungs, bone marrow and bones (also brain)
Which childhood tumor is associated with “blueberry muffin baby”?
Neuroblastoma; arise in the adrenal medulla produce catecholamines
Which two paraneoplastic syndromes can contribute to limbic encephalitis?
- small cell carcinoma of the lung - ANNA-1 antibody
- Ovarian teratoma - NMDA receptor
Tuberous Sclerosis Complex
one of the familial tumor syndromes; can see “candle gutterings” - wax-like masses building into the ventricles; and cutaneous lesions - angiofibromas and subungal fibromas
Which one of the drugs from Segars AED lecture is used for Tuberous Sclerosis Complex?
Cannabidiol (Epidiolex)
Von Hippel-Lindau Disease
a familial tumor syndrome; AD inheritance causes POLYCYTHEMIA (down regulation of HIF1 which regulates VEGF); hemangioblastomas of the CNS; cysts in the pancreas,liver and kidneys, renal cell carcinoma and pheochromocytoma
Main symptom of Von Hippel-Lindau Disease?
POLYCYTHEMIA (down regulation of HIF1 which regulates VEGF)
What are the 3 peripheral nerve sheath tumors?
- Schwannoma - NF2 mut; causes acoustic neuroma CN VIII; does NOT invade nerve
- Neurofibroma - NF-1 mut; does invade nerve
- MPNST - malignant - NF-1 mut
