Tumors of the Nervous System part 2 Flashcards
What age group do Embryonal tumors usually affect?
KIDS
What is the most common type of Embryonal tumor in kids?
Medulloblastoma
What grade is a Medulloblastoma and where does it usually sit anatomically?
Grade 4
= Sits midline cerebellum and occludes CSF flow
There are 4 unique subtypes of Medulloblastomas based on molecular changes. List them and denote which has the best/worst prognosis.
- WNT chr.6/beta catenin = best prognosis
- SHH
- Group 3 MYC and 17(I17Q) = worst prognosis
- Group 4 MYCN and I17Q
There are 4 unique subtypes of Medulloblastomas based on molecular changes. List them and denote which has the best/worst prognosis.
- WNT chr.6/beta catenin = best prognosis
- SHH
- Group 3 MYC 17(I17Q) = worst prognosis
- Group 4 MYCN I17Q
Where can Medulloblastomas spread?
Drop metz
= Disseminates via CSF to cauda equine
What is a good feature about treating Medulloblastomas?
Exquisitely Radiosensitive!
Medulloblastomas involve anaplastic cells, abundant mitoses and what type of Rosettes?
Homer-Wright Rosettes
Another type of Embyronal tumor is an Atypical Teratoid/Rhabdoid Tumor (AT/RT). What is its grade, location and chromosome mutation?
Grade 4
Posterior fossa
Chromosome 22
What cells are present and what color is the cytoplasm with an Atypical Teratoid/Rhabdoid Tumor (AT/RT)?
Rhabdoid cells with eosinophilic cytoplasm
What 2 molecules are present with an Atypical Teratoid/Rhabdoid Tumor?
EMA
Vimentin
What patients often get Primary CNS Lymphomas?
AIDs patients
– Latent EBV infection
What cell origin are Primary CNS Lymphomas?
B cell origin (CD20)
Where do Primary CNS Lymphomas accumulate and what word describes their cells?
Accumulate around vessels
– hooping
Where do Germ Cell Tumors occur?
Along MIDLINE
What causes a Meningioma?
Radiation induced
How do Meningiomas look and what do they do anatomically?
Bosselated (large bumps) dural mass –> compress brain and penetrate bone
What tumor is a dural mass that compresses brain and penetrates bone often from radiation?
Meningioma
What gene mutation is often associated with a Meningioma?
NF2 – higher grade with multiple tumors
What histo manifestations do Meningiomas have? (2)
- Calcified Psammoma bodies
2. Whorled clusters of monotonous cells
What histo manifestations do Meningiomas have? (2)
- Calcified Psammoma bodies
2. Whorled clusters of monotonous cells
What receptor do Meningiomas express which causes them to grow rapidly during pregnancy?
Progesterone receptors
Metastatic Brain Tumors are usually from what 4 sources?
Lung
Breast
Melanoma
Choriocarcinoma
Where do Metastatic Brain Tumors go?
Meninges or intraparenchymal (gray-white junction)
What can be symptoms of a Metastatic Brain Tumor?
Hemorrhage
Meningeal Carcinomatosis = neuropathies from nerve compression
What are 3 common Familial Tumor Syndromes?
Von Hippel Lindau
Neurofibromatosis 1 and 2
The Familial Tumor Syndromes in this deck are inherited how?
Autosomal Dominant
What 4 things will present with Von Hippel Lindau Syndrome?
Hemangioblastoma of CNS
Renal cell carcinoma
Polycythemia
Pheochromocytoma
What 4 things will present with Von Hippel Lindau Syndrome?
Hemangioblastoma of CNS
Polycythemia
Renal Cell Carcinoma
Pheochromocytoma
Neurofibromatosis 1 mutation
NF1 - 17Q11 = Neurofibromin
What 3 things will present with Neurofibromatosis 1?
Neurofibromas
Eye lesions
Cafe au lait spots
What 3 things will present with Neurofibromatosis 1?
Neurofibromas
Eye lesions
Cafe au lait spots
Neurofibromatosis 2 mutation
NF2 - 22Q12 = Merlin
What will present with Neurofibromatosis 2?
Bilateral Schwannomas => CN8 affected
What will present with Neurofibromatosis 2?
Bilateral Schwannomas => CN8
