Nervous System Neoplasms Flashcards
Tumor Grading Features
Grade I, Grade II (2) Grade III (2) Grade IV (3)
Benign
Infiltrative, atypia
Anaplasia, Mitoses
Microvascular infiltration, necrosis, fatal
Pilocytic Astrocytoma
Grade, Locations (2) Genetics, Morphology (5)
Grade I
Cerebrum white matter
Cerebellum
NF1 Defect causing Neurofibromin LOF
(neurofibromatosis)
Cystic with mural nodule Biphasic pattern Hairlike cells with bipolar processes Rosenthal fibers Eosinophilic granular bodies
Glioblastoma
Grade, Location, Primary (3) vs Secondary (2) Morphology (4) Histology (3)
Grade IV
All throughout brain
Primary:
No precursor
EGFR and PTEN mutations
Secondary:
IDH1/IDH2 mutation
Better prognosis
GFAP (+)
Contrast ring enhancing lesion
Hypodense central necrosis
Black thrombosed veins
Serpentine Necrosis
Pseudo-Palisading
Vascular/Endothelial proliferation
Oligodendroglioma
Location, Morphology, Histology (2) Genetics (3)
Cerebral hemispheres white matter
Cortex Calcification
Perinuclear halos (fried egg nucleus) Anastomosing capillaries (chicken wire)
IDH1
IDH2
1p19q loss
(favorable outcomes)
Ependymoma
Ages, Grade, Locations (2), Morphology (3)
First two decades of life
Grade III
4th ventricle (enhancing) Supratentorial
Ependymal Rosettes
Tubular structures with central canal
Fibrillary background
Ventricular Tumors
Choroid Plexus and Colloid Cyst
Age group, Location, Complication
Choroid Plexus Papilloma
Seen in Children
Lateral Ventricles
Obstructive Hydrocephalus
Colloid Cyst Seen in young adults 3rd Ventricle Non-communicating Hydrocephalus (via Foramen of Monro obstruction)
Medulloblastoma
Grade, Age, Locations (2) Genetics (2) Morphology (3) Treatment, Spread Pattern
Grade IV
Children
Cerebellum
Midline cerebrum
*worst prognosis
MYC amplification
I17Q mutation
Sheets of anaplastic cells
Abundant mitoses
Homer-Wright Rosettes
Extremely sensitive to radiation therapy
Drop Metastases (CSF to cauda equina)
Atypical Teratoid/Rhabdoid Tumor
Grade, Age, Locations (2) Morphology (3) Genetics (2) Prognosis
Grade IV
Children under 2 years
Posterior Fossa
Supratentorial
EMA (+)
Vimentin (+)
Rhabdoid cells
Chr 22 hSNF5/INI1
Highly aggressive, fatal within a year
Primary CNS Lymphoma Disease Associations (2) Origin, Morphology, Staining
HIV
EBV
CD20+ B cells
Cells accumulate around vessels
Hooping: cells separated by reticulin and silver stain
Germ Cell Tumors
Locations (3)
Midline Cerebrum
Pineal gland
Suprasellar
Pineal Tumors
Examples (3) with Descriptions (1/2/3)
Germinoma: most common
Pineocytoma: low grade, in adults
Pineoblastoma: high grade, in kids, RB gene
Meningioma
Location, Genetics (2) Histology (2) Complication
Dura mater
NF2
TRAF7
Whorled cluster of monotonous cells
Psammoma bodies
Present progesterone receptor, danger of preterm pregnancy
Metastatic Brain Tumors Origin Sites (4) Location, Morphology, Complications (3)
Lung
Breast
Melanoma
Choriocarcinoma
Meninges are common site
Cribiform glands with necrosis
Melanoma and Choriocarcinoma metastases tend to hemorrhage
Spinal cord compression
Carcinomatosis
Subacute Cerebellar Degeneration
Etiology, Associated Carcinomas (3)
Treatment (2)
*Paraneoplastic syndrome
PCA-1 antibody
Ovarian
Breast
Uterine
Immunotherapy to remove antibodies
Removal of tumor
Limbic Encephalitis
Etiologies with Associated Cancers (3)
Treatment (2)
*Paraneoplastic syndrome
ANNA-1 antibody: Small cell carcinoma of lung
NMDA receptor: ovarian teratomas
VGKC-Complex Antibody causes peripheral neuropathy
Immunotherapy to remove antibodies
Removal of tumor
Cowden Syndrome
Gene and Associated Tumor
PTEN mutation
Dysplastic gangliocytoma
Tuberous Sclerosis Complex
Genes (2), Tumors (6) Symptoms (3)
TSC1
TSC2
Cortical Hamartomas
Subependymal nodules
Subependymal giant cell astrocytomas
Retinal Hamartomas
Renal Angiomyolipomas
Cardiac Rhabomyomas
Seizures
Autism
Mental Retardation
Von Hippel Lindau Disease
Pathogenesis (3) Clinical Features (5)
VHL mutation downregulates HIF-1 which incrreases VEGF
Polycythemia Hemangioblastomas of Cerebellum and Retina Liver Cysts Renal Cell carcinoma Pheochromocytoma
Li-Fraumeni Syndrome
Gene and Associated Tumor
TP53
Medulloblastomas
Turcot Syndrome
Gene and Associated Tumors (2)
APC gene
Medulloblastoma
Glioblastoma
Gorlin Syndrome
Gene and Associated Tumor
PTCH gene
Medulloblastoma
Neurofibromatosis Type 2
Gene, Tumors (4) Schwannoma Morphology (2)
NF2 loss (MERLIN)
Cerebellar pontine angle (schwannoma)
Acoustic neuroma (schwannoma)
Meningiomas
Ependymomas
Antoni A: Verocay bodies (palisading nuclei)
Antoni B: hypocellular, myxoid ECM
Neurofibromatosis Type 1
Gene, Tumors (4), Non-Tumor Presentations (2)
NF1
Plexiform Neurofibromas
Diffuse Neurofibromas (subcutaneous)
Localizing cutaneous neurofibromas
Optic Nerve gliomas
Lisch nodules on iris
Cafe au lait spots (hyperpigmented near mouth)
Malignant Peripheral Neural Sheath Tumor
Grade, Etiology, Morphology, Subtype with Description
High grade tumors
Malignant transofrmation of plexiform neurofibromas in NF1
Marbleized tumors
Divergent differentiation
Poorly differentiated
Triton Tumor: Rhabdomyoblastic morphology