Robbins 27-29 - CNS Tumors + Eye Flashcards
2 categories of astrocytomas
- Infiltrating
2. Pilocytic
40-60
Mass lesion in CNS
Seizures, headaches, focal neurologic signs
Grade 2/4
Diffuse infiltrating astrocytoma
40-60
Mass lesion in CNS
Seizures, headaches, focal neurologic signs
Grade 3/4
Anaplastic astrocytoma
40-60
Mass lesion in CNS
Seizures, headaches, focal neurologic signs
Grade 4/4
Glioblastoma
PTEN mutation
EGFR amplification
Classic primary glioblastoma
TP53 mutation
PDGFRA overexpression
Proneural secondary glioblastoma
NEFL, GABRA1, SYT1 expression
Neural glioblastoma
NF1 deletion
Mesenchymal glioblastoma
Child tumors
- Low grade astrocytoma (pilocytic) (1/4)
- Ependymoma
- Medulloblastoma
Adult tumors
- Glioblastoma
- Meningioma
- Low grade astrocytoma/glioma (2/4)
Young
Radiation, family history, NF, Li-Fraumeni
Seizure, headache, AMS
Posterior fossa, ocular or ataxia symptoms
Pilocytic astrocytoma
Older
Radiation, family history, NF, Li-Fraumeni
Seizure, headache, AMS
Progressive, necrosis, cross midline
Glioblastoma
Adult
Frontal lobe
Seizure, headache, increased ICP, ataxia
Halo (fried egg), spherical, calcification
Oligodendroglioma
Child
Fourth ventricle, hydrocephalus, papilledema
Headache, ataxia
Pseudovascular perirosettes
Ependymoma
Adult
Spinal cord
Cauda equina syndrome (bowel/bladder/sexual)
NF-2
Ependymoma
Adult female, NF-2
Seizure, headache, ACM
Meningioma
Child Cerebellum/posterior fossa, midline Hydrocephalus, ataxia, visual symptoms Li-Fraumeni syndrome Radiosensitive
Medulloblastoma
NF-2, hearing problems, vestibular problems
Brainstem
Schwannoma
Heterogeneous cell types
NF-1
Large peripheral nerve or nerve roots
Neurofibroma
NF-1
Large peripheral nerves (chest, abdomen, pelvis, neck, limbs)
Metastatic/progressive
Poorly differentiated, multiple cell types
MPNST
Cowden Syndrome
Gangliogliocytoma
PTEN mutation
Li-Fraumeni syndrome
Medulloblastoma
TP53 mutation
Turcot syndrome
Medulloblastoma, glioblastoma
APC mutation
Gorlin syndrome
Medulloblastoma
PTCH mutation
Tuberous sclerosis complex
Hamartomas, sub-ependymal giant-cell astrocytomas
Von Hippel-Lindau Disease
Hemangioblastomas of CNS (cerebellum and retina)
Cysts of pancreas, liver, kidneys
Renal cell carcinoma
Pheochromocytoma
Dysregulation of EPO –> polycythemia
Neurofibromatosis 1
Neurofibromas of peripheral nerves MPNSTs Gliomas of optic nerve Hamatromas Pheochromocytomas
Neurofibromatosis 2
Bilateral schwannomas of CN VIII
Meningiomas
Ependymomas (of spinal cord)
Tinnitus, hearing loss
NF2 mutation
Well-circumscribed, encapsulated mass along nerve
Schwannoma
Pedunculated nodules on skin
NF-1
Superficial cutaneous neurofibroma
Plaque-like elevation of skin
NF-1
Diffuse neurofibroma
IDH1, IDH2 mutations Calcification Spherical nuclei Clear halo of vacuolated cytoplasm Network of anastamosing capillaries
Oligodendroglioma
Mixture of neuronal and glial cells Seizures Temporal lobe Cystic component Random orientation of neurites, binucleate forms
Ganglioglioma
Superficial temporal lobe
Attenuation of overlying skull
Grade I/IV
Dysembryoplastic neuroepithelial tumor
Young child (
Atypical teratoid/rhabdoid tumor
Immunosuppressed Elderly Multiple foci within parenchyma (gray and white) Spread via ventricles Extensive necrosis CD19/CD20 positive
Primary CNS lymphoma (B-cell)
Japan, child male
Primary brain germ cell tumor
CSF AFP and b-HCG
Germinoma (metastatic germ cell tumor)
Metastatic carcinomas to CNS
Common metastatic site?
Lung, breast, melanoma, kidney, GI
Meninges
PCA-1 antibody (Anti-Yo)
Cerebellar degeneration
Neoplastic ovarian, uterine, or breast carcinoma
ANNA-1 antibody (Anti-Hu)
Neoplastic small cell lung carcinoma
Inflammatory enlargement of lacrimal gland
Sarcoidosis
Inferior, medial proptosis
Sarcoidosis (lacrimal gland)
Lymphoma, pleomorphic adenoma, adenoid cystic carcinoma (neoplasms)
Axial proptosis
Glioma, meningioma (tumor of optic nerve)
Predisposing factors to sinus infection spread to orbit
Immunosuppression
Diabetic ketoacidosis
Periocular ecchymosis + orbital neoplasm
Metastatic neuroblastoma
Wilms tumor
Eyelid neoplasm
Basal cell carcinoma
Redness and itching of conjunctiva
Significan conjunctival scarring
Infection
Chlamydia trachomatis (trachoma)
Submucosal elevation of conjunctiva in sun-exposed regions
Pinguecula or pterygium
Saucer-like depression in corneal tissue (delle)
Pinguecula
Fair-skinned person, middle age
Unilateral conjunctival neoplasm carcinoma in situ
Melanoma in situ
Conjunctival differentiation into squamous epithelium and goblet cells
Mucoepidermoid carcinoma
Risk factors for cataracts
Galactosemia, DM, Wilson’s disease, atopic dermatitis
Corticosteroids, radiation, trauma
Most common primary intraocular malignancy of adults
Uveal melanoma
Thickening of BM of epithelium of pars plicata
DM marker
Cherry-red spot
Central retinal artery occlusion
Most common primary intraocular malignancy of children
Retinoblastoma
Unilateral papilledema
Primary neoplasm of optic nerve
Bilateral papilledema
Increased CSF pressure