Cancer Flashcards
Where does medulloblastoma metastasize?
Leptomeningeal spaces - patchy CNS involvement typically causes multifocal, asymmetric neurologic signs and symptoms
Bilateral vestibular schwannomas are associated with…
Neurofibromatosis type II
Von Hippel Lindau triad of tumors
CNS and retinal hemangioblastoma
Pheochromocytoma
RCC (clear cell)
ADPKD CNS involvement
Aneurysms
What sellar pathology calcifies vs not?
Craniopharyngiomas
Pituitary adenomas and Rathke cleft cysts do not calcify
Tuberous sclerosis complex
Autosomal dominant neurocutaneous disorder - benign hamartomas
Seizures
Subependymal nodules
Cortical glioneuronal hamartomas (tubers)
Cardiac rhabdomyoma
Ash leaf spots (hypopigmented macules)
Angiofibromas
Shagreen patches (thick orange peel skin)
Astrocytoma prognosis is most affected by what?
Tumor grade (anaplasia, necrosis, neovascularity, etc.), not vascular invasion
Pseudopalisading pleomorphic tumor cells bordering central areas of necrosis, hemorrhage, or microvascular proliferation
Astrocytoma
“Fried egg” cells - round nuclei with clear cytoplasm
Oligodendroglioma - often in frontal lobes, commonly calcified
Spindle cells concentrically in whorled pattern, as well as psammoma bodies (laminated calcifications)
Meningioma - also note dural tails (origin is arachnoid cell)
Closely arranged, thin-walled capillaries and minimal intervening parenchyma
Hemangioblastoma - often cerebellar; associated with VHL syndrome when found with retinal involvement
S-100+ tumor
Vestibular schwannoma (acoustic neuroma) - Schwann cell origin
Tuberous sclerosis - treatment for infantile spasm
Vigabatrin - enhances GABA transmission
ACTH is alternative/adjunctive
Tuberous sclerosis - treatment for seizures
Standard antiepileptics
mTOR inhibitors if above failed
Sirolimus may slow disease progression
Sturge-Weber syndrome - genetics
Somatic mosaic mutation in GNAQ gene; therefore, not inherited
Belzutifan
HIF-2alpha inhibitor used in unresectable VHL tumors
Why does ataxia-telangiectasis predispose to infection?
IgA deficiency
Which of the neurocutaneous disorders is autosomal recessive inheritance?
Ataxia-telangiectasia; the others (besides Sturge-Weber) are autosomal dominant
