Prayson Tumors Flashcards
Low grade astrocytoma
WHO II. 90% derived from fibrillary astrocytes. Frontral lobe> parietal > temporal > occipital. Most common in 4th decade (30s). 5-10 year survival after surgery
Anaplastic astrocytoma
WHO III. Fourth and fith decades (30-40s),
Small cell GBM
WHO IV. EGFR amplificiation
Glioblastoma
WHO IV. Most common glioma, 5th and 6th decade (40-50s), M>F. Risks: hydrocarbon exposure and radiation. Giant cell variant may have slightly better prognosis. Survival without therapy 14-16 weeks, w/ therapy 1 year. Metasis rare (#1 lung, #2 bone)
Gliosarcoma
WHO IV. Temporal lobe is most common site, increased mets (sarcoma part). Sarcoma may be fibrosarcoma, fibrous histiocytoma, rarely chondrosarc, rhabdo, or angio.
Pilocytic astrocytoma
WHO I. Most common glioma in children, assoc w/ NF1 (especially brainstem and optic nerve). Cystic mass with enhancing mural nodule. 5yr survival 85%. Infiltration of meninges does not affect prognosis
Subependymal giant cell astrocytoma
WHO I. Increased intracranial pressure 2/2 obstruction of foramen of Monro. Assoc w/ tuberous sclerosis, usually <20 years. Elevated nodule involving lateral or 3rd ventricle. Good prognosis, low recurrence rate
Pleomorphic xanthoastocytoma
WHO II. Most common in first 2 decades (<20), siezures. Assoc w/ cortical malformations. Temporal and parietal lobes, cystic w/ mural nodule, superficial nodule in contact w/ leptomeninges. Well circumscribed. Perivascular lymphs, lipidized astrocytes, reticulin rich,
Anaplastic: increased mitosis and necrosis.
Malignant transformation or recurrence in 15-20%
Optic nerve glioma
First decade, NF1 assoc- bilateral. adults-ant. optic pathway aggressive. Growth within optic sheath-
Astroblastoma
Children and young adults, cerebral hemispheres, discrete circumscribed mass. Perivascular rosettes formed by elongated cells.
Desmoplastic Infantile astrocytoma/ganglioglioma
WHO I. Superficial frequently dural based. Most <1 year of age, M>F. Desmoplasia with spindled astrocytes, derived from subpial astrocytes. Cellular foci of small blue cell with high proliferation do not alter prognosis. Increased head circumfrence with bulging fontanelles. Cystic, GFAP +
Chrodiod glioma
WHO II. Anterior 3rd ventricle, F>M. Obstructive hydrocephalus. Solid (soft and mucoid tumor) composed of clusters or cord of epitheliod cells with variably mucinous stroma, stromal lymphoplasmacytic infiltrate and numeorus russell bodies. GFAP+ Rare, slow growing, recur if incompletely excised
Ganglioglioma
WHO I. Glioneuronal tumor. Most common in temporal lobe and children > adults. Most pt.s present w/chronic epilepsy. Cystic tumor w/ calcified mural nodule. Atypical neurons, calcifications are common, granular bodies, perivascular lymphs, Assoc w/ cortical malformations. Rare anaplastic WHO grade III, w/ increased mitosis and necrosis
Gangliocytoma
WHO I. Composed of mature ganglion cells
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos)
Cowden’s synd (PTEN germline mutation). Hamartomatous lesion of ganglion cells
Glioneuronal tumors
Ganglioglioma, papillary glioneuronal tumor, rosette-forming glioneuronal turmor of the 4th ventricle (all WHO I)
Angiocentric glioma
WHO I. Children/young adults, epilepsy assoc. Angiocentric growth of monomorphos biopolar cells and features of ependymal differentation. rare slow growning
Oligodendroglioma
WHO II. Mean age 35-45, white matter origin, frontal lobe most common site. chicken wire vasculature, calcifications prominent at the periphery, may have meningeal invasion, minigemistocytes-degenerative change.
GFAP - or focally + (minigemistocytes +), S100, Leu-7+
1p/19q codeletion/ IDH mutant, TERT mutant
Survival 10-15 years
Anaplastic- WHO III, >5 mits/10 HPF
Dysembryoplastic neuroepithelial tumor (DNET)
WHO I. Young pts w/ hx of chronic epilepsy. Multinodular, multicystic, cortical based. Assoc w/ cortical dysplasia. Microcystic, floating neurons
Central neurocytoma
WHO II. 20-40 years, Intraventricular near septum pellucidum or in 3rd ventricle (rare extraventricular). descrete, often partly calcified, uniform nuclie with speckled chromatin, fibrillary background, mitosis rare.
GFAP -, synapto, NSE, and B-tubulin +
EM: Dense core granules, neurotubules, and neurofilaments
Flexner-Winersteiner Rosettes
Small lumen, cuboidal lining. True Rosette
Retinoblastoma
Ependymal Rosettes
Ependymal lining, ciliary attachment (blephroplasts), canal/chanel with long lumen. True rosette
Homer Wright
Fibrillary core, neuroblastic differentation. Psuedorosette
Medulloblastoma, neuroblastoma
Ependymoma
WHO II. More common in children, Most common tumor of the spinal cord, ventricle (especially 4th). 71% are infratentorial. Well demarcated, soft, lobular mass. Perivascular pseudorosettes and true ependymal rosettes, marked fibrillarity. Can have calcs/ossificatin / cartilage. Obstruction of CSF is presenting symptom.
Variants (no clinical significance): Cellular, papillary, clear cell, tanycytic, melanotic, lipomatous, giant cell, signet ring
GFAP, S100, EMA (dots and rings)+ CD99 highlights surface membranes in intracytoplasmic lumina.
NF2 mutations common in spinal tumors, Ch 22q loss and 1q gains are common
Extent of resection (best predictor) and location and are most prognostic, better prognosis in adults and spinal and supratentorial tumors.
Anapastic- WHO III, Increased mits, necrosis, etc
Subependymoma
WHO I. Adults M>F, asymptomatic, septum pellucidum, floor, lateral recess of 4th ventricle, no rosettes or pseudorosettes, microcystic change
GFAP and S100+,
Myxopapillary ependymoma
WHO I. M>F, 30-40 years, may be locally infiltrative, hemorrhagic or lobular. 25% encapsulated. Filum terminale > subcu sacrococcygeal region or other spinal cord location. Intracellular mucin, mucoid stroma.
GFAP, mucicarmine, and alcian blue +
Meningioma
WHO I. Middle aged and elderly, F>M, Assoc w/ NF2, breast CA, prior radiation. 13-26% of primary intracranial tumors. Falx cerebri is most common site of origin, most arise from arachnoid cap cells proximal to the meninges. Can cause hyperostosis of bone
Secretory- PAS+ eosinophilic intracellular lumina
Metaplastic-bone, cartilage, fat
EMA, Somatostatin receptor 2a, vimentin+, PR> ER, S100 and CK are varaibly +
Deletion of Ch 22q is most common cytogentic finding
Atypical meningioma
WHO II. Chordoid, Clear cell (glycogen rich cytoplasm).
>/= 4 mits/10 HPFs, brain invasion, or 3 of the following: hypercellularity, small cell change, promient nucleoli, loss of architecture, necrosis.
M>F, 5-7% of meningiomas, more likely to recur
Anaplastic menigioma
WHO III. Papillary (perivascular psuedopapillary pattern) , rhabdoid (eccentric nuclei, prominent nucleoli, and cytoplasmic inclusions of intermediate filaments).
>/= 20 mits/ 10 HPFs; median survival <2 years, can met
Hemangiopericytoma
WHO I-III. M>F, 40-50 yoa, usually dural based, supratentorial (10% spinal). Staghorn vessels, reticulin rich, can cause lytic destruction of bone (not hyperostosis), rare adipocytic component
STAT6 (NAB2-STAT6 fusion), CD34, vimentin+, EMA -
WHO I- benign, hypocellular, collagenized, classic SFT phenotype
WHO II- malignant, <5 mits/10 HPF, hemangiopericytoma pattern
WHO III (Anaplastic)- malignant, >/= 5mits/10 HPF, hemangiopericytoma pattern
Mitotic count is best indicator of outcome
Chordoma
Derived from notochordal tissue remnants (ecchordosis physaliphora). Most common sites saccrococcygeal > clivus. Bone based tumor, more common in adults. Epitheloid cells and physaliphorous cells in a mucoid matrix, can contain cartilage (chondroid chordoma)
Vimentin, CK, EMA, S100, brachyurea +
Locally recurr, occasionally met (lungs, lymph nodes, skin), younger age and gross total resection do better
Melanocytoma
Any age (peak in 40s), low mitotic activity, minimal necrosis, usually abundant melanin. may recur locally
Diffuse melanocytosis
Children, leptomeningeal proliferation of nevoid cells, Assoc w/ neurocutaneous melanosis synd. poor prognosis
Primary meningeal melanoma
Mostly adults (very rare). Cytologic atypia, high mitotic index, necrosis, hemorrhage. S100, HMB45, Melan-A+, CK and GFAP-. poor prognosis
Hemangioblastoma
WHO I. Young adults, Cerebellum most common, brain stem and spinal cord. Cyst w/ enhancing mural nodule. Assoc w/ VHL (Ch 3p) and 10% w/ secondary polycythemia vera (produces erythropoietin). Adjacent piloid gliosis w/ rosenthal fibers. Mast cells
Chroid plexus papilloma
WHO I. 1st 2 decades. Lateral ventricle is most common in kids, 4th vent is most common in adults. May contain metaplastic elements, oncocytic change or mucinous degen.
Assoc w/ Li-Fraumeni, SV40
Choroid plexus carcinoma
WHO III. 80% occur in children. grossly invasive, solid, necrotic, atypia, mitoses. 5 yr survival is 40%
Embryonal tumor with multilayered rosettes (ETMR)
WHO IV. Children <4, most commonly occur in the cerebral hemispheres (frontal and parietotemporal) Frequent multi-layered rosettes and mitoses
Includes embryonal tumor with abundant neuropil and true rosettes ependymoblastoma and medulloepithelioma.
Amplification and fusions of the C19MC locus on Ch19
Medulloblatoma
WHO IV. 70% occur before 16 years of age, most before 10. M>F, most arise in vermis and project into 4th ventricle. Ataxia, gait disturbance, HA, vomiting. Homer-wright pseuodorosettes (<40% of cases). Synapto +, variably GFAP+
Histologic variants: Classic, nodular-desmoplastic (good prognosis, tends to be hemispheric and arise in older kids and adults), extensive nodularity, large cell/anaplastic
Genetic variants: WNT (good prognosis), SHH +/- P53 (-p53 good/standard risk, +p53 poor prognosis), Group 3/4 (standard to poor prognsis)
Isochromosome 17q in 50%
Atypical teratoid/rhabdoid tumor (AT/RT)
WHO IV. Infants to age 2, slightly more common in males. 50% occur in post. fossa. 1/3 present w/ mets. Cytoloplasmic eosinophilic inclusion composed of intermediate filaments, rhabdoid cells are EMA and vimentin +, variable Neurofilament, GFAP, SMA and CK. INI-1 lost (monosomy or deletion on Ch 22).
Most pts die within 1 year
Schwannoma
Assoc w/ NF2. S100+, GFAP-, NF2 gene in 60% of sporadic
Plexiform- NF2
Melanotic- assoc w/ Carney’s complex (AD, cardiac myxoma, pigmented skin lesions, and endocrine abnormalities)
Neurofibroma
Assoc. w/ NF1. Painful. Composed of schwann cells, perineurial cells, and fibroblasts. Wagner-Meissner corpuscles. Scattered S100+
Malignant peripheral nerve sheath tumor
~50% arise in NF-1. Usually arise from a neurofibroma. Hypercellularity, necrosis, increased mitoses, frequently invasive. Epithelioid, glandular and triton (rhabdomyosarcomatous diff). 5 yr survival 35%
Perineurioma
adolescents and young adults. Intraneural tumors w/ segmental nerve enlargement. Proliferation of perineural cells in endonerium around nerve (pseudo-onion bulbs). EMA +
Mortons Neuroma
thickening and degenration of the interdigital nerve of foot
Lymphoma
50-60s, M>F, Assoc w/ AIDs. 60% supratentorial, frontal lobe most common. 25-50% are multiple (60-85% in AIDs and post transplant pts). Usually DLBCL, non-germinal center (Activated B-cell) type. EBV in immunocompromised.
Langerhan’s Cell histiocytosis
Children, Yellow or white dural nodules or granular parenchymal infiltrate, osteolytic bone changes. Atypical histocytes: CD1a and S100 +, Birbeck granules (tennis rackets), eosinophils, plasma cells
Hand-schiller- Christian dx: involvement of brain and bone
Rosai-Dorfman
Adults, dural based. Sheets or nodules of histiocytes (CD1a-) assoc w/ emperipoiesis
Colloid cyst
Third ventricle near the foramen of Monro; young to middle aged adults. HA, transient paralysis of lower extremities, incontinence, personality changes. Single layer of columnar epithelium, may contain goblet and ciliated cells. Amorphous brightly eosinophilic cyst fluid.
EMA, CEA, CK, S100+
Rathke cleft cyst
Intra or suprasellar. Ciliated columnar epithelium, possible squamous metaplasia. CK8 and CK20+ (cranios are negative for both of these)
Endodermal cyst
Many are intraspinal, aka enterogenous, neruentric, enterogenic
Choroid plexus cyst
epithelial lined cyst of choroid plexus, tranthyretin +
Arachnoid cyst
Temporal lobe subarachnoid space most common. EMA+
Epidermoid cyst
CP angle is most common
Dermoid cyst
Posterior fossa, midline, 4th ventricle
Paraganglioma
WHO I. Spinal intradural turmos in Cauda equina is most common. Intracranial tumors usually represent extension of jugulotympanic tumors. 40-50 years, M>F. Assoc. w/ VHL and MEN type IIa and IIb.
Cheif cells: Synapto, chromo and NF+; Sustentacular cells: S100+
Pineocytoma
WHO I. Adolescents and adults. Lobular arrangement of small cells with largely fibrillar hypocellular zones- pineocytomatous rosettes.
Synapto, NSE and NF+
Pineal Parenchymal tumor of intermediate differentiation
WHO II-III.
Low grade <6 mits/10 HPF and expression of NF.
Pineoblastoma
WHO IV. Diffuse growth of small blue cells w/ occasional homer wright and Flexner-wintersteiner rosettes (no pineocytomatous rosettes). Necrosis and high miotic index.
Germ cell tumors
Midline, usually suprasellar and pineal. Children and young adults (before 20). Percocious puberity, papilledema, gaze palsies. Diffuse dissemination occasionaly seen. Germinoma is most common.
PLAP, Oct 3/4, a-fetoprotein (yolk sac), CD30 (embryonal), HCG (chorio)
Adamantinomatous craniopharyngioma
Beta-catenin (CTNNB1 mutation 95%), claudin 1, CK5/6, CK7+. Rare malignant transformation to SCC after radiotherapy. Bimodal age distribution children (5-15 yrs) and adults (45-60 yrs). Motor oil
Papillary craniopharyngioma
BRAF V600E (>95%). Exclusively in adults (40-55 yrs). Well differentiated squamous epithelium without surface keratinization.
Pituitary adenoma
10-20% of all intracranial neoplasms. Adults, F>M, Most common is prolactinoma.
Assoc w/ MEN1
Mets
Most common CNS tumor, Lung, melanoma, breast, RCC, and GI. 70% are multifocal, present at gray-white jxn
Lipoma
Midline: corpus callosum, 3rd ventricle, CP angle, quadrigeminal area. Can be assoc w/ agensis of the corpus callosum.
Pilomyxoid astrocytoma
WHO grade II. Variant of pilocytic astrocytoma. Rosenthal fibers and eosinophilic granules are not found in pilomyxoid astrocytoma. usually hypothalamic, cerebellum is uncommon.
Assoc w/ NF1
