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CNS > Prayson Tumors > Flashcards

Prayson Tumors Flashcards

1
Q

Low grade astrocytoma

A

WHO II. 90% derived from fibrillary astrocytes. Frontral lobe> parietal > temporal > occipital. Most common in 4th decade (30s). 5-10 year survival after surgery

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2
Q

Anaplastic astrocytoma

A

WHO III. Fourth and fith decades (30-40s),

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3
Q

Small cell GBM

A

WHO IV. EGFR amplificiation

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4
Q

Glioblastoma

A

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)

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5
Q

Gliosarcoma

A

WHO IV. Temporal lobe is most common site, increased mets (sarcoma part). Sarcoma may be fibrosarcoma, fibrous histiocytoma, rarely chondrosarc, rhabdo, or angio.

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6
Q

Pilocytic astrocytoma

A

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

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7
Q

Subependymal giant cell astrocytoma

A

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

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8
Q

Pleomorphic xanthoastocytoma

A

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%

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9
Q

Optic nerve glioma

A

First decade, NF1 assoc- bilateral. adults-ant. optic pathway aggressive. Growth within optic sheath-

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10
Q

Astroblastoma

A

Children and young adults, cerebral hemispheres, discrete circumscribed mass. Perivascular rosettes formed by elongated cells.

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11
Q

Desmoplastic Infantile astrocytoma/ganglioglioma

A

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 +

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12
Q

Chrodiod glioma

A

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

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13
Q

Ganglioglioma

A

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

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14
Q

Gangliocytoma

A

WHO I. Composed of mature ganglion cells

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15
Q

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos)

A

Cowden’s synd (PTEN germline mutation). Hamartomatous lesion of ganglion cells

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16
Q

Glioneuronal tumors

A

Ganglioglioma, papillary glioneuronal tumor, rosette-forming glioneuronal turmor of the 4th ventricle (all WHO I)

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17
Q

Angiocentric glioma

A

WHO I. Children/young adults, epilepsy assoc. Angiocentric growth of monomorphos biopolar cells and features of ependymal differentation. rare slow growning

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18
Q

Oligodendroglioma

A

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

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19
Q

Dysembryoplastic neuroepithelial tumor (DNET)

A

WHO I. Young pts w/ hx of chronic epilepsy. Multinodular, multicystic, cortical based. Assoc w/ cortical dysplasia. Microcystic, floating neurons

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20
Q

Central neurocytoma

A

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

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21
Q

Flexner-Winersteiner Rosettes

A

Small lumen, cuboidal lining. True Rosette

Retinoblastoma

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22
Q

Ependymal Rosettes

A

Ependymal lining, ciliary attachment (blephroplasts), canal/chanel with long lumen. True rosette

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23
Q

Homer Wright

A

Fibrillary core, neuroblastic differentation. Psuedorosette

Medulloblastoma, neuroblastoma

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24
Q

Ependymoma

A

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

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25
Q

Subependymoma

A

WHO I. Adults M>F, asymptomatic, septum pellucidum, floor, lateral recess of 4th ventricle, no rosettes or pseudorosettes, microcystic change
GFAP and S100+,

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26
Q

Myxopapillary ependymoma

A

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 +

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27
Q

Meningioma

A

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

28
Q

Atypical meningioma

A

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

29
Q

Anaplastic menigioma

A

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

30
Q

Hemangiopericytoma

A

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

31
Q

Chordoma

A

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

32
Q

Melanocytoma

A

Any age (peak in 40s), low mitotic activity, minimal necrosis, usually abundant melanin. may recur locally

33
Q

Diffuse melanocytosis

A

Children, leptomeningeal proliferation of nevoid cells, Assoc w/ neurocutaneous melanosis synd. poor prognosis

34
Q

Primary meningeal melanoma

A

Mostly adults (very rare). Cytologic atypia, high mitotic index, necrosis, hemorrhage. S100, HMB45, Melan-A+, CK and GFAP-. poor prognosis

35
Q

Hemangioblastoma

A

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

36
Q

Chroid plexus papilloma

A

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

37
Q

Choroid plexus carcinoma

A

WHO III. 80% occur in children. grossly invasive, solid, necrotic, atypia, mitoses. 5 yr survival is 40%

38
Q

Embryonal tumor with multilayered rosettes (ETMR)

A

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

39
Q

Medulloblatoma

A

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%

40
Q

Atypical teratoid/rhabdoid tumor (AT/RT)

A

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

41
Q

Schwannoma

A

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)

42
Q

Neurofibroma

A

Assoc. w/ NF1. Painful. Composed of schwann cells, perineurial cells, and fibroblasts. Wagner-Meissner corpuscles. Scattered S100+

43
Q

Malignant peripheral nerve sheath tumor

A

~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%

44
Q

Perineurioma

A

adolescents and young adults. Intraneural tumors w/ segmental nerve enlargement. Proliferation of perineural cells in endonerium around nerve (pseudo-onion bulbs). EMA +

45
Q

Mortons Neuroma

A

thickening and degenration of the interdigital nerve of foot

46
Q

Lymphoma

A

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.

47
Q

Langerhan’s Cell histiocytosis

A

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

48
Q

Rosai-Dorfman

A

Adults, dural based. Sheets or nodules of histiocytes (CD1a-) assoc w/ emperipoiesis

49
Q

Colloid cyst

A

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+

50
Q

Rathke cleft cyst

A

Intra or suprasellar. Ciliated columnar epithelium, possible squamous metaplasia. CK8 and CK20+ (cranios are negative for both of these)

51
Q

Endodermal cyst

A

Many are intraspinal, aka enterogenous, neruentric, enterogenic

52
Q

Choroid plexus cyst

A

epithelial lined cyst of choroid plexus, tranthyretin +

53
Q

Arachnoid cyst

A

Temporal lobe subarachnoid space most common. EMA+

54
Q

Epidermoid cyst

A

CP angle is most common

55
Q

Dermoid cyst

A

Posterior fossa, midline, 4th ventricle

56
Q

Paraganglioma

A

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+

57
Q

Pineocytoma

A

WHO I. Adolescents and adults. Lobular arrangement of small cells with largely fibrillar hypocellular zones- pineocytomatous rosettes.
Synapto, NSE and NF+

58
Q

Pineal Parenchymal tumor of intermediate differentiation

A

WHO II-III.

Low grade <6 mits/10 HPF and expression of NF.

59
Q

Pineoblastoma

A

WHO IV. Diffuse growth of small blue cells w/ occasional homer wright and Flexner-wintersteiner rosettes (no pineocytomatous rosettes). Necrosis and high miotic index.

60
Q

Germ cell tumors

A

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)

61
Q

Adamantinomatous craniopharyngioma

A

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

62
Q

Papillary craniopharyngioma

A

BRAF V600E (>95%). Exclusively in adults (40-55 yrs). Well differentiated squamous epithelium without surface keratinization.

63
Q

Pituitary adenoma

A

10-20% of all intracranial neoplasms. Adults, F>M, Most common is prolactinoma.
Assoc w/ MEN1

64
Q

Mets

A

Most common CNS tumor, Lung, melanoma, breast, RCC, and GI. 70% are multifocal, present at gray-white jxn

65
Q

Lipoma

A

Midline: corpus callosum, 3rd ventricle, CP angle, quadrigeminal area. Can be assoc w/ agensis of the corpus callosum.

66
Q

Pilomyxoid astrocytoma

A

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

CNS (17 decks)

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