Neuro tumors Flashcards

1
Q

Characteristics of astrocytoma

A

Originates from atrocytes

Immunoreactivity for glial fibrillary acidic protein (GFAP) and diffuse (ill-demarcated) pattern of growth

Pilocytic astrocytoma is a well-differentiated, benign astrocytic tumor that arises throughout the neuroaxis; it is common in children and young adults

Sites of involvement include posterior fossa (cerebellum) and diencephalon

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2
Q

Radiographical characteristics of astrocytoma

A

Most show cystic lesion with a mural nodule

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3
Q

Histologically characteristics of astrocytoma

A

Shows spindly neoplastic astrocytes with long bipolar processes
Rosenthal fibers

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4
Q

What are rosenthal fibers?

A

Thick, corkscrew-like eosinophilic structures that derive from hypertrophic processes of atrocytes

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5
Q

Characteristics of fibrillary (diffuse) astrocytoma

A

Low grade tumor that arises in the cerebral hemisphere of young to middle-aged and the brainstem of children
IDH1 positive

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6
Q

Characteristics of anaplastic astrocytoma

A

Cellular, pleomorphic and mitotically active

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7
Q

Characteristics of glioblastoma

A

Most common CNS primary malignancy in adults

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8
Q

Histological characteristics of gliomblastoma

A

Shows necrosis and/or vascular proliferation in addition to features seen in anaplastic astrocytoma

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9
Q

Characteristics of glioblastoma multiforme

A

Tendency to cross midline by involving the corpus callosum (“butterfly glioma”)

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10
Q

Characteristics of oligo dendroglioma

A

Occurs more often in adults than in children
Cortical location; may cause seizures
Slow-growing
Tumors with deletions of 1P or 19q respond well to therapy

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11
Q

Histological characteristics of oligo dendroglioma

A

Perinuclear halos are a fixation artifact that is not seen on frozen section

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12
Q

Characteristics of ependymoma

A

Located in the fourth ventricle in children, where it presents with obstructive hydrocephalus
In adults the spinal cord is the most common site
Multifocality in the spinal cord is associated with NF2

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13
Q

Histological characteristics of ependymoma

A

Pseudorosettes are a helpful diagnostic feature on microscopic study

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14
Q

Characteristics of embryonal (primitive) tumors

A

Group of small round cell tumors that occur predominantly in children
In the cerebellum they are called medulloblastoma
Most common malignant brain tumor in children

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15
Q

Characteristics of schwannoma

A

Originates from schwann cells of cranial or spinal nerves
Most frequent location is on CN8 at the cerebellopontine angle (CPA)
Manifests characteristically with unilateral loss of hearing and tinnitus
Prognosis good after resection
S100 positive
NF1

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16
Q

NF2 schwannoma

A

Bilateral acoustic schwannoma

17
Q

Histological characteristics of schwannoma

A

Has spindly cells arranged in hypercellular Antoni A areas; alternating hypocellular Antoni B areas
Verocray bodies

18
Q

What are verocay bodies?

A

Parallel rows of neoplastic Schwann cells

19
Q

Characteristics of meningioma

A

Originates from meningothelial cells of the arachnoid
Common in adults (F>M), rare in children
Dura based mass
Clinical features: Headache, seizures and neurological deficits
Abnormalities of chromosome 22 sometimes present
Multiple meningiomas occur in NF2 patients

20
Q

Histological characteristics of meningioma

A

Shows cellular whorls and psammoma bodies

Syncytial pattern is common

21
Q

Characteristics of craniopharyngioma

A

Arises from odotogenic epithelium within the suprasellar/diencephalic region
Most commonly affects children and young adults
Most common presenting symptoms: Headache, hypopituitarism, and visual field disturbances
Contains deposits of calcium
Benign but tends to recur after resection
Beta-catenin (CTNNBIc) gene mutations have been reported

22
Q

Histological characteristics of craniopharyngioma

A

Shows squamous cells

23
Q

Characteristics of lymphomas

A

Most common CNS tumor in the immunosuppressed
May be multiple
Don’t respond well to chemo

24
Q

Characteristics of germ cell layers

A

More common in children than adults

25
Q

Histological characteristics of germ cell tumors

A

Germinoma most common histologic type

26
Q

Characteristics of Sturge-Weber syndrome

A

Also known as Encephalotrigeminal angiomatosis
Presentation:
Facial angioma (Port-wine stain) in the V1 + V2 distribution of trigeminal nerve
Leptomeningeal angioma, often same side as the port-wine stain

Associated conditions:
Mental retardation
Epileptic spam at infancy
Early onset glaucoma
Skull radiopacities ("Tram track" calcification)
27
Q

Characteristics of Charcot-Marie-Tooth disease

A

Also known as hereditary motor and sensory neuropathy (HMSN)
Mutations in a peripheral myelin protein (PMP22) forms unstable myelin
Distal symmetric sensorimotor polyneuropathy with atrophy of the calf muscles (“stork legs”) and pes cavus deformity
Nerve conduction studies: decrease impulse conduction velocity

28
Q

Histological characteristics of Charcot-Marie-Tooth disease

A

Nerve biopsy-segmental demylination and remyelination with onion bulb formation

29
Q

Characteristics of neurofibroma

A

Heterogenous in composition- Schwann cells are admitted with perineural-like cells, fibroblasts, mast cells and CD34+ spindle cells
Solitary are not associated with NF1
Multiple are associated with NF1
Often young, 20-30

30
Q

Classifications of neurofibroma

A

Cutaneous or dermal
Diffuse
Plexiform

31
Q

Characteristics of cutaneous or dermal neurofibroma

A

Small nodular tumors of the skin and subcutaneous tissue, arising from small cutaneous nerves

32
Q

Characteristics of diffuse neurofibroma

A

present as large plaque-like elevation of skin

33
Q

Characteristics of plexiform neurofibroma

A

multiple nodular masses affecting nerve roots, plexuses or large nerves (appear as “bag of worms”)

34
Q

Characteristics of malignant peripheral nerve sheath tumors (MPNST)

A

Mostly high grade tumors, but uncommon
Can be sporadic tumors or NF1-associated tumors arising through malignant transformation of a plexiform neurofibroma
Most commonly found on the extremities and trunk, and less often on the head and neck
An interesting feature of MPNST is a “divergent differentiation” i.e. the presence of other lines of differentiation, including glandular, cartilaginous, osseous or rhabdomyoblastic morphology
S100+

35
Q

Characteristics of conjunctival carcinoma

A

Keratinizing squamous cell carcinoma
Develop from precancerous lesion (Bowden’s disease-squamous cell carcinoma in situ)
Often associated with human papillomavirus (HPV) types 16 and 18
Clinical presentation: Whitish, bulging thickening of the epithelium with an irregular surface

36
Q

Characteristics of uveal melanoma

A

Most common primary intra-ocular malignancy of adults
Develops from choroidal melanocytes
Common location” Choroid~18%, Ciliary body ~15%, Iris~5%
Risk factors: Uveal nevus, light skin color, and light color of iris
Clinic: Visual field defects, floaters, and/or photopsia due to tumor-related retineal detachment
Fundoscopy: A pigmented lesion near temporal margin of the optic disk

37
Q

Histological characteristics of retinoblastoma

A

Clinical features:
Leukocoria (“cat’s eye pupil”); Strabismus
Progressive loss of vision- due to tumor infiltration into the macula or vitreous body
Sudden loss of vision- due to retineal detachment

38
Q

Histological characteristics of retinoblastoma

A

Small blue cells- round with hyperchromatic nuceli, scanty cytoplasm
Rosettes and fleurette
Necrosis; viable tumor around blood vessels
Dystrophic calcification- characteristic

39
Q

Characteristics of tuberous scelerosis

A

Characterized by hamartomas (cortical tubers)
Presentation: Seizure (infantile spasms)

Mutation:
TSC1 (encodes hamartin on chromosome 9)
TSC2 (encodes tuberin on chromosome 16)

Skin:
Adenoma sebaceum (angiofibromas)
Hypopigmented macule (ash leaf spot)
Shagreen patches

Visceral cysts: Liver, kidney, pancreas
Retina- Retinal glial hamartomas
Heart-Cardiac rhabdomyomas
Renal-Renal angiomyolipomas