Brain Tumors; Path Brain Tumors

Front 1

Presenting symptoms of brain tumor are related to what?

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Non-specific related to increased ICP

Site-specific related to tumor location

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Presenting symptoms of brain tumor : Non-specific related to increased ICP

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Headache
AMS
Nausea/Vomiting
changes in vision

Front 3

Presenting symptoms of brain tumor : site-specific/focal

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Seizures
Hemiparesis
Dysphasia
Ataxia
Sensory Changes

Front 4

In children, two most common cancer categories

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1) Leukemia
2) CNS tumors

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Initial PE findings of brain tumor patients : non-specific

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Non-specific: AMS, subtle changes in personality, cognitive fxn etc

<b>Papilledema</b> - often asymptomatic

CN VI paresis

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T/F Papilledema is often asymptomatic

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T

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CN VI Paresis: why is it so common?

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It has a long intracranial course and is vulnerable as it passes over the petrous bone of the skull base.

Front 8

proptosis: defn

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condition resulting in forward displacement and entrapment of the eye from behind by the eyelids

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chemosis: defn

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swelling (or edema) of the conjunctiva.

Front 10

Von Hippel-Lindau syndrome: associated tumors

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Hemangioblastoma

Renal cysts/tumor

Front 11

How should new onset seizures be evaluated?

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MRI or CT

Front 12

Method of choice for imaging suspected brain tumors and why

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MRI - better resolution than CT of soft tissue

Front 13

When is CT used before MRI?

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When stroke/hematoma are in the differentials (CT shows fresh bleed better)

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T/F BOTH MRI and CT are valuable after detection of tumor

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T

Front 15

CSF cytology: use

Back 15

Can help make dx and prognosticate.

Presence of tumor cells in CSF indicates subarachoid dissemination - poor prognostic indictator

Front 16

Lumbar puncture: risks in brain tumor

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Brainstem herniation. Increased pressure within the skull (intracranial), due to a brain tumor or other space-occupying lesion, can lead to compression of the brainstem after a sample of cerebrospinal fluid is removed. A computerized tomography (CT) scan or MRI prior to a lumbar puncture can be obtained to determine if there is evidence of increased intracranial pressure.

Front 17

Early medical mgmt of brain tumors

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high dose steroids

IV mannitol - reduce swelling and increases blood flow

intensive medical mgmt - rehydration, nutrition, etc

Front 18

Primary treatment for most brain tumors

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resection

Front 19

Prolactinoma: tx

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DA agonist (bromocriptine)

Front 20

Radiation is as or more effective than resection for ____ and ____.

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germinomas; lymphomas

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T/F Radiation should be used for multiple intracranial mets

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T

Front 22

T/F Gliomas are diffuse tumors

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T

Front 23

Radiation therapy: when is it used?

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1) High-grade gliomas post max resection

2) Multiple mets

3) Benign tumors (meningiomas and pituitary adenomas) that are unresectable and thought to recur

4) Lymphomas and germinomas

Front 24

Chemotherapy: when is it used? problems?

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Problems - most chemo doesn't cross BBB

Useful in peds tumors, especially medulloblastoma

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Three parameters in brain tumor that predict outcome

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1) Histology type and grade

2) Age

3) Performance status of pt at dx

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Most common brain tumor type in all ages

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Glioma

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2nd most common brain tumor type in adults

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Meningioma

Front 28

4 types of gliomas

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Astrocytoma (malignant and benign)

Oligodendroglioma

Ependymoma

Medulloblastoma

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GBM - mean survival time

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about 1 year

Front 30

Hemangioblastoma: tumor marker

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Erythropoietin -> Elevated hematocrit

Front 31

Elevated HCG is a marker for what tumors?

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1) Choriocarcinoma

2) Endodermal sinus tumor

3) Germinoma

Front 32

Cancers that often met to brain

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lung
breast
kidney
melanoma
GI tract

Front 33

MGMT Methylation/Silencing: Relation to Survival in Patients with Malignant Glioma

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Patients with MGMT Methylation/Silencing had longer survival times

Front 34

classification of brain tumors based on ______

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cells of origin

Front 35

Glioblastoma multiform is derived from ______

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Astrocytes

Front 36

Medulloblastoma is derived from ____

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primitive (embryonal) cells

Front 37

PNET of the CNS generally refer to (infra, supra) tentorial PNETs

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supra. Infratentorial PNETs are called medulloblastoma

Front 38

Medulloblastoma : location

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cerebellum

Front 39

Primitive cells: what tumors derive from these?

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Medulloblastoma (cerebellum)

PNETs (elsewhere - supratentorium)

Front 40

Ganglioglioma is derived from _____.

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Glioneuronal cells

Front 41

Schwannoma and neurofibroma are derived from _____

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nerve sheath cells

Front 42

T/F Malignant brain tumors often met outside CNS.

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F

Front 43

T/F Biological behaviors of brain tumor (growth, invasion) are usually predictable from histologic appearance.

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T

Front 44

T/F Metastatic tumors are usually well-circumscribed.

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T

Front 45

carcinomatous meningitis: defn

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Metastatic tumors that infiltrate CNS diffusively, mostly in leptomeninges and subarachnoid space.

Front 46

Immunosuppression is associated with what type of brain tumor?

Back 46

Primary CNS lymphoma

Front 47

Prior radiation treatment is associated with what type of brain tumor?

Back 47

Meningiomas
Gliomas

Front 48

In children, 70% of tumors are (infra, supra) tentorial while in adults, 70% of tumors are (infra, supra) tentorial

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infra; supra

Front 49

2 broad divisions of astrocytoma

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1) Diffuse/infiltrative
2) Circumscribed

Front 50

Diffuse/infiltrative astrocytoma: location and age

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Mostly in cerebral hemispheres in adults

Front 51

Diffuse/infiltrative astrocytoma: 3 grades/types

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1) Low-grade (WHO grade II)
2) Anaplastic (WHO grade III)
3) Glioblastoma (WHO grade IV)

Front 52

Most malignant form of astrocytoma

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Glioblastoma

Front 53

Glioblastoma : characteristic histological features

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Linear strips of necrosis with viable tumor cells alongside (pseudopalisading)

Front 54

pseudopalisading: defn

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linear strips of necrosis with viable GBM cells alongside

Front 55

3 most common adult brain tumors

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1) GBM
2) Meningioma
3) Schwannoma

Front 56

3 most common childhood brain tumors

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1) Pilocytic astrocytoma
2) Ependymoma
3) Medulloblastoma

Front 57

T/F GBM often crosses the corpus callosum, producing a butterfly lesion

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T

Front 58

GBM tumor cells are _____ positive

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GFAP

Front 59

Circumscribed astrocytoma: 2 types

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1) Pilocytic astrocytoma
2) Pleomorphic xanthoastrocytoma (PXA)

Front 60

Pilocytic astrocytoma: age and location

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Mostly children, along cerebellum (also optic nerves and hypothalamus)

Front 61

Pleomorphic xanthoastrocytoma (PXA): age and location

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Mostly children; Temporal lobe or other cerebral location

Front 62

Pilocytic astrocytoma: characteristic imaging

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Cystic lesion with a mural nodule (nodule in wall of cyst)

Front 63

Pilocytic astrocytoma: characteristic histology

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Rosenthal fibers - thick eosinophilic astrocyte processes, hair like (hence pilocytic)

Biphasic appearance (compact and loose)

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Pilocytic astrocytoma: genetic assns

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Neurofibromatosis Type I

Front 65

Pleomorphic xanthoastrocytoma (PXA): characteristic histology

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Large pleomorphic glial cells mixed with cells have a bubbly "xanthomatous" cytoplasm

Front 66

Circumscribed astrocytomas : Treatment

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Often can be removed by complete excision

Front 67

Oligodendroglioma: characteristic histology

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"Fried egg" appearance - round regular nuclei often with perinuclear clear space

Front 68

Oligodendroglioma: characteristic imaging findings

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Calcifications in white matter usually involving frontal lobe

Front 69

Oligodendroglioma: clinical symptoms and why

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seizures often because cells infiltrate into superficial cerebral gray matter

Front 70

Ependymoma: age and location

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Children; most commonly in 4th ventricle

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Ependymoma: characteristic histology

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Perivascular pseudorosettes

Front 72

Choroid plexus tumors: age and location

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Children; Within proximity of ventricles, esp lateral and 4th ventricles

Front 73

Choroid plexus tumors: histology

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look like normal (but more complex epithelium) choroid plexus

Front 74

Choroid plexus tumors: complications

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hydrocephalus due to overproduction of CSF

Front 75

Glioneuronal tumors: defn

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neuroepithelial tumors composed of mature neurons (gangliocytoma) or mixed population of ganglion cells and glial cells (ganglioglioma).

Front 76

Glioneuronal tumors: age and location

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All age groups but MOSTLY YOUNG.

Temporal lobe

Front 77

Glioneuronal tumors: Complications/Associations

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most common tumor type implicated in epilepsy

Front 78

Small round blue cell tumors : think _____

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PNETs and medulloblastoma

Front 79

T/F Medulloblastomas are highly malignant and radioinsensitive.

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F. They are highly malignant, often disseminate along CNS paths, but they are radioSENSITIVE.

Front 80

Meningioma: Age, Sex, Location

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Age: middle aged adults

Sex: women

Location: dura over cerebral convesity, or on skull base. rarely in spinal canal.

Front 81

Most common benign CNS tumor in adults

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Meningioma

Front 82

Meningioma: characteristic histology

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Whorled pattern of cells with or without psammoma bodies

Front 83

Meningioma: characteristic imaging

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Round mass attached to dura, some with a "dural tail"

Front 84

Most common type of intracranial germ cell tumor

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Germinomas

Front 85

Germinoma: age and location

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Children/Young Adults

<b>Midline</b>, usually pineal and more rarely suprasellar regions

Front 86

Craniopharyngioma: age and location

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Children

Suprasellar region

Front 87

Craniopharyngioma: characteristic symptoms

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Visual and endocrine deficits

Front 88

Craniopharyngioma: origin

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epithelial remnants of Rathke's pouch

Front 89

Craniopharyngioma: Histology

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Sheets of epithelial cells with keratin-filled cysts and calcifications

Front 90

derived from remnants of Rathke's pouch

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Craniopharyngioma

Front 91

Pituitary adenoma: what causes symptoms?

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1) Excessive hormone production
2) Compression of optic chiasm - bitemporal hemianopsia

Front 92

microadenoma: defn

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hormone-producing tumors that, because of hormonal effects, are discovered at early stage

Front 93

macroadenoma: defn

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typically non-functioning, come to clinical attention through their mass effect on nearby structures

Front 94

Primary CNS lymphoma associated with HIV - what type of lymphoma? Location?

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mostly large B-cell type

usually in cerebrum

Front 95

Primary CNS Lymphoma: prognosis

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Very poor. Highly malignant

Front 96

Intraspinal tumors: 3 groups

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1) Extradural
2) Intradural extramedullary (inside dura but outside cord proper)
3) Intramedullary (within the cord proper)

Front 97

Extradural Intraspinal tumors: what are they?

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Mostly mets from lung, breast, prostate and other sites to vertebra with extension into epidural space

Front 98

Intradural Intraspinal tumors: what can they be? (4)

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A) Meningioma - mostly women

B) Nerve sheath tumors - schwannoma, neurofibroma

C) Drop metastases - spread from intracranial tumors

D) Mets from breast, lung, GI, etc.

Front 99

Intramedullary Intraspinal tumors: what can they be? (4)

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1) Ependymoma

2) Astrocytoma

3) Hemangioblastoma

4) Mets

Front 100

Two main age groups that get CNS tumors:

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1) Kids: first two decades

2) Older adults: 60s and 70s

Front 101

Tumors of the spinal cord: age

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Adults

Front 102

Mets to CNS are approximately ___% of CNS tumors

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50.

Front 103

80% of adult primary CNS tumors

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Infiltrative Astrocytomas

Front 104

Which astrocytoma :Characteristically do not enhance post contrast?

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Diffuse astrocytoma - WHO Grade II

Front 105

Which astrocytoma :Characteristically has ring-enhancing lesion?

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GBM - WHO Grade IV

Front 106

T/F Low grade astrocytomas can undergo malignant degeneration to become high grade tumors

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T

Front 107

IDH1 mutation: which astrocytomas?

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IDH1 mutation encountered in grades II and III, and secondary GBM

Front 108

Oligodendroglioma: Mutations seen

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deletion of chromosomes 1p / 19q

Front 109

Myxopapillary ependymoma : location

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Conus medullaris, conus/cauda equina/filum terminale region

Front 110

Homer Wright rosettes
seen in what?

Back 110

Medulloblastoma

Front 111

Homer Wright rosettes: defn

Back 111

differentiated tumor cells surround the neuropil.

Front 112

Atypical teratoid / rhabdoid tumor (AT/RT): defn and clinical characteristics

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Primitive CNS neoplasm of infancy
Can arise anywhere in brain
Always fatal

Front 113

Meningioma: associated with what genetic condition?

Back 113

NF2. Younger patients

Front 114

Most common lesion of the pituitary fossa / sella region

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Pituitary adenoma

Front 115

Neurofibromin gene on C17

Back 115

Neurofibromatosis Type I

Front 116

Neurofibromatosis Type I: What neoplasms are seen?

Back 116

Multiple Neurofibromas (cutaneous and plexiform);

Malignant peripheral nerve sheath tumor

Optic gliomas

Pigmented lesions (Lisch nodules and café au lait spots)

Front 117

Merlin gene on C22

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Neurofibromatosis Type II

Front 118

Neurofibromatosis Type II: What neoplasms are seen?

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Bilateral acoustic neuroma

Multiple meningiomas at earlier age

Spinal ependymomas

Front 119

Von Hippel - Lindau: defective gene location

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Chromosome 3

Front 120

TSC1 and TSC2 genes

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Tuberous Sclerosis

Front 121

Tuberous sclerosis: what neoplasms?

Back 121

Skin- Adenoma sebaceum

CNS- candle guttering, tubers,

SubEpendymal Giant cell Astrocytoma (SEGA)

Heart- rhabdomyoma

Kidney- angiomyolipoma

Multiorgan cysts