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Other name for Neurofibroma type I?
- Peripheral neurofibromatosis or Von-Recklinghausen’s syndromeQ
- Type I is most prevalent type – 90%
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Chromosome of NF-1 gene?
Chromosome 17Q
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Diagnostic Criteria for NF-1?
- Diagnosed when any two of the following are present):
- 1. ≥6 café-au-lait maculesQ
- 2. Axillary or inguinal frecklingQ
- 3. ≥2 iris Lisch nodulesQ
- 4. ≥2 neurofibromas or one plexiform neurofibromaQ
- 5. Sphenoid dysplasia or cortical thinning of long bone, with or without pseudoarthrosis
- 6. Optic gliomasQ
- 7. A first degree relative with NF-1 whose diagnosis was based on the aforementioned criteria
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Other name for Neurofibroma type II?
- Central neurofibromatosis or bilateral acoustic neurofibromatosisQ
- Type 2 is less prevalent (10%)
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Chromosome of NF-2 gene?
Chromosome 22Q
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Diagnostic Criteria for NF-2Q?
- Diagnosed when any one of the following is present
- 1. Bilateral 8th nerve masses consistent with acoustic neuromasQ
- 2. A parent, sibling, or child with NF-2 and either
- 3. Unilateral 8th nerve mass or any two of the following:
- - NeurofibromaQ
- - MeningiomaQ
- - GliomaQ
- - SchwannomaQ
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Tumors associated with Von Hippel Lindau Syndrome?
- Hemangioblastomas
- RCCQ
- PheochromocytomaQ
- Benign cysts in kidney, epididymis, liver or pancreasQ
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Findings in tuberous sclerosis?
- Skin lesions - Adenoma sebaciumQ (facial angiofibroma), Ash leaf shaped hypopigmented maculesQ, Shagreen PatchQ
- Neurological – SeizureQ, Mental retardationQ, Subependymal nodulesQ, HydrocephalusQ
- Benign neoplasm – RhabdomyomaQ, AngiomyomaQ of liver, kidney, pancreas etc, EpendymomaQ, AstrocytomaQ
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Features of Sturge Weber syndrome?
- Large unilateral cutaneous angiomaQ (port-wine stain)
- Focal seizuresQ typically occurs opposite to the side of lesionQ
- Adrenal pheochromocytoma
- Cerebral angiomas lead to cortical atrophyQ
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What is Ependymoma?
Tumor that arise from the ependymal lining of the cerebral hemispheres and remnants of the central canal of the spinal cord
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MC histologic type of ependydoma?
Myxopapillary ependymomaQ, which typically arises from the filum terminaleQ of the spinal cord and appears in the lumbosacral regionQ.
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CT finding of Ependydoma?
Diffusely enhancing massesQ
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Psychiatric symptoms in brain tumors?
- • Psychiatric symptoms are more common in frontal lobe tumorsQ.
- • Depression: More common in frontal lobe tumorsQ
- • Psychosis: More common in temporal lobe tumorsQ
- • Delirium is most often a component of rapidly growingQ, large, or metastatic tumors.
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Features of meningioma?
- Firm and fibrous to finely gritty due to calcifications
- Psammoma bodiesQ.
- Calcification is also seen in ependymoma, but more common in meningiomaQ.
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Common uses of Stereotactic Radiosurgery?
- • Metastatic tumors
- • Benign lesions of the cranial nerves
- • Arteriovenous malformationsQ
- • Trigeminal neuralgia
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Tumors that spread through CSF?
- • MedulloblastomaQ
- • PinealoblastomasQ
- • Germ cell tumorsQ
- • CNS LymphomasQ
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Witzelsucht syndrome?
Self amusement from poor jokes and puns, also known as pathological joking, is seen in frontal lobe tumorsQ
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MC primary intra-axial tumor in the adult posterior fossaQ?
Hemangioblastoma
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Histological features of Oligodendroglioma (ODG)?
“fried egg” cytoplasm, “chicken wire” vasculature, and microscopic calcificationsQ
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Chromosomal deletion and Oligodendroglioma?
Chromosomal deletion, 1p and 19q, has been associated with robust response to temozolomideQ.
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MC tumors of the brainQ?
Metastatic brain tumors
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Most common primary site for metastatic brain tumors?
CA lungQ (50%) >breast cancerQ (15-20%)
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IOC for metastatic brain tumors?
- MRI with gadolinium enhancementQ
- Lesions are at the gray-matter and white-matter junction, well circumscribed, surrounded by edemaQ.
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Treatment of metastatic brain cancer?
Surgery is recommended for accessible lesions (up to 3) causing mass effect followed by whole-brain radiation therapy (WBRT) to eradicate micrometastasesQ.
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