Other name for Neurofibroma type I?
- Peripheral neurofibromatosis or Von-Recklinghausen’s syndromeQ
- Type I is most prevalent type – 90%
Chromosome of NF-1 gene?
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
Other name for Neurofibroma type II?
- Central neurofibromatosis or bilateral acoustic neurofibromatosisQ
- Type 2 is less prevalent (10%)
Chromosome of NF-2 gene?
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
Tumors associated with Von Hippel Lindau Syndrome?
- Benign cysts in kidney, epididymis, liver or pancreasQ
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
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
What is Ependymoma?
Tumor that arise from the ependymal lining of the cerebral hemispheres and remnants of the central canal of the spinal cord
MC histologic type of ependydoma?
Myxopapillary ependymomaQ, which typically arises from the filum terminaleQ of the spinal cord and appears in the lumbosacral regionQ.
CT finding of Ependydoma?
Diffusely enhancing massesQ
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.
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.
Common uses of Stereotactic Radiosurgery?
- • Metastatic tumors
- • Benign lesions of the cranial nerves
- • Arteriovenous malformationsQ
- • Trigeminal neuralgia
Tumors that spread through CSF?
- • MedulloblastomaQ
- • PinealoblastomasQ
- • Germ cell tumorsQ
- • CNS LymphomasQ
Self amusement from poor jokes and puns, also known as pathological joking, is seen in frontal lobe tumorsQ
MC primary intra-axial tumor in the adult posterior fossaQ?
Histological features of Oligodendroglioma (ODG)?
“fried egg” cytoplasm, “chicken wire” vasculature, and microscopic calcificationsQ
Chromosomal deletion and Oligodendroglioma?
Chromosomal deletion, 1p and 19q, has been associated with robust response to temozolomideQ.
MC tumors of the brainQ?
Metastatic brain tumors
Most common primary site for metastatic brain tumors?
CA lungQ (50%) >breast cancerQ (15-20%)
IOC for metastatic brain tumors?
- MRI with gadolinium enhancementQ
- Lesions are at the gray-matter and white-matter junction, well circumscribed, surrounded by edemaQ.
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.