Cerebellar hemangioblastoma and retinal tumors are seen in:
Correct Answer: Von Hippel Lindau syndrome
Description: Ref: Chapter 28. CNS. page - 1343: Robbins and Cot ran Pathologic Basis of Disease: 8th editionExplanation:The Von Hippel Lindau gene (VHL) is tumor suppressor gene located on Chromosome 3p25-p26 and down-regulates expression of vascular endothelial growth factor; hence its dysregulation is associated with hemangioblastomas and retinal tumors.The neurofibromatosis type 1 (Chr. 17) is associated with:Optic nerve gliomasLisch nodules (pigmented nodules of the iris)Cafe au lait spots (cutaneous hyperpig- mented macules)Neurofibromatosis type 2 (Chr. 22) is associated with:8th nerve schwannomasMultiple meningiomasGliomasEpendymoma of spinal cordTuberous sclerosis complex: It is associated with:Cortical tubersSubependymal nodulesSubependymal giant cell astrocytomasRenal angiomyolipomasRetinal glial hamartomasPulmonary lymphangioleiomyomatosisCardiac rhabdomyomasShagreen patches (cutaneous leathery thickenings)Ash-leaf patches (hypopigmented areas)Subungual fibromas
Category:
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