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8 Cards in this Set
- Front
- Back
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major clinical features of VHL
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CNA hemangioblastomas, Retinal hemangioblastomas, Renal Cell Carcinoma, Pheochromocytomas/paragangliomas, neuroendocrine tumors of pancreas, endolymphatic sac tumors, papillary cystadenomas of epididymus
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CNS hemangioblastomas are
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tumors throughout the CNS that are benign and unpredictable
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Renal Cell Carcinomas are
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accompanied by asymptomatic cysts
unpredictable growth |
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treatment of renal cell carcinomas
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surgery, radiofrequency or cryoablation
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histology of renal cell carcinoma
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clear cell type
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pheochromocytomas are
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neuroendocrine tumors of the medulla
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criteria for gene mutation analysis for VHL
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pt with classical VHL disease and or first degree family members
a person from a family where germline VHL gene has been identified pt has multicentric tumors on one organ, bilateral tumors, two organ systems affected, one VHL associated tumor at young age patient from a family with one hemangioblastoma, renal cell carcinoma, or pheochromocytoma |
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Screening for VHL
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eye-infancy, yearly
MRI-CNS- 10-12yrs. yearly Abdominal US- teens, yearly CT/MRI, audiology-symptoms catecholamines- 2 yrs. yearly |
