SYB 3
Inherited SyndromeHistory55 year old female presents with headaches, slurred speech and changes in short-term memoryPt also complained of gait abnormalitiesH/o renal cell carcinomaMRI
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HemangioblastomaUncommon brain tumor of vascular originCerebellum 83%Spinal cord 13%BenignSporadic tumor Usually solitaryVon-Hippel-Lindau diseaseFrequently multiple tumorsVon-Hippel-Lindau DiseaseAutosomal Dominant syndromeManifestations include a variety of benign and malignant tumors HemangioblastomasClear cell renal cell carcinomasPheochromocytomasEndolymphatic sac tumors (middle ear)Serous cystadenomas and neuroendocrine tumors (pancreas)Papillary cystadenomas (epididymis and broad ligament)Surveillance ProtocolAnnual Exam that includes:Plasma catecholamines and abdominal CT w/contrast PheochromocytomaRetinal exam with pupil dilation Retinal angiomasMRI of the brain and entire spine with gadoliniumHemangioblastomaMRI or CT of the abdomenRenal cell carcinoma
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