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종결 Case No. 145 0000-00-00~0000-00-00
출제자 : 관리자 Hit : 289
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Period 0000-00-00~0000-00-00
Diagnosis
Clinical information 31세 남자. 1개월간의 두통과 어지러움으로 내원. 과거력상 pheochromocytoma 진단 받음.
Discussion Hemangioblastoma

Imaging findings:
?Intra axial posterior fossa mass
?Some cystic lesions with well enhancement
?Highly vascularized mass on DSA


Reference: Hemangioblastoma
?Incidence
?7% of 1?posterior fossa tumor in adult
?Sporadic or VHL (10~20%)
?Age
?3rd~5th decade, rare in children
?Location
?80~85%; Cbll
?3~13%; spinal cord
?Supratentorial; uncommon
?Curable by surgery

von Hippel-Lindau Syndrome
?Autosomal dominant
?Variety of benign & malignant tumors
?Hemangioblastoma including retinal angioma; 60~80%
?RCC; m/s cause of death
?Pheochromocytoma
?Endolymphatic sac tumor of middle ear
?Serous cystadenoma & neuroendocrine tumor of pancreas
?Papillary cystadenoma of epididymis
?VHL type I
?No pheochromocytoma
?VHL type II
?High risk of pheochromocytoma
?Type IIA; low risk of RCC
?Type IIB; high risk of RCC
?Type IIC; only pheochromocytoma without hemangioblastoma & RCC
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