von Hippel-Lindau病に合併した内リンパ嚢腫の1例

A case of endolymphatic cyst associated with von Hippel-Lindau disease

von Hippel-Lindau病（VHL病）は，複数の臓器に腫瘍性あるいは嚢胞性病変が多発する常染色体優性遺伝性疾患である^1）。VHL病の3.6～16％で内リンパ嚢腫を合併することが知られている。今回，VHL病に合併した内リンパ嚢腫の症例を経験したので，文献的考察を加えて報告する。

A woman in her 30s came to our hospital with nausea and vomiting. She was referred to our hospital because of a mass in the fourth ventricle and hydrocephalus. After a thorough examination, central nervous system multiple hemangioblastoma and bilateral adrenal pheochromocytoma were suspected, and the diagnosis of von Hippel-Lindau disease was made. CT scan of the dorsal surface of the right pyramidal bone showed bone destruction, and MRI showed high-signal mass shadows on both T1- and T2-weighted images, suggesting an endolymphatic cyst. Endolymphatic cysts are rare temporal bone tumors and are known to be associated with von Hippel-Lindau disease.