雄性化腎上腺皮質癌：一例報告

Virilizing Adrenocortical Carcinoma: Report of A Case

兒童的腎上腺皮質癌，在台灣是不常見的案例。林季珍醫師等人曾於1976年在中兒醫誌發表一混合性腎上腺皮質癌。新光醫院小兒科報告一例單純性雄性化腎上腺皮質癌。一歲的女童在最近二個月內，有恥毛發育合併有陰蒂增生及臉上出現青春痘的現象。病人身體檢查發現右上腹有鼓脹現象，摸起來有腫塊的感覺。外生殖器方面，恥毛發育相當於Tanner stagen II，陰蒂長度有2.5公分。內分泌學檢查顯示男性荷爾蒙和DHEA-S有明顯增高的現象。影像學檢查方面，骨齡超前為二歲六個月，腹部電腦斷層顯示有一個完整包被膜的腫瘤在右腎上面。病人接受外科手術切除腫瘤，術後病理診斷為一個9 X 8 X 7公分大的腎上腺皮質癌。文中討論我們對此病人的診治經驗、治療的方式和後續追蹤、癒後因子，並參考國內、外文獻做整理回顧。

Virilizing adrenocortical carcinoma is an extremely rare disease in children. We report a 1 year-old girl who had progressive development of pubic hair, facial acne and clitoris hypertrophy in a few months. Besides, abdominal distension with a palpable mass over right abdomen was noted. Pubic hair was Tanner stage II and clitoris length was 2.5 cms. Serum levels of testosterone, 17- OHP and DHEA-S were increased. Image studies showed a well capsulated mass above the right kidney. Complete removal of the tumor was accomplished through a transabdominal approach. The diagnosis of adrenocortical carcinoma was confirmed histologically. One month after operation, the levels of testosterone and DHEA-S returned to the normal range. We present this case and review the literature.