雄性型腎上腺皮質癌：病例報告

Virilizing Adrenocortical Carcinoma : A Case Report

10.7023/TJFM.200012.0226

黃真儀(Chen-Yi Huang)；李俊賢(Jun-Hsien Lee)；陳昱宏(Yu-Hung Chen)

adrenocortical carcinoma ； virilization ； androgen

台灣家庭醫學雜誌

10卷4期（2000 / 12 / 01）

226 - 232

繁體中文

腎上腺皮質癌在小孩子中是很罕見的惡性腫瘤，發生率極低，每年約每百萬人中才會發現一例新病例。絕大多數是有功六性的，如男性荷爾蒙分泌過多，會有男性儘或性早熟傾向；糖類皮質醇分泌過多時，會有庫欣氏症候群現象；也有混合型，即二者兼具。本文報告一位1 歲2個月大小女孩，被父母親發現過去5個月有陰蒂肥大現象，且最近3個月女童逐漸長出陰毛與青春痘，腹部亦有鼓脹情形。實驗室檢查顯示男性荷爾蒙有明顯增加，且腹部超音波和電腦斷層掃描發現右腎上方有一巨大腫瘤，但並未侵犯腎臟或下腔靜脈，也無肝臟或肺臟的轉移。病人經手術切除腫瘤後，臨床懷徵及實驗室數據逐漸恢復正常，而病理報告顯示為腎上腺皮質癌。早期完整切除腫瘤是最佳首選治療方法，且術後需定期追蹤檢查，以防復發或轉移。臨床上若發現兒童有緎早熟或男性化表現時，配合完整理學、實驗室與影像學檢查，當能早期發現此罕見惡性腫瘤，早期治療。

Adrenocortical carcinoma (ACC) is a very rare tumor in children with an annual incidence of one new case per million population and is usually functional, causing either virilization, Cushing’s syndrome, or both. Here, a 1 year and 2 months old girl with virilization due to adrenocortical carcinoma is described. She was presented with clitoris enlargement for 5 months and suffered from acne vulgaris, increasing abdominal mass and pubic hair for 3 months. The laboratory test made after admission showed that she had excessive production of androgens. Abdominal ultrasound and CT scan showed a large right-sided suprarenal mass but no distant metastases were found. Her clinical features have been significantly improved after surgical removal of the large tumor. The correct diagnosis of ACC is based on clinical, laboratory findings and various imaging methods. Complete surgical resection is the treatment of choice and has the best chance of cure in children with adrenocortical carcinoma.