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作者报道1966～1987年21年间收治的40例儿童肾上腺皮质癌。诊断以手术或尸解所见肿瘤及病理检查为标准。38例术后确诊,2例尸解确诊。女性28例、男性12例,平均年龄3.9岁(1天～15.7岁)。生化评估由服用地塞米松前后检验患者24小时尿液的17-OH、17-KH和血浆脱氢表雄甾酮(DHA)水平得出。75%的ACC患者于出现临床症状后≥6个月作出诊断,仅11例6个月内作出诊断。治疗以手术为首选方式。38手术治疗,26例完全切除,6例部分切除,6例仅作活检。26例完全切除者5例复发,其中4例采用放疗和O,P′DDD化疗,仍因病情加重死亡,1例第三次切除复发瘤后,应用顺铂和VP-16化疗每月一个疗程共作了8个疗程,生存
The authors reported 40 children with adrenocortical carcinoma treated during the 21 years from 1966 to 1987. The diagnosis is based on the tumors and pathological examinations seen by surgery or autopsy. 38 cases were diagnosed after operation, and 2 cases were confirmed by autopsy. There were 28 females and 12 males with an average age of 3.9 years (1 day to 15.7 years). The biochemical evaluation was based on testing 24-hour urine 17-OH, 17-KH, and plasma dehydroepiandrosterone (DHA) levels before and after dexamethasone administration. 75% of ACC patients were diagnosed ≥6 months after the onset of clinical symptoms, and only 11 patients were diagnosed within 6 months. Treatment is the preferred method of surgery. 38 Surgical treatment, 26 cases were completely resected, 6 cases were partially resected, and 6 cases were only biopsy. Of the 26 patients who had undergone complete resection, 5 had recurrences, 4 of whom were treated with radiotherapy and O, P′DDD chemotherapy, and were still aggravated by illness. One patient received a third course of recurrent tumor resection and was treated with cisplatin and VP-16 once a month. A total of 8 courses were made to survive