Frémond B, Jouan H, Sameh A H, Le Gall E, Bergeron C, Manac'h A, Gruel Y, Babut J M
Chir Pediatr. 1987;28(2):97-101.
The authors report 2 cases of hepatocellular tumour in children treated with anabolic androgens for aplastic anemia. In both cases, the presentation was by a picture of acute abdomen due to hemoperitoneum caused by tumour rupture. In the first case, there was multiple hepatic adenomas necessitating right hepatic lobectomy. The second infant had a single tumour of segment IV treated by simple excision of the tumour. It was a hepatocellular-carcinoma. Follow-up for one year after the initial operation showed no signs of recurrence in both infants. The review of the literature permitted us to find 48 other cases of hepatocellular tumour secondary to androgen therapy. In order of frequency, the hepatocellular-carcinoma is the most frequent and it is usually single; followed by the adenoma which is usually multiple. The other types of tumours are rare: focal nodular hyperplasia, angiosarcoma and cholangiocarcinoma. The hepatocellular-carcinoma and adenoma have some characteristic features: spontaneous regression may occur after withdrawing of androgens; the risk of rupture is important; their evolution is almost always favorable despite of a severe histopathological picture; the alpha-foeto-protein is nearly always negative; and the metastasis are exceptional. The hepatocellular-carcinomas associated with androgen therapy are probably just adenomas with marked dysplasia, but their long term malignant potential remain unknown. Except in case of rupture, surgical intervention should be postponed until the effect of discontinuing the hormonal therapy is assessed, because of the potential for spontaneous regression. The administration of antineoplastic chemotherapeutic agents should be reserved for the tumours showing evidence of malignancy.
作者报告了2例接受合成代谢雄激素治疗再生障碍性贫血的儿童肝细胞肿瘤病例。两例病例均表现为因肿瘤破裂导致腹腔积血而出现的急腹症。第一例有多个肝腺瘤,需行右肝叶切除术。第二例婴儿有一个IV段的单一肿瘤,通过单纯肿瘤切除术治疗。这是一例肝细胞癌。初次手术后随访一年,两名婴儿均无复发迹象。文献回顾使我们找到了另外48例雄激素治疗继发肝细胞肿瘤的病例。按发生频率排序,肝细胞癌最为常见,通常为单发;其次是腺瘤,通常为多发。其他类型的肿瘤较为罕见:局灶性结节性增生、血管肉瘤和胆管癌。肝细胞癌和腺瘤有一些特征:停用雄激素后可能会自发消退;破裂风险很大;尽管组织病理学表现严重,但其发展几乎总是良好的;甲胎蛋白几乎总是阴性;转移罕见。与雄激素治疗相关的肝细胞癌可能只是具有明显发育异常的腺瘤,但其长期恶性潜能仍不清楚。除破裂情况外,由于存在自发消退的可能性,手术干预应推迟到评估停用激素治疗的效果之后。抗肿瘤化疗药物的使用应仅限于显示有恶性证据的肿瘤。