van Hoeven K H, Factor S M, Kress Y, Woodruff J M
Department of Pathology, Albert Einstein College of Medicine, Bronx, New York.
Am J Surg Pathol. 1993 Nov;17(11):1176-81.
We report a primary smooth-muscle tumor of undetermined malignant potential of the liver in a child with acquired immune deficiency syndrome (AIDS). This patient represents the eighth child infected with the human immunodeficiency virus who developed a mesenchymal tumor other than Kaposi's sarcoma. All these children were younger than 10 years of age. These tumors often were histologically or clinically malignant and all but one were smooth-muscle tumors. These tumors arose exclusively in visceral organs, and the hepatobiliary, gastrointestinal, and tracheopulmonary systems were involved. Transmission of the virus occurred both vertically (in six children) and via blood transfusion (in two). Given the rarity of smooth-muscle tumors in uninfected children, the unusual frequency of these tumors suggests that immunosuppression induced by the virus permits the unregulated proliferation of a primitive mesenchymal cell disposed to myogenous differentiation, a situation not unlike that observed in the development of AIDS-related Kaposi's sarcoma in adults.
我们报告了一名患有获得性免疫缺陷综合征（AIDS）的儿童肝脏原发性具有不确定恶性潜能的平滑肌瘤。该患者是第八例感染人类免疫缺陷病毒并发生除卡波西肉瘤之外的间充质肿瘤的儿童。所有这些儿童均未满10岁。这些肿瘤在组织学上或临床上常为恶性，除1例以外均为平滑肌瘤。这些肿瘤仅发生在内脏器官，累及肝胆、胃肠和气管肺系统。病毒传播途径包括垂直传播（6例儿童）和输血传播（2例）。鉴于未感染儿童中平滑肌瘤罕见，这些肿瘤出现的异常频率表明，病毒诱导的免疫抑制使得倾向于肌源性分化的原始间充质细胞不受控制地增殖，这种情况与成人艾滋病相关卡波西肉瘤的发生情况类似。
