Harris A C, Wakely P E, Kaplowitz P B, Lovinger R D
Department of Pathology, Medical College of Virginia, Virginia Commonwealth University, Richmond 23298.
Arch Pathol Lab Med. 1991 Feb;115(2):150-4.
An 8-year-old girl exhibited severe, progressive virilization of 2 years' duration associated with markedly elevated circulating testosterone concentrations. Based on her initial clinical presentation and results of a chemical evaluation, she was originally thought to have non-classic 21-hydroxylase deficiency, but her condition did not respond to corticosteroid therapy. Further evaluation confirmed the presence of an ovarian neoplasm. The excised ovary contained an attached gray-brown mass. Light microscopic and ultrastructural examination revealed the mass to be a steroid cell tumor. Because Reinke's crystals were not present, it was designated to be a steroid cell tumor not otherwise specified. This case represents one of 22 reported cases of steroid cell tumor occurring in children described in the literature, most of which have been associated with heterosexual precocity. To our knowledge, steroid cell tumors are benign when they occur in prepubertal children. Although they are rare, steroid cell tumors of the ovary should be considered in cases of childhood virilization.
一名8岁女孩出现了持续2年的严重进行性男性化，伴有循环睾酮浓度显著升高。根据她最初的临床表现和化学评估结果，最初认为她患有非经典21-羟化酶缺乏症，但她的病情对皮质类固醇治疗无反应。进一步评估证实存在卵巢肿瘤。切除的卵巢上附着有一个灰棕色肿块。光镜和超微结构检查显示该肿块为类固醇细胞瘤。由于不存在Reinke晶体，故将其指定为未另行指定的类固醇细胞瘤。该病例是文献中报道的22例儿童类固醇细胞瘤病例之一，其中大多数与异性性早熟有关。据我们所知，类固醇细胞瘤发生在青春期前儿童时是良性的。尽管罕见，但在儿童男性化病例中应考虑卵巢类固醇细胞瘤。
