Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Hum Pathol. 2012 Apr;43(4):597-600. doi: 10.1016/j.humpath.2011.06.012. Epub 2011 Oct 10.
Extracardiac rhabdomyomas are rare benign entities that usually occur in the head and neck region. Although genital rhabdomyoma is known to occur in the lower genital tract of young and middle-aged women, involvement of the anatomically adjacent urethra by rhabdomyoma is exceedingly rare. We present a case of genital rhabdomyoma arising from the urethra of an infant girl. The tumor was characterized by the submucosal presence of mature-appearing rhabdomyoblastic cells containing conspicuous cross-striations, with the cells set in a collagenous stroma. Necrosis and mitoses were absent. Skeletal muscle differentiation of the tumor cells was supported by positive immunohistochemical staining for desmin and myogenin. To our knowledge, this is the first case of urethral genital-type rhabdomyoma in a child.
心脏外横纹肌瘤是罕见的良性实体瘤，通常发生在头颈部。虽然已知生殖系统横纹肌瘤发生在年轻和中年女性的下生殖道，但横纹肌瘤累及解剖上相邻的尿道极为罕见。我们报告一例发生于女婴尿道的生殖系统横纹肌瘤。肿瘤的特征是黏膜下存在成熟的横纹肌母细胞，含有明显的横纹，细胞位于胶原基质中。无坏死和有丝分裂。肿瘤细胞的骨骼肌分化通过肌红蛋白和结蛋白的免疫组织化学染色阳性得到支持。据我们所知，这是首例儿童尿道生殖型横纹肌瘤。
