Altchek Albert, Deligdisch Liane, Norton Karen, Gordon Ronald, Greco M Alba, Magid Margret S
Department of Obstetrics, Gynecology and Reproductive Science, Mount Sinai School of Medicine and Hospital, New Yourk, NY, USA.
Obstet Gynecol. 2007 Jul;110(1):103-8. doi: 10.1097/01.AOG.0000266979.42237.cc.
To report and propose a consensus term for eight cases of a newly recognized, asymptomatic, rapidly growing unilateral labium majus mass without palpable borders in prepubertal girls, appearing to be temporally associated with the physiologic increase of adrenal hormones.
Histologic examination, special stains, and immunohistochemistry were performed on all cases. In our personal cases, electron microscopy and chromosomal analysis were also performed, together with pelvic magnetic resonance imaging (MRI), inguinal exploration, vaginoscopy with biopsies, and adrenal hormone levels.
Of the eight cases, seven were Ashkenazi Jewish girls from the same area in New York City. The unilateral masses were asymptomatic, soft, without palpable borders. The overlying skin had a slightly tan peau d'orange surface. The masses could not be completely excised because they extended into the contiguous pelvic floor. Histologically, the masses were composed of bland hypocellular fibrous tissue extending into the deep subcutaneous tissue. The masses blended into the surrounding tissue and adjacent pelvic floor as shown by MRI preoperatively and postoperatively. Residual tissue did not progress after incomplete resection.
These fibrous lesions develop in months at the time of physiologic increase in adrenal hormone secretion just before puberty and subsequently appear to stop growing. The surgeon should not attempt a complete removal but simply excise sufficient tissue for a reasonable cosmetic result and to confirm the diagnosis. The lesions reflect fibroblastic hyperplasia, which is possibly hormone-driven. The ethnic and geographic clustering of cases raises consideration of environmental exposures or genetic predisposition.
报告并提出一个共识术语，用于描述8例青春期前女孩新发现的、无症状、生长迅速的单侧大阴唇肿物，肿物无明显边界，似乎与肾上腺激素的生理性增加在时间上相关。
对所有病例进行组织学检查、特殊染色和免疫组化。在我们的个人病例中，还进行了电子显微镜检查、染色体分析，以及盆腔磁共振成像（MRI）、腹股沟探查、阴道镜检查及活检和肾上腺激素水平检测。
8例病例中，7例为来自纽约市同一地区的阿什肯纳兹犹太女孩。单侧肿物无症状，质地柔软，无明显边界。肿物表面皮肤呈轻度棕褐色橘皮样外观。肿物因延伸至邻近的盆底而无法完全切除。组织学上，肿物由淡染、细胞稀少的纤维组织构成，延伸至深部皮下组织。术前和术后MRI显示，肿物与周围组织及邻近盆底融合。不完全切除后残留组织未进展。
这些纤维性病变在青春期前肾上腺激素分泌生理性增加时数月内形成，随后似乎停止生长。外科医生不应试图完全切除，只需切除足够的组织以获得合理的美容效果并确诊即可。这些病变反映了成纤维细胞增生，可能由激素驱动。病例的种族和地理聚集引发了对环境暴露或遗传易感性因素的思考。
