Yen Elizabeth, Deen Malik, Marshall Ian
Department of Pediatrics, Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
Department of Pathology, Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
J Pediatr Adolesc Gynecol. 2014 Oct;27(5):e121-4. doi: 10.1016/j.jpag.2013.09.007. Epub 2014 Mar 19.
Sclerosing stromal tumors are extremely rare sex cord stromal tumors of the ovary, with approximately 100 cases reported since first described in 1973. These tumors present predominantly in the 2nd and 3rd decades of life, typically present with pelvic/abdominal pain and tenderness, mass, and/or abnormal menses, and with hormonal activity reported predominantly in postmenarchal females. Only 5 cases of these tumors have been reported in premenarchal girls, with age ranging from 7 months to 12 years. Only 2 demonstrated hormonal manifestations, with vaginal bleeding due to hyperestrogenism in the 7 month old, and virilization in an 11-year-old female.
We report a 9-year-old female who was diagnosed with this ovarian tumor, and who presented with virilization.
This report is remarkable as our patient not only was diagnosed with an ovarian tumor that is extremely rare in this age group but is the youngest reported patient with this tumor who presented with virilization.
硬化性间质瘤是极其罕见的卵巢性索间质肿瘤,自1973年首次描述以来,报告的病例约有100例。这些肿瘤主要发生于生命的第二个和第三个十年,通常表现为盆腔/腹部疼痛和压痛、肿块和/或月经异常,且激素活性主要见于初潮后的女性。初潮前女孩中仅报告了5例此类肿瘤,年龄范围为7个月至12岁。仅有2例表现出激素异常,1例7个月大的女孩因雌激素过多出现阴道出血,另1例11岁女性出现男性化。
我们报告1例9岁女性,被诊断为此种卵巢肿瘤,并出现男性化表现。
本报告值得关注,因为我们的患者不仅被诊断出患有该年龄组极其罕见的卵巢肿瘤,而且是报告中出现男性化表现的该肿瘤患者中最年轻的。