Youngest reported patient presenting with an androgen producing sclerosing stromal ovarian tumor

Abstract

Background: 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.

Case: We report a 9-year-old female who was diagnosed with this ovarian tumor, and who presented with virilization.

Summary and conclusion: 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.

Keywords: Androgens; Ovarian tumor; Puberty; Sclerosing stromal tumor; Virilization.