Sclerosing stromal tumor of the ovary with masculinization, Meig’s syndrome and CA125 elevation in an adolescent girl: A case report

BACKGROUND: Sclerosing stromal tumor (SST) is an extremely rare sex cord stromal tumor of the ovary. It was first reported and named in 1973. These tumors typically present with pelvic/abdominal pain and tenderness, a mass, and/or abnormal menses, but rarely present with masculinity in children and adolescents. Only 2 cases of these tumors have been reported in premenarchal girls, who demonstrated hormonal activity, with a history of the development of a virilizing female due to hyperandrogenism. Here, we report a case of a giant SST with obvious masculinity combined with Meig’s syndrome and CA125 elevation. CASE SUMMARY: A 17-year-old female presented with a 7-year history of the development of masculinity and a 2-year history of amenorrhea. She had hirsutism, acne, obvious laryngeal prominence, and voice deepening. Physical examination showed a male suprapubic hair pattern and a 4.0 cm × 1.5 cm enlarged clitoris. Laboratory tests showed that the testosterone level was > 15.00 ng/mL (normal range: 0.14-0.76 ng/mL), and androstenedione level was > 10.00 ng/mL (normal range: 0.3-3.3 ng/mL). A computed tomography scan of the abdomen and pelvis was carried out and showed a large, solid and cystic, partly calcified pelvic mass in the right ovary measuring 27.1 cm × 20.0 cm × 11.0 cm, 15 cm above the umbilicus (to the level of the upper part of L1). Intraoperative findings at laparotomy revealed a large tumor arising from the right ovary. Approximately, 500 mL of pale-yellow clear liquid was found in the pelvic cavity. A right salpingo-oophorectomy was performed. Microscopic examination and immunohistochemical staining of the surgical specimen showed an SST of the ovary. CONCLUSION: This report is remarkable as our patient was not only diagnosed with an SST of the ovary, which is extremely rare in this age group, but was the largest and most obvious reported patient with this tumor who presented with virilization. Therefore, gynecologists should be aware of this potential complication in adolescent girls with a mass in the ovary.