(119) Sclerosing Stromal Ovarian Tumors: A Rare Cause of Primary Amenorrhea and Hyperandrogenism in Adolescents

Background: Sclerosing stromal tumors (SST) of the ovary are extremely rare sex cord-stromal tumors with about 200 cases reported in the literature. Most patients (70%) are between 14 and 29 years, and present with nonspecific symptoms of menstrual irregularity and pelvic pain. SSTs are usually benign, unilateral, well-circumscribed, non-hormone producing masses. This case describes a rare androgen-producing SST in an adolescent presenting with primary amenorrhea.

Case: A 17-year-old female with no significant medical history was referred to a pediatric and adolescent gynecology clinic for a complex right ovarian mass found during workup for primary amenorrhea. Thelarche and pubarche were at age 11.

The patient’s parent reported a deep voice since childhood and excess facial hair since age 10. She reported acne at age 16, worsening over one year to include her chest, back, and upper arms, as well as extensive hair on her legs and abdomen. She reported weight gain of 20 lbs over 4 months, constipation, bloating, and darkened skin in her axillae, beneath the breasts, and abdomen. She denied abdominal pain, urinary problems, clitoral enlargement or genital abnormalities.

Her pediatrician obtained laboratory studies (Table 1), which were remarkable for elevated 17-hydroxyprogesterone, androstenedione, and testosterone. Pelvic ultrasound noted a 10cm solid right ovarian mass, and pelvic MRI demonstrated a heterogenous mass concerning for carcinoma, with mixed internal components, fluid-intense cystic spaces, bridging septations, and internal enhancing soft tissue elements (Fig. 1).

She underwent exploratory laparotomy via Pfannenstiel incision, with pelvic washings, right salpingo-oophorectomy, and abdominal exploration for cancer staging. Pathologic analysis demonstrated a sclerosing stromal tumor, with no findings of malignancy. The patient recovered well from surgery. Repeat androgen testing is pending.

Comments: Hormone-producing sclerosing stromal tumors (SST) of the ovary are a rare but benign etiology to maintain in the differential diagnosis for adolescents with unilateral ovarian mass, hyperandrogenism, and menstrual abnormalities. Understanding SSTs is important due to the clinical and histopathologic resemblance to various ovarian neoplasms and is difficult to diagnose through pre-operative studies. Tumor markers, hormone testing, and pelvic imaging, should be performed for patients under the age of 30 with large solid tumors. All SST cases have been benign except for one patient with low-grade malignancy in 1990. Surgical resection of the tumor is curative, as no local or distant recurrences have been reported.