Endocrine Abstracts

Introduction

Steroid cell tumors (SCTs) are a rare subgroup of sex-cord tumors of the ovary that account for less than 0.1% of all ovarian tumors. They are classified into three categories based on the cell origin: stromal luteoma, Leydig-cell tumor, and not otherwise specified (NOS). NOS tumors are the most common subtype and comprise the largest proportion of cases (60%).The clinical presentations are not specific, including abdominal pain, distention and bloating. However, the more significant presentations are those associated with the hormonal activity and virilizing properties of the tumor. The cornerstone of SCT-NOS treatment is surgery.

Case

A 55-year-old female came to our hospital in November 2020 with months’ history of increasing facial and truncal hair. She also had hypertension and diabetes mellitus. In the medical history of the patient, she had been operated for papillary thyroid cancer, sleeve gastrectomy, total abdominal hysterectomy and left salpingo-oophorectomy 30 years ago. Physical examination revealed obesity (body mass index, 37.3 kg/m²).She had a Ferriman–Gallwey score of 24. Abdominal ultrasound identified a 40 × 20 mm solid, right ovarian mass.Biochemical data shows the levels of folicle stimulating hormone and luteinising hormone were as high as 52.1 U/l and 33.3 U/l, respectively. Serum adrenocorticotropic hormone, cortisol, estradiol, serum prolactin, dihydroepiandosterone sulfate were found within reference intervals.Total and free serum testosterone levels were found to be as high as 450 ng/dl (normal 7–49 ng/dl) and 19.99 (normal<4.2 pg/ml), respectively. Magnetic resonance imaging with contrast confirmed the ultrasound findings and detected no adrenal gland enlargement or tumor.The right salpingo-oophorectomy was done, and the specimen was sent to our department for histopathological examination. On surgical staging laparotomy, it was found that there was an enlarged (3×3×2 cm) right ovary with both solid and cystic component and normal ovarian contour.Microscopically, mitotic activity 2–3/10, mild atypia, and no significant necrosis. Immunohistochemistry revealed a result of MelanA(+), ER (+), CD56(+), Calretinin (+), Inhibin-α (+), ki-67 (5–6%+). Histological features were consistent with SCTs-NOS type.At post-operative follow-up, her total serum testosterone and serum free testosterone level had gone down to 13 ng/dl and 0.73 pg/ml, respectively.

Conclusion

SCTs, NOS, are rare ovarian tumors which can be difficult to diagnose.Careful history and physical examination, in addition to laboratory values and imaging studies, are helpful in making the diagnosis.They are usually benign, unilateral and are characterized by hyperandrogenism and virilization. SCTs should be considered in differential diagnosis among women presenting with symptoms of virilization.