Steroid cell tumors of the ovary are extremely rare, accounting for only 0.1% of all ovarian tumors. Of these, steroid cell tumors, not otherwise specified (NOS) constitute about 56% of all steroid cell tumors. Most steroid cell tumors secrete steroid hormones, and only about 10% to 15% of patients are asymptomatic. The morphology of steroid cell tumors, NOS is predominantly reported to be solid, and a review of case reports from 1979 until now revealed only 5 cases that were mainly cystic tumors. The present case, in a patient who had undergone a previous hysterectomy and surgery for a peritoneal inclusion cyst, is reported due to its rarity and its unusual presentation, together with a brief review of the literature. The tumor showed no clinical signs and symptoms typical of a steroid hormone secreting tumor and had an atypical morphology, being primarily multi-septate cystic with a minor solid portion.
Keywords: Cystic; Ovary; Steroid cell tumor.