Benign and malignant ovarian steroid cell tumors, not otherwise specified: case studies, comparison, and review of the literature

Abstract

Ovarian steroid cell tumors, not otherwise specified (NOS) are rare sex cord-stromal tumors of the ovary with malignant potential. So far only a few cases were reported in English literature through the Pubmed search. Here we report two cases of such tumor, one was benign (first case underwent laparoscopic cystectomy) and the other was malignant (died 10 months later after initial diagnosis), both presented with amenorrhea and clinical signs or symptoms of virilization. In malignant case, we provided evidence (tumor embolus) in addition to the reported five characteristics associated with malignancy. On further evaluation, laboratory investigations revealed hyperandrogenism in the male range, while follicle stimulating hormone (FSH) and luteinising hormone (LH) levels were within normal limits. Various aspects of the presentation, diagnosis, and treatment of these tumors are discussed.

Figure 1

Benign ovarian steroid cell tumor, NOS. (A) Macroscopic appearance: Note the well-circumscribed, solid, yellow-brown tumor. (B) Microscopic appearance: cells with small round nuclei, mild atypia, and no mitosis are arranged in a diffuse pattern of columns or nests separated by a rich vascular network. The cells demonstrate abundant pale cytoplasm and no crystals of Reinke (H&E, 20×).

Figure 2

The dynamic changes of the patients’ serum testosterone level before or after surgery.

Figure 3

Malignant Steroid cell tumor, NOS. Microscopic appearance: Cells with irregular nuclei or atypia (A, H&E, 40×), and mitosis (B, H&E, 20×) are arranged in a diffuse pattern of columns or nests separated by a rich vascular network. Diffuse hemorrhage and necrosis are observed (C, H&E, 20×). The presence of intravascular tumor embolus further supports the diagnosis of malignancy (D, H&E, 10×).