Ovarian steroid cell tumor, not otherwise specified: A case report and literature review

Abstract

Steroid cell tumors (SCT), not otherwise specified (NOS) are particularly rare ovarian sex cord-stromal tumors, which comprise <0.1% of all ovarian tumors. These tumors are uncommon in patients' prior to puberty without any typical syndromes involving hirsutism, virilization and hypertension. We here in present the case of a 5-year-old female patient who presented with sudden abdominal pain, repeated vomiting and a pelvic mass. Our patient underwent urgent exploratory laparotomy and right salpingo-oophorectomy and the histopathological examination revealed an ovarian SCT-NOS. The patient has been followed up for 5 years since the surgery, without evidence of disease recurrence. The purpose of this study was to discuss the available information on the presentation, diagnosis and recommended treatment of ovarian SCT-NOS; and describes the immunohistochemical characteristics of these tumors.

Keywords: not otherwise specified; ovarian steroid cell tumors.

Figure 1.

Hematoxylin and eosin (HE) staining of the ovarian tumor, not otherwise specified. The tumor was composed of cells with abundant eosinophilic to clear cytoplasm and round nuclei with prominent nucleoli (magnification, ×160).

Figure 2.

Immunohistochemical examination of the ovarian tumor (magnification, ×160). The tumor cells were positive (brown staining) for inhibin, vimentin (vim), progesterone receptor (PR), calretinin (CR), somatostatin (SMA), synaptophysin (Syn), CD99 and weakly positive for D2-40, ER, estrogen receptor; CEA, carcinoembryonic antigen; CK, cytokeratin; HMB45, human melanoma black 45; Plap, placental alkaline phosphatase.