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

Abstract

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.

Ovarian Steroid Cell Tumor (Not Otherwise Specified): A Case Report of Ovarian Hyperandrogenism

“…There are steroid cell tumors that cannot be categorized as either stromal luteomas or Leydig cell tumors. This group of tumors accounts for 60% of all ovarian steroid cell tumors [3][4][5].…”

“…The tumors can occur at any age with a mean age of 43 years, which is younger than other steroid tumors. They rarely occur before puberty [4]. Among the patients affected with SCT-NOS, 56-77% have symptoms of androgenic changes, such as hirsutism and virilization including acne, clitoral enlargement, deep voice, and alopecia.…”

“…Among the patients affected with SCT-NOS, 56-77% have symptoms of androgenic changes, such as hirsutism and virilization including acne, clitoral enlargement, deep voice, and alopecia. 6-23% have estrogenic manifestations such as menorrhagia, postmenopausal bleeding, or even endometrial carcinoma [4]. Only 6-10% are clinically associated with Cushing's syndrome, and 25% of SCT-NOS are nonfunctioning [5].…”

Malignant Ovarian Steroid Cell Tumor, Not Otherwise Specified, Causes Virilization in a 4-Year-Old Girl: A Case Report and Literature Review

“…This category of tumor accounts for only 1.3-2.3% of SCST and can occur at any age, although the mean age of occurrence is 43 years [2]. SCT-NOS in children and adolescents are extremely rare, with only about 9 cases found in a PubMed search [2][3][4][5][6][7][8][9][10][11]. In some pediatric SCT-NOS cases, the functional features of steroid hormone-secreting cells produce characteristic clinical signs of virilization and Cushing's syndrome.…”

“…In this report, we describe the case of a 4-year-old girl who presented with typical symptoms of SCT-NOS before puberty: abnormal height growth, virilization, and hirsutism. All reported pediatric cases of SCT-NOS have been benign in character; however, histopathological studies in this case revealed malignant features, resulting in the decision to administer postoperative chemotherapy [2][3][4][5][6][7][8][9][10][11]. SCT-NOS of the ovary should be considered in cases of childhood virilization.…”