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

Abstract

We report a case of a 4-year-old girl with an ovarian steroid cell tumor, not otherwise specified (SCT-NOS). She was admitted to the hospital with progressing virilization and Cushing's syndrome, which included abnormality of the perineum, hirsutism, hypertrichosis, flushing of face, hoarseness, and weight gain. Blood testing showed a significantly increased testosterone level and slightly increased cortisol level. Computed tomography scan revealed an 8.0 × 5.0 × 5.0 cm tumor of the right ovary. The patient underwent right salpingo-oophorectomy, and pathological examination showed malignant potential. Three courses of bleomycin, etoposide, and cisplatin were administered as postoperative chemotherapy. After tumor resection, her testosterone decreased to undetectable levels. However, during the course of the treatment, the patient suffered from adrenal insufficiency resulting in the need for hydrocortisone replacement therapy. Although SCT-NOS in childhood are typically benign, pathological findings should be carefully observed for potential malignancy. In cases of cortisol-producing SCT-NOS, serum levels should be monitored, and hydrocortisone replacement therapy should be considered before resection.

Ovarian steroid cell tumor causing isosexual pseudoprecocious puberty in a young girl: an instructive case and literature review

“…Nevertheless, reported cases in children were so scarce that their typical features remained elusive. For better understanding, previously reported children cases of SCTs (n = 15) were thoroughly reviewed (Table 2) [2,[4][5][6][7][8][9][10][11][12][13][14][15]. After excluding the three cases without documented clinical manifestation and one case presenting only with mass effect, a total of 12 patients including the present case were analyzed (Table 3) [4][5][6][7][8][10][11][12][13][14][15].…”

“…For better understanding, previously reported children cases of SCTs (n = 15) were thoroughly reviewed (Table 2) [2,[4][5][6][7][8][9][10][11][12][13][14][15]. After excluding the three cases without documented clinical manifestation and one case presenting only with mass effect, a total of 12 patients including the present case were analyzed (Table 3) [4][5][6][7][8][10][11][12][13][14][15]. Most of them showed heterosexual precocity (66%) with symptoms of virilization (50%), hirsutism (25%), amenorrhea (17%), hypertrichosis (17%), facial acnes (17%) and temporal balding (8%).…”

“…On the contrary, isosexual precocity accounted for only 33% of all cases and the predominant symptoms were early breast development (33%), followed by vaginal bleeding (25%) and nipple pigmentation (8%). Other non-specific symptoms irrelevant to sex hormone included Cushing's syndrome (33%) and hypertension (17%)(Table 3) [4][5][6][7][8][10][11][12][13][14][15]. Herein, improved understanding of aforementioned features will add a new dimension to the precise management of ovarian SCTs.…”

Failure of multiple surgical procedures and adjuvant chemotherapy in early-stage steroid-cell ovarian tumor treatment: a case report and literature review

“…The adjuvant chemotherapy regimens currently recommended for treatment are as follows: BEP; cisplatin, doxorubicin, and cyclophosphamide; taxane and platinum; and bleomycin, vinblastine, and cisplatin. 23 – 27 It was reported that intraperitoneal dissemination and liver metastases were completely removed with debulking surgery, radiofrequency ablation of the liver metastasis, and adjuvant BEP (follow-up, 43 months) in a patient with a recurrent SCT-NOS occurring 5 years after the initial surgery. 28 In another study, the treatment protocol consisted of docetaxel and nedaplatin, and the patient survived for 2 years with multiple bone metastases.…”

Ovarian Steroid Cell Tumor Masquerading as Steroid-Unresponsive Congenital Adrenal Hyperplasia

“…Yılmaz-Ağladıoğlu et al 7 reported a 13-year-old female who experienced a 6 year delay in diagnosis of SCT, NOS, by ultrasound due to presumed CAH. Yoshimatsu et al 10 documented an ovarian tumor, diagnosed as SCT, NOS, visualized on CT and MRI in a 4-year-old girl with virilization. These cases differed from ours as diagnostic imaging studies were positive.…”