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

Abstract

Ovarian steroid-cell tumors (SCTs) are a rare subgroup of sex-cord tumors of the ovary, accounting for less than 0.1% of all ovarian tumors. Not otherwise specified (NOS) tumors are the most common subtype. More than half of patients with SCTs-NOS show hyperandrogenic symptoms. The primary treatment for SCTs is surgery, as most cases are early-staged and benign. Because of the low incidence of metastatic disease, there is insufficient reliable information on the role of adjuvant therapy and the most effective treatment regimen. In this report, a rare case of a recurrent SCT-NOS in a 36-year-old female patient without endocrine symptoms is presented, highlighting the significance of appropriate pathological evaluation and immunohistochemical testing for the accurate diagnosis of this malignancy, particularly in the case of hormonally “silent” tumors. The metastatic tumor described here showed no response to four courses of adjuvant chemotherapy after several debulking surgeries. Based on the clinical findings, the neoplastic etiology should always be considered during the resection of ovarian tumors to prevent possible disease dissemination due to inappropriate surgical techniques.

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

“…However, further studies on more cases of different ages are warranted to confirm our aforementioned findings. Among SCTs adults, more than half of them presented with hyperandrogenic signs such as hirsutism, acne, deepened voice, clitoromegaly, amenorrhea, and infertility [3]. Only a few remaining cases exhibited hyperestrogenism in terms of menorrhagia, postmenopausal bleeding, sometimes even endometrial cancer [3].…”

“…Among SCTs adults, more than half of them presented with hyperandrogenic signs such as hirsutism, acne, deepened voice, clitoromegaly, amenorrhea, and infertility [3]. Only a few remaining cases exhibited hyperestrogenism in terms of menorrhagia, postmenopausal bleeding, sometimes even endometrial cancer [3]. Nevertheless, reported cases in children were so scarce that their typical features remained elusive.…”

“…As expected, clinical symptoms of SCTs could remit a few months later. In summary, SCTs are rare tumors but usually benign [3,8], and could be effectively managed with surgical intervention when prompt and precise diagnosis was made.…”

Somatic gene mutations in malignant steroid cell tumours and response to multiple treatments

“…Analyses of gene mutations in ovarian interstitial tumours have identified FOXL2 mutations in adult ovarian granulosa cell tumours and DICER1 mutations in Sertoli-Leydig cell tumours22 23; however, no study has used NGS oncology panels to investigate the mutational status in malignant ovarian steroid cell tumours. The treatment for tumours harbouring the identified gene mutations has not been established 1 5…”

“…Malignant ovarian steroid cell tumours are a rare subgroup of sex cord-stromal tumours, accounting for less than 0.1% of all ovarian tumours. Although mostly benign, these tumours may cause virilisation 1. Steroid cell tumours can be divided into stromal luteoma, Leydig cell tumours and steroid cell tumours not otherwise specified (NOS) that arise from an unknown location.…”