Sertoli–Leydig cell tumor of the ovary: Analysis of a single institution database and review of the literature

Abstract

Aim To evaluate the clinical characteristics and outcome of ovarian Sertoli–Leydig cell tumors (SLCTs) managed at a single institution. Methods The hospital records of 17 patients with the diagnosis of ovarian SLCT between 1994 and 2018 were reviewed retrospectively. Results The median age of the patients was 30 years (range, 18–67 years). All the patients had unilateral tumors. All of the 17 were stage 1 tumors. Two (11.8%) patients were stage 1C1 and two (11.8%) patients were stage 1C2. Thirteen (76.5%) patients were stage 1A. Three (17.6%) of the tumors were well differentiated, 11 (64.7%) were intermediately differentiated, 1 (5.9%) was poorly differentiated, and the degree of the differentiation was not identified for 2 (11.8%) patients. One showed retiform pattern and one had heterologous elements at the histopathologic evaluation. Among the 17 patients, we identified structural/vascular renal and ureteral anomalies in 3 (17.6%) patients. Eight patients underwent total abdominal hysterectomy and bilateral salpingo‐oophorectomy, seven underwent unilateral salpingo‐oophorectomy or oophorectomy and two underwent cystectomy with or without additional surgical staging procedures. Four patients received adjuvant chemotherapy. All the 17 patients were alive and free of disease for 1–287 months after the diagnosis. Median follow‐up time was 78 months. None of the patients recurred. Conclusion Sertoli–Leydig cell tumors are rare ovarian malignancies with low recurrence rates and have a favorable outcome compared to malignant epithelial tumors of the ovary. Main treatment is surgical resection and it is appropriate to prefer fertility sparing conservative surgeries for young patients.

Outcomes after Fertility-sparing Surgery for Women with Ovarian Cancer: A Systematic Review of the Literature

“…With regard to SCST, we identified 8 studies [95−102], of which 3 investigated SCST [100−102], 1 studied Sertoli-Leydig tumor exclusively [97], and the remaining 4 included only adult granulosa cell tumor [95,96,98,99]. In 6 of these studies, FSS was not associated with worse progression-free survival or OS than with conventional surgery [96−101].…”

Sex Maintenance in Mammals

“…Cell transdifferentiation has also been described in cases of human gonadal cancers. The Sertoli-Leydig cell tumor (SLCT) of the ovary is a rare type of tumor normally affecting middle-aged women, which is characterized by the presence of testicular structures including Sertoli-like cells and Leydig cells that produce androgens [ 38 ]. On the other hand, cases of granulosa-cell tumors have also been reported in which neoplastic proliferation of intratubular sex cord cells progresses to an invasive tumor, simultaneously experiencing granulosa cell differentiation and losing Sertoli cell features [ 39 , 40 ].…”

Genetics and Mutational Landscape of Ovarian Sex Cord-Stromal Tumors

“…Sertoli-Leydig cell tumors (SLCT) also called androblastomas and arrhenoblastomas, exhibit cellular and molecular markers consistent with a dysgenesis of the ovarian stromal cells, reminiscent of disorders of gonadal dysgenesis [14]. They are rare, accounting for less than 0.5% of all ovarian cancers [3] and can occur in women of all age groups, but they are more often encountered in women under 40 years of age [15]. Patients usually present with symptoms related to androgen excess but can also present with estrogenic manifestations or have an asymptomatic clinical profile.…”

“…Patients usually present with symptoms related to androgen excess but can also present with estrogenic manifestations or have an asymptomatic clinical profile. SLCT are typically unilateral tumors and over 97% are diagnosed at Stage 1 [3,15]. The prognosis is correlated with the degree of differentiation and stage of the tumor with the five year survival rate of well differentiated SLCT being ~100% [3].…”

“…Further, Caburet et al combined transcriptomic data from a previous study [62], seeking to identify gene copy number changes that may reflect putative driver changes in the pathogenesis of aGCT [61]. Twenty genes were identified from the regions of chromosomal imbalance with a plausible, pathological role across nine chromosomes (1,5,11,12,(14)(15)(16)(17)22) including the AKT1 gene being the most frequently amplified (6 of 10 tumors) and the nuclear receptor, rev-erbAα being the second most frequent (5 of 10 GCT). The latter is consistent with the findings of our previous study examining gene expression of all 48 nuclear receptors in aGCT [63].…”