Ovarian sex cord-stromal tumors: an update on clinical features, molecular changes, and management

Harbi, Rehab Al; McNeish, Iain A.; El‐Bahrawy, Mona

doi:10.1136/ijgc-2020-002018

Cited by 46 publications

(48 citation statements)

References 105 publications

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“…However, this could also reflect differences in the set of mutated genes responsible for SLCT. In particular, SLCT is known to be associated with somatic and germinal mutations of DICER1, and has been identified in 50-60% of SLCT [43][44][45][46][47]. Tumours with DICER1 mutations appear to express more androgenic manifestations, develop at a younger age and behave more aggressively [44,[46][47][48].…”

Section: Discussionmentioning

confidence: 99%

“…In particular, SLCT is known to be associated with somatic and germinal mutations of DICER1, and has been identified in 50-60% of SLCT [43][44][45][46][47]. Tumours with DICER1 mutations appear to express more androgenic manifestations, develop at a younger age and behave more aggressively [44,[46][47][48]. Recent studies have reported ovarian metachronous SLCT in a germline DICER1 population, and a significantly higher proportion of DICER1 mutations in the younger population [38,49].…”

Section: Discussionmentioning

confidence: 99%

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Ovarian Sertoli–Leydig cell tumours: A systematic review of relapsed cases

Nef

Huber

2021

European Journal of Obstetrics & Gynecology and Reproductiv

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Objective: To synthesize the evidence on Sertoli-Leydig cell tumour (SLCT) relapses, and identify the clinicopathological characteristics and prognosis of patients with recurrent SLCT. Methods: A literature search was undertaken of all published cases of SLCT relapse found in PubMed, Embase and Web of Science databases between January 1998 and January 2021. All articles in English reporting at least one case of SLCT relapse and mentioning the relapse location or the follow-up data were included. All reported data on relapsed cases were extracted. Student's t-test and Chi-squared test were used for the descriptive analysis, and the Kaplan-Meier statistical method was applied for survival analysis. Results: Eighty-five patients from 33 articles were included in this review. The median age was 20 years (range 3-76 years) with a median time to relapse of 14 months (range 1-168 months). Forty-eight percent (36/75) of relapses were local and 52% (39/75) were distant. In the subgroup of conservative primary surgery, contralateral ovarian SLCT events (metachronous or recurrent) were more frequent in the paediatric population than in the adult population (58.3 vs 18.2%; p = 0.005). Eleven cases had multiple relapses. Twenty-one percent (12/57) of cases were treated with conservative surgery after recurrence, and 64.9% (37/57) of cases were treated with radical surgery which tends to have a better 2-year survival rate (78.5% vs 61.0%; p = 0.177). Overall median survival was 48 months after recurrence (95% confidence interval ±21.0 months) with overall 5-year survival of 38.9%. The mean survival time was significantly higher for patients diagnosed at an early stage (I and II) compared with patients diagnosed at an advanced stage (p = 0.003). Discussion: The results showed that SLCT relapses have a poor prognosis and occur mainly in young patients, soon after the initial diagnosis. The majority of SLCT relapses are located in the abdominopelvic region. Contralateral ovarian SLCT events (metachronous or recurrent) occurred more frequently in paediatric cases. Multi-modal treatment with surgery and chemotherapy appears to be the best approach. The best chemotherapeutic regimen has yet to be defined.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Ovarian Sertoli–Leydig cell tumours: A systematic review of relapsed cases

Nef

Huber

2021

European Journal of Obstetrics & Gynecology and Reproductiv

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“…Sex cord-stromal tumors are classified into three main groups according to the WHO, and include pure stromal tumors, pure sex cord tumors, and mixed sex cord stromal tumors[ 7 ]. OSCTs belongs to a subtype of pure stromal tumors and is an ovarian tumor composed entirely or mostly of cells that secrete steroid hormones.…”

Section: Discussionmentioning

confidence: 99%

Postmenopausal women with hyperandrogenemia: Three case reports

Zhu¹,

Zhou²,

Jiang³

2021

WJCC

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BACKGROUND Diagnosing hyperandrogenemia in postmenopausal women is very difficult. It occasionally manifests as excessive hair growth or with no clinical manifestations, and is therefore often misdiagnosed or missed altogether. Ovarian steroid cell tumors that cause hyperandrogenemia in women account for approximately 0.1% of all ovarian tumors. Due to the low incidence, corresponding imaging reports are rare, so ovarian steroid cell tumors lacks typical imaging findings to differentiate it from other ovarian tumors. Therefore, we summarized its clinical and imaging characteristics through this case series, and elaborated on the differential diagnosis of steroid cell tumors. CASE SUMMARY We report three cases of postmenopausal women with hyperandrogenemia. Only 1 patient showed virilization symptoms, the other two patients were completely asymptomatic. All patients underwent total hysterectomy + bilateral adnexectomy. Histological results showed one case of Leydig cell tumor and two cases of benign, non-specific steroid cell tumor. After the operation, the androgen levels of all patients returned to normal, and there was no clinical recurrence since follow-up. CONCLUSION Although virilization caused by increased serum testosterone levels is an important clinical feature of ovarian steroid cell tumors, it is often asymptomatic. A solid, slightly hypoechoic, round or oval mass with uniform internal echo, richer blood flow in the solid part, and low resistance index are typical imaging features of ovarian steroid cell tumors. Diagnosis of ovarian steroid cell tumors after menopause is challenging, but surgery can be used for both diagnosis and clear treatment.

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“…Sex cord-stromal tumors of the ovary comprise a rare and enigmatic group of neoplasms defined by a beguiling paradox-indolent-behaving diseases that can nevertheless present serious clinical challenges in the advanced or recurrent setting. Al-Harbi et al 1 set out to synthesize and distil an extensive literature covering the epidemiology, treatment outcomes, and molecular pathogenesis of this heterogeneous group of neoplasms for which the published literature is farranging. In this attempt, they should be commended, even though several seminal publications failed to receive mention in their review.…”

Section: Gynecological Cancermentioning

confidence: 99%