Dataset Information

Oncological Prognosis and Fertility Outcomes of Different Surgical Extents for Malignant Ovarian Sex-Cord Stromal Tumors: A Narrative Review.

ABSTRACT:

Background

Malignant ovarian sex-cord stromal tumors (MOSCSTs) are rare neoplasms that account for approximately 5-7% of all ovarian malignancies. The majority (70%) of patients had an early stage; thus, surgery is the predominant treatment. Patients were relatively young at the onset of the tumor. Moreover, the prognosis of patients with this tumor is better than that of malignant epithelial ovarian tumors and tends to recur late with an indolent clinical course. Thus, patients may be more inclined to conservative surgical procedures. There is, however, no objective criterion for selecting a suitable surgical procedure. Clinically, surgical extent depended on the preoperative evaluations, age, and willingness of patients, and gynecologists were relatively subjective when choosing surgery. The prognosis of patients with different surgical extents is still controversial. The review aimed to summarize the impacts of different surgical extents on oncological prognosis and fertility outcomes.

Methods

The literature search was performed in PubMed (https://www.ncbi.nlm.nih.gov/pubmed/), and publications between January 2011 and December 2021 in English including clinical cohort studies and case reports were eligible for inclusion.

Results

We finally identified 12 large-sample retrospective studies and 18 cases of MOSCSTs. The primary surgical procedures include fertility-spring surgery (FSS), total hysterectomy with unilateral or bilateral salpingo-oophorectomy (TAH-USO/BSO), FSS with complete staging procedure, complete staging surgery (CSS), and debulking surgery. FSS includes cystectomy (CYS), unilateral salpingo-oophorectomy (USO) or bilateral salpingo-oophorectomy (BSO) with uterine preservation that allows for potential future assisted reproductive approaches. Complete staging procedure includes peritoneal cytologic examinations, inspections of peritoneal surfaces, random peritoneal biopsies and omentectomy. FSS with complete staging procedure means surgical procedure with uterine preservation and complete staging procedure. And, generally, CSS means TAH-BSO with complete staging procedure.

Conclusion

It can be concluded that USO can be done in young, fertility-desired patients with tumors confined to the ovary but avoid CYS. FSS with complete staging procedure is feasible among stage IC-III patients who have fertility desire. Patients can choose to have a complete surgery once their family is complete or without fertility requirements. CSS is recommended for patients with risk factors such as high stage, poor differentiation, and large tumor size and without fertility desire. A close follow-up is essential.

SUBMITTER: Li J

PROVIDER: S-EPMC8864408 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature

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Publications

Oncological Prognosis and Fertility Outcomes of Different Surgical Extents for Malignant Ovarian Sex-Cord Stromal Tumors: A Narrative Review.

Li Jiawei J Li Jun J Jiang Wei W

Cancer management and research 20220218

<h4>Background</h4>Malignant ovarian sex-cord stromal tumors (MOSCSTs) are rare neoplasms that account for approximately 5-7% of all ovarian malignancies. The majority (70%) of patients had an early stage; thus, surgery is the predominant treatment. Patients were relatively young at the onset of the tumor. Moreover, the prognosis of patients with this tumor is better than that of malignant epithelial ovarian tumors and tends to recur late with an indolent clinical course. Thus, patients may be m ...[more]

PMID: 35221723

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Project description:Sertoli-Leydig cell tumors are characterized by the presence of somatic DICER1 hotspot mutations. In this study, we sought to define the association between DICER1 hotspot mutations and different morphologic subtypes of ovarian Sertoli-Leydig cell tumors. Furthermore, we aimed to assess whether DICER1 hotspot mutations occur in other ovarian sex cord-stromal tumors, testicular sex cord-stromal tumors, or other female genital tract tumors with rhabdomyosarcomatous differentiation. We subjected a series of ovarian Sertoli-Leydig cell tumors (n=32), Sertoli cell tumors (n=5) and gynandroblastomas (n=5), testicular sex cord-stromal tumors (n=15) and a diverse group of female genital tract tumors with rhabdomyosarcomatous morphology (n=10) to DICER1 hotspot mutation analysis using Sanger sequencing. We also tested two gynandroblastomas for the presence of FOXL2 hotspot mutations (p.C134W; c.402C>G). Twenty of 32 (63%) Sertoli-Leydig cell tumors harbored a DICER1 hotspot mutation, of which 80% had the p.E1705K mutation. No association was found between DICER1 mutation status and the presence of heterologous or retiform differentiation in Sertoli-Leydig cell tumors. DICER1 mutations were found at similar frequencies in gynandroblastoma (2/5; 40%) and ovarian Sertoli cell tumors (5/8; 63%; P>0.1), and all mutated tumors harbored a p.E1705K mutation. DICER1 hotspot mutations were also identified in a single cervical rhabdomyosarcoma and in the rhabdomyosarcomatous component of a uterine carcinosarcoma. No DICER1 mutations were detected in testicular sex cord-stromal tumors. Two DICER1 wild-type gynandroblastomas harbored a p.C134W FOXL2 hotspot mutation in both tumor components. In this study we confirmed that DICER1 hotspot mutations occur in over half of ovarian Sertoli-Leydig cell tumors, and are unrelated to tumor differentiation. We also widened the spectrum of ovarian sex cord-stromal tumors with sertoliform differentiation, in which DICER1 mutations are known to occur, to include Sertoli cell tumors and gynandroblastomas. Our results suggest that DICER1 mutations may not have a role in testicular sex cord-stromal tumorigenesis.

Project description:BackgroundFertility-sparing surgery (FSS) has been widely used for patients with early-stage mucinous ovarian carcinoma (MOC). However, there is limited evidence regarding the reproductive outcomes as well as the impact of growth pattern on oncological outcomes after FSS. This study aims to evaluate the oncological and reproductive outcomes of patients with stage I primary MOC after FSS.MethodsThis retrospective study enrolled 159 women with histologically confirmed unilateral stage I MOC treated at Peking Union Medical College Hospital between 1997 and 2019. Sixty-seven cases were pathologically reviewed for the growth pattern. Seventy-eight patients had FSS, defined as conservation of the uterus and at least part of one ovary, while 81 underwent radical surgery (RS). Oncofertility outcomes were compared between the groups and clinicopathological factors associated with disease-free survival (DFS) were analyzed by univariate and multivariate analyses. Patients in the FSS group were contacted to collect data on reproductive outcomes.ResultsEighteen patients developed recurrent disease during a median follow-up of 69 months, including 12 in the FSS and six in the RS group. There was one death each in the FSS and RS groups. There was no significant difference in DFS between the groups. CA125 >35 U/ml, stage IC, and incomplete staging were correlated with worse DFS according to multivariate analysis (P=0.001; 0.020 (stage IC) and 0.004 (incomplete staging) respectively). There was no significant difference in DFS between patients with stage IA and stage IC1 in the FSS group, while DFS was poorer in patients with stage IC2/3 than stage IA (P=0.028). In addition, DFS was significantly poorer in patients who underwent unilateral salpingo-oophorectomy (USO) compared with those receiving USO plus staging surgery (P=0.015). There was a tendency towards poorer DFS in the infiltrative tumors compared with the expansile tumors (P=0.056). Of 23 patients who attempted to conceive, 21 (91.3%) achieved 27 pregnancies, including 26 spontaneous pregnancies and one following assisted reproductive technology. Twenty patients gave birth to 24 healthy babies, including 21 full-term and three premature births. The live-birth rate was 88.9%.ConclusionsFSS is a suitable option for young women with unilateral stage I expansile MOC, with acceptable oncological outcomes and meaningful pregnancy rates. Re-staging should be proposed in patients who undergo incomplete staging surgery.

Dataset Information

Oncological Prognosis and Fertility Outcomes of Different Surgical Extents for Malignant Ovarian Sex-Cord Stromal Tumors: A Narrative Review.

Background

Methods

Results

Conclusion

Publications

Oncological Prognosis and Fertility Outcomes of Different Surgical Extents for Malignant Ovarian Sex-Cord Stromal Tumors: A Narrative Review.

Similar Datasets

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