Recurrent gynandroblastoma of the ovary with germline DICER1 mutation: A case report and review of the literature.

Sex cord-stromal tumors (SCSTs) are ovarian tumors that generally present with an adnexal mass and signs/symptoms of hormone excess. Gynandroblastoma is a rare subtype of SCST with a combination of female and male sex cord differentiation. These tumors typically present in premenopausal women and are diagnosed at early stages with benign clinical courses. Here, we present a rare case of recurrent gynandroblastoma in a premenopausal woman with a DICER1 germline mutation. The patient was referred to our clinic for new symptoms of hormonal imbalance with a history of ovarian juvenile granulosa cell tumor (JGCT). Evaluation revealed a 5x5cm complex right adnexal mass and rising inhibin B. Patient underwent total abdominal hysterectomy with right salpingo-oophorectomy, omentectomy and right pelvic and para-aortic lymphadenectomy. Pathology showed a right ovarian gynandroblastoma. Somatic biallelic mutations in the RNase IIIb domain of DICER1 were identified; a 23-gene germline panel confirmed a germline DICER1 pathogenic variant. Cascade testing of her children documented that both daughters inherited the pathogenic variant. Testing for DICER1 mutations has important implications for individual and familial tumor risk assessment given what we know about DICER1 mutation and increased childhood cancer risk.

Risk Stratification of Adnexal Cysts and Cystic Masses: Clinical Performance of Society of Radiologists in Ultrasound Guidelines.

Purpose To evaluate the performance of the 2010 Society of Radiologists in Ultrasound (SRU) consensus guidelines in the risk stratification of symptomatic and asymptomatic adnexal cysts. Materials and Methods An institutional review board-approved retrospective review was performed, including adnexal cysts detected with ultrasonography (US) with surgical diagnosis or at least 2 years of imaging or clinical follow-up, from January to June 2011. SRU management recommendations were scored as 0, no follow-up; 1, US follow-up; 2, magnetic resonance (MR) imaging follow-up; and 3, surgical evaluation. Distribution of outcomes (nonneoplastic cyst, benign neoplasm, malignant neoplasm) was compared in each rating group by using the Cochran-Armitage trend test. Where SRU guidelines allow more than one management option, they were classified as being interpreted in either a "surgically focused" environment, with limited MR imaging availability, or an "MR-capable" center, where MR imaging is selected whenever it is an option. Predictors of neoplasms and malignancy were evaluated by using multivariate logistic regression. Results A total of 570 cysts in 500 women aged 18-90 years (mean, 42 years) were included. There were 475 (83.3%) nonneoplastic cysts, 77 (13.5%) benign neoplasms, and 18 (3.2%) malignant neoplasms. Of the 500 women, 161 (32.2%) were asymptomatic. In the surgically focused interpretation of guidelines, proportions of any neoplasm and malignant neoplasm, respectively, were 1% and 0% in SRU 0, 17% and 1% in SRU 1, 48% and 0% in SRU 2, and 48% and 16% in SRU 3 (P < .0001 for both trends). In the interpretation of SRU guidelines with MR imaging when it was an option, proportions of any neoplasm and malignant neoplasm, respectively, were 1% and 0% in SRU 0, 17% and 1% in SRU 1, 38% and 5% in SRU 2, and 81% and 52% in SRU 3 (P < .0001, both trends) and 82 (89.1%) fewer benign cysts would have gone directly to surgical evaluation. In multivariate regression, SRU rating predicted both any neoplasm (odds ratio, 2.58; P < .0001) and malignant neoplasm (odds ratio, 4.94; P = .005). Conclusion SRU consensus guidelines effectively stratified the risk of neoplasia and malignancy. Selecting MR imaging when it is an option in the guidelines would have reduced the number of benign cysts sent for surgical evaluation. © RSNA, 2017.

Maintaining sexual health throughout gynecologic cancer survivorship: A comprehensive review and clinical guide.

OBJECTIVE: The diagnosis and treatment of gynecologic cancer can cause short- and long-term negative effects on sexual health and quality of life (QoL). The aim of this article is to present a comprehensive overview of the sexual health concerns of gynecologic cancer survivors and discuss evidence-based treatment options for commonly encountered sexual health issues. METHODS: A comprehensive literature search of English language studies on sexual health in gynecologic cancer survivors and the treatment of sexual dysfunction was conducted in MEDLINE databases. Relevant data are presented in this review. Additionally, personal and institutional practices are incorporated where relevant. RESULTS: Sexual dysfunction is prevalent among gynecologic cancer survivors as a result of surgery, radiation, and chemotherapy-negatively impacting QoL. Many patients expect their healthcare providers to address sexual health concerns, but most have never discussed sex-related issues with their physician. Lubricants, moisturizers, and dilators are effective, simple, non-hormonal interventions that can alleviate the morbidity of vaginal atrophy, stenosis, and pain. Pelvic floor physical therapy can be an additional tool to address dyspareunia. Cognitive behavioral therapy has been shown to be beneficial to patients reporting problems with sexual interest, arousal, and orgasm. CONCLUSION: Oncology providers can make a significant impact on the QoL of gynecologic cancer survivors by addressing sexual health concerns. Simple strategies can be implemented into clinical practice to discuss and treat many sexual issues. Referral to specialized sexual health providers may be needed to address more complex problems.

Burnout and associated factors among members of the Society of Gynecologic Oncology.

OBJECTIVE: Burnout is specific to the work domain and in physicians is indicative of emotional exhaustion, depersonalization in relationships with coworkers and detachment from patients, and a sense of inadequacy or low personal accomplishment. The purpose of this study was to determine the burnout rate among gynecologic oncologists and evaluate other personal, professional, and psychosocial factors associated with this condition. STUDY DESIGN: This study used a cross-sectional design. Current members of the Society of Gynecologic Oncology were sent an anonymous email survey including 76 items measuring burnout, psychosocial distress, career satisfaction, and quality of life. RESULTS: A total of 1086 members were invited, 436 (40.1%) responded, and 369 (84.6%) of those completed the survey. Of physicians, 30% scored high for emotional exhaustion, 10% high for depersonalization, and 11% low for personal accomplishment. Overall, 32% of physicians scored above clinical cutoffs indicating burnout. In all, 33% screened positive for depression, 13% endorsed a history of suicidal ideation, 15% screened positive for alcohol abuse, and 34% reported impaired quality of life. Nonetheless, 70% reported high levels of personal accomplishment, and results suggested most were satisfied with their careers, as 89% would enter medicine again and 61% would encourage their child to enter medicine. Respondents with high burnout scores were less likely to report they would become a physician again (P = .002) or encourage a child to enter medicine (P < .001), and more likely to screen positive for depression (P < .001), alcohol abuse (P = .006), history of suicidal ideation (P < .001), and impaired quality of life (P < .001). CONCLUSION: Burnout is a significant problem associated with psychosocial distress and lower levels of career satisfaction in gynecologic oncologists. Burnout in obstetrics-gynecology and gynecologic oncology is of particular concern as young age and female gender are often identified as risk factors for this significant problem. Interventions targeted at improving quality of life, treatment of depression, or alcohol abuse may have an impact on burnout. However, significant barriers may exist as 44.5% of respondents in this study reported that they would be reluctant to seek medical care for depression, substance use, or other mental health issues due to concerns about their medical license.

Options for repair of rectus abdominis myocutaneous perineal/vaginal flap prolapse: A case series.

•The VRAM flap is commonly used for perineal and vaginal reconstruction at the time of pelvic exenteration.•Prolapse of the VRAM flap may be under reported.•We have shown successful repair of VRAM flap prolapse via an obliterative technique and sacral suspension.