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Cureus Mar 2024Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome presents with complex diagnostic and therapeutic challenges and is characterized by uterine...
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome presents with complex diagnostic and therapeutic challenges and is characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. A 14-year-old female with a history of anorectal malformation and urogenital sinus anomaly presented with menstrual blood in her urine, abdominal pain, and distension. Investigations revealed a bicornuate uterus, vesicovaginal fistula, and right ovarian cyst, leading to the diagnosis of OHVIRA syndrome. A multidisciplinary approach resulted in salpingo-oophorectomy and subtotal hysterectomy. This case highlights the diagnostic challenges and emphasizes the role of advanced imaging and a multidisciplinary team in managing such complex conditions. It stresses the importance of patient-centered surgical planning tailored to the individual's anatomy and reproductive goals. Early recognition and a tailored, multidisciplinary approach are crucial in managing OHVIRA syndrome and improving outcomes for patients with rare congenital anomalies.
PubMed: 38665700
DOI: 10.7759/cureus.56961 -
International Journal of Gynecological... Apr 2024To assess the role of histopathological and molecular features in predicting the risk of nodal metastases in apparent early-stage endometrial cancer patients undergoing...
OBJECTIVE
To assess the role of histopathological and molecular features in predicting the risk of nodal metastases in apparent early-stage endometrial cancer patients undergoing sentinel node mapping.
METHODS
This is a prospective trial. Consecutive patients with apparent early-stage endometrial cancer, undergoing laparoscopic hysterectomy, bilateral salpingo-oophorectomy, and sentinel node mapping, were enrolled. Histological and molecular features were used to predict the node positivity.
RESULTS
Charts of 223 apparent early-stage endometrial cancer patients were included in this study. Four (1.8%) patients were excluded from this study due to the lack of data about molecular features. Additionally, nine (4%) patients did not meet the inclusion criteria (due to the presence of peritoneal carcinomatosis or bulky nodes (the presence of p53 abnormality correlated with the presence of advanced stage disease (p<0.001)). The study population included 178 (84.8%) and 32 (15.2%) patients with endometrioid and non-endometrioid endometrial cancer, respectively. According to pathological uterine risk factors, 93 (44.3%), 45 (21.4%), 40 (19.1%), and 32 (15.2%) were classified as low, intermediate, intermediate-high, and high-risk, respectively. Using the surrogate molecular classification, 10 (4.8%), 42 (20%), 57 (27.1%), and 101 (48.1%) were included in the POLE mutated, p53 abnormal, MMRd/MSI-H, and NSMP, respectively. Overall, 41 (19.5%) patients were detected with positive nodes. Molecular features were not associated with the risk of having nodal metastases (OR 1.03, 95% CI 0.21 to 5.05, p=0.969 for mutated; OR 0.788, 95% CI 0.32 to 1.98, p=0.602 for p53 abnormal; OR 1.14, 95% CI 0.53 to 2.42, p=0.733 for MMRd/MSI-H). At multivariable analysis, only deep myometrial invasion (OR 3.318, 95% CI 1.357 to 8.150, p=0.009) and lymphovascular space invasion (OR 6.584, 95% CI 2.663 to 16.279, p<0.001) correlated with the increased risk of positive nodes.
CONCLUSION
Our data suggest that molecular classification does not seem useful to tailor the need of nodal dissection in apparent early-stage endometrial cancer. p53 abnormality predicts the risk of having advanced disease at presentation. Further external validation is needed.
CLINICAL TRIAL REGISTRATION
NCT05793333.
PubMed: 38658017
DOI: 10.1136/ijgc-2024-005416 -
Journal of Medical Case Reports Apr 2024Sex cord-stromal tumors with annular tubules are a rare tumor accounting for less than 1% of all ovarian malignancies. However, they are characterized by very late...
BACKGROUND
Sex cord-stromal tumors with annular tubules are a rare tumor accounting for less than 1% of all ovarian malignancies. However, they are characterized by very late recurrence, which can be as late as 30 years after diagnosis and treatment.
CASE PRESENTATION
A 16-year-old female Caucasian patient was treated in our department for a stage IA ovarian sex cord-stromal tumors with annular tubules. She underwent a left salpingo-oophorectomy and ipsilateral pelvic node biopsy with no adjuvant treatment. She was seen for amenorrhea after being lost to follow up for 16 years. The diagnosis of recurrence was made by radiology and the elevation of serum inhibin B level. The patient underwent resection of the tumor, left segmental colectomy, and paraaortic lymphadenectomy because the mass was massively adherent to the left mesocolon. Histology confirmed the diagnosis with no metastatic lymph nodes. No adjuvant therapy was indicated. The patient was lost to follow-up again for 4 years and re-presented for amenorrhea. Serum inhibin B level was high. A second recurrence was suggested, and the patient underwent a laparoscopic surgery. We performed left pelvic and paraaortic lymphadenectomy, and 3 months after surgery the patient was pregnant.
CONCLUSION
Sex cord-stromal tumors with annular tubules is a slow-growing ovarian tumor with a high potential for recurrence and metastasis. Surgery is the mainstay of treatment. Due to the rarity of these tumors, they are often unsuspected and thus incompletely staged before primary surgery; the diagnosis is made by histological examination. The prognosis of these patients is unknown, and they require long-term follow-up.
Topics: Humans; Female; Ovarian Neoplasms; Adolescent; Neoplasm Recurrence, Local; Sex Cord-Gonadal Stromal Tumors; Lymph Node Excision; Lymphatic Metastasis; Lymph Nodes; Salpingo-oophorectomy; Inhibins
PubMed: 38654310
DOI: 10.1186/s13256-024-04476-5 -
Journal of Minimally Invasive Gynecology Apr 2024To demonstrate how a radical hysterectomy with sentinel node resection for cervical cancer can be performed via vaginal Natural Orifice Transluminal Endoscopic Surgery...
OBJECTIVE
To demonstrate how a radical hysterectomy with sentinel node resection for cervical cancer can be performed via vaginal Natural Orifice Transluminal Endoscopic Surgery (vNOTES).
DESIGN
Demonstration of the technique in 10 steps. making use of narrated original video footage SETTING: The surgical treatment of cervical cancer is traditionally performed via one of the following techniques: Wertheim radical hysterectomy via laparotomy, Schauta radical hysterectomy vaginally, laparoscopic radical hysterectomy or robotic radical hysterectomy. The results of the LACC trial showed that minimally invasive radical hysterectomy was associated with lower rates of disease-free survival and overall survival than open radical hysterectomy among women with early-stage cervical Cancer [1]. For endometrial cancer, a vNOTES retroperitoneal approach to sentinel node resection was first published in 2019 [2]. Based on the experience with this approach and with Schauta-Stoeckel radical hysterectomy for cervical cancer [3], a new approach was developed to perform a radical hysterectomy via vNOTES whereby most of the procedure is performed retroperitoneally [4]. This video article demonstrates in 10 steps how a radical hysterectomy via vNOTES is performed.
INTERVENTIONS
Radical hysterectomy via vNOTES demonstrated making use of original video footage of a 57-year-old woman operated on for cervical adenocarcinoma 7 weeks after a LEEP cone. The steps of the procedure are: 1. Vaginal cuff creation, 2. Development of lateral retroperitoneal space and sentinel node resection, 3. Uterine artery and vein transection, 4. Hypogastric nerve dissection, 5. Development of central retroperitoneal space and rectum dissection, 6. Posterior colpotomy, 7. Parametrium dissection, 8. Bladder pillar dissection, 9. Anterior colpotomy, 10. Salpingo-oophorectomy or salpingectomy. 3 Patients were so far treated by this new technique that allowed for good hemostatic control.
CONCLUSION
vNOTES enables a potentially less invasive approach to radical hysterectomy performed largely retroperitoneally and completely transvaginally, leaving no visible scars. The endoscopic approach offers excellent visualization of the retroperitoneal and parametrial anatomy. This is a new approach that requires further validation and should only be performed in a research setting, taking into account the current reservations about endoscopic surgery for cervical cancer resulting from the LACC trial. VIDEO ABSTRACT.
PubMed: 38642886
DOI: 10.1016/j.jmig.2024.04.009 -
Acta Obstetricia Et Gynecologica... Jul 2024The current standard treatment for endometrial cancer is a laparoscopic hysterectomy with adnexectomies and bilateral sentinel node resection. A retroperitoneal vNOTES...
INTRODUCTION
The current standard treatment for endometrial cancer is a laparoscopic hysterectomy with adnexectomies and bilateral sentinel node resection. A retroperitoneal vNOTES sentinel node resection has several theoretical potential advantages. These include being less invasive, leaving no visible scars, operating without Trendelenburg, and therefore offering the anesthetic advantage of easier ventilation in obese patients and following the natural lymph node trajectory from caudally to cranially and therefore a lower risk of missing the sentinel node. The aim of this study is to determine the feasibility of a retroperitoneal vNOTES approach to sentinel lymph node dissection for staging of endometrial cancer.
MATERIAL AND METHODS
A prospective multicenter case series was performed in four hospitals. A total of 64 women with early-stage endometrial carcinoma suitable for surgical staging with sentinel lymph node removal were operated via a transvaginal retroperitoneal vNOTES approach. The paravesical space was entered through a vaginal incision after injecting the cervix with indocyanine green. A vNOTES port was placed into this space and insufflation of the retroperitoneum was performed. Sentinel lymph nodes were identified bilaterally using near-infrared light followed by endoscopic removal of these nodes.
RESULTS
A total of 64 women with early-stage endometrial cancer underwent sentinel lymph node removal by retroperitoneal vNOTES technique. All patients also underwent subsequent vNOTES hysterectomy and bilateral salpingo-oophorectomy. The median age was 69.5 years, median total operative time was 126 min and the median estimated blood loss was 80 mL. In 97% of the cases bilateral sentinel nodes could be identified. A total of 60 patients had negative sentinel nodes, three had isolated tumor cells and one had macroscopically positive sentinel nodes. No complications with sequel occurred.
CONCLUSIONS
This prospective multicenter case series demonstrates the feasibility of the vNOTES approach for identifying and removing sentinel lymph nodes in women with endometrial carcinoma successfully and safely. vNOTES allows sole transvaginal access with exposure of the entire retroperitoneal space, following the natural lymph trajectory caudally to cranially, and without the need for a Trendelenburg position.
Topics: Female; Humans; Endometrial Neoplasms; Prospective Studies; Middle Aged; Neoplasm Staging; Retroperitoneal Space; Aged; Sentinel Lymph Node Biopsy; Lymph Node Excision; Laparoscopy; Feasibility Studies; Adult; Sentinel Lymph Node
PubMed: 38623778
DOI: 10.1111/aogs.14843 -
Cancers Mar 2024Risk-reducing mastectomy (RRM) and risk-reducing salpingo-oophorectomy (RRSO) are the most effective breast and ovarian cancer preventive interventions. EQ-5D is the...
BACKGROUND
Risk-reducing mastectomy (RRM) and risk-reducing salpingo-oophorectomy (RRSO) are the most effective breast and ovarian cancer preventive interventions. EQ-5D is the recommended tool to assess the quality of life and determine health-related utility scores (HRUSs), yet there are no published EQ-5D HRUSs after these procedures. These are essential for clinicians counselling patients and for health-economic evaluations.
METHODS
We used aggregate data from our published systematic review and converted SF-36/SF-12 summary scores to EQ-5D HRUSs using a published mapping algorithm. Study control arm or age-matched country-specific reference values provided comparison. Random-effects meta-analysis provided adjusted disutilities and utility scores. Subgroup analyses included long-term vs. short-term follow-up.
RESULTS
Four studies (209 patients) reported RRM outcomes using SF-36, and five studies (742 patients) reported RRSO outcomes using SF-12/SF-36. RRM is associated with a long-term (>2 years) disutility of -0.08 (95% CI -0.11, -0.04) (I 31.4%) and a utility of 0.92 (95% CI 0.88, 0.95) (I 31.4%). RRSO is associated with a long-term (>1 year) disutility of -0.03 (95% CI -0.05, 0.00) (I 17.2%) and a utility of 0.97 (95% CI 0.94, 0.99) (I 34.0%).
CONCLUSIONS
We present the first HRUSs sourced from patients following RRM and RRSO. There is a need for high-quality prospective studies to characterise quality of life at different timepoints.
PubMed: 38611036
DOI: 10.3390/cancers16071358 -
Breast Cancer Research and Treatment Jul 2024Carriers of pathogenic variants in BRCA1/2 have an elevated lifetime cancer risk warranting high-risk screening and risk-reducing procedures for early detection and...
Uptake of screening and risk-reducing recommendations among women with hereditary breast and ovarian cancer syndrome due to pathogenic BRCA1/2 variants evaluated at a large urban comprehensive cancer center.
PURPOSE
Carriers of pathogenic variants in BRCA1/2 have an elevated lifetime cancer risk warranting high-risk screening and risk-reducing procedures for early detection and prevention. We report on prevention practices among women with pathogenic BRCA variants in order to document follow through with NCCN recommendations and to identify barriers to guideline-recommended care.
METHODS
Our cohort included women who had genetic testing through a cancer genetic clinic and completed a 54-item questionnaire to measure socio-demographics, medical history, rates of cancer screening and risk-reducing surgery, disclosure of test results, and cancer worry. Outcomes included rates of completion of risk-reducing salpingo-oophorectomy (RRSO), risk-reducing mastectomy (RRM), and NCCN risk-reducing and age-dependent screening guidelines (version 3.2019). Multivariable logistic regression analyses were used to evaluate potential predictors of these outcomes.
RESULTS
Of 129 evaluable women with pathogenic BRCA1/2 variants, 95 (74%) underwent RRSO and 77 (60%) had RRM, respectively, and 107 (83%) were considered adherent to NCCN guidelines. Women with a history of breast or ovarian cancer were more likely to have RRM (OR = 4.38; 95% CI 1.80-11.51; p = 0.002). Increasing age was associated with an increased likelihood of RRSO (OR = 1.05; 95% CI 1.01-1.09; p = 0.019) and decreased likelihood for RRM (OR = 0.95; 95% CI 0.92-0.99; p = 0.013). Women who had RRM were 3 times more likely to undergo RRSO (OR = 2.81; 95% CI 1.10-7.44; p = 0.025). Women who had genetic testing after June 2013 were less likely to have RRM than those tested before June 2013 (OR = 0.42; 95% CI 0.18-0.95; p = 0.040. None of the other measured factors were associated with rates of RRSO, RRM or follow through with NCCN recommendations. There was near universal (127/129) reported disclosure of genetic test results to family members, resulting in the discovery of a median of 1 relative with a pathogenic variant (range = 0-8).
CONCLUSION
An evaluation of follow up practice in a cohort of women with pathogenic variants in BRCA1/2 revealed high rates of reported completion of screening and surgical risk-reducing recommendations. Educational efforts should continue to reinforce the importance of follow-through with guideline recommended care among this high-risk group.
Topics: Humans; Female; Hereditary Breast and Ovarian Cancer Syndrome; Middle Aged; Adult; BRCA1 Protein; Genetic Testing; BRCA2 Protein; Early Detection of Cancer; Genetic Predisposition to Disease; Aged; Risk Reduction Behavior; Ovarian Neoplasms; Breast Neoplasms; Mutation; Salpingo-oophorectomy
PubMed: 38605155
DOI: 10.1007/s10549-024-07283-0 -
Case Reports in Women's Health Jun 2024Xanthogranulomatous oophoritis (XO) is a rare pseudotumor representing a destructive chronic inflammatory process often mistaken for malignancy or tubo-ovarian abscess....
Xanthogranulomatous oophoritis (XO) is a rare pseudotumor representing a destructive chronic inflammatory process often mistaken for malignancy or tubo-ovarian abscess. Xanthogranulomatous inflammation is most commonly seen in the kidneys and gallbladder and very rarely affects the genitourinary system. Definitive treatment is with surgical removal of affected tissue. This report presents the case of a 42-year-old woman with an 8 cm complex right adnexal cyst concerning for a dermoid cyst presenting with intermittent torsion. Final pathology after right salpingo-oophorectomy demonstrated xanthogranulomatous oophoritis. This case is of clinical significance for distinguishing the condition from common benign pathology or cancer since the recommended surgical procedure is different than for a dermoid cyst or malignancy. Correct identification of the condition is crucial for appropriate treatment and to avoid unnecessary morbid procedures if the mass is mistaken for malignancy or future repeat surgery if mistaken for a dermoid cyst or other common benign condition. This case documents the presentation of xanthogranulomatous oophoritis masquerading as a dermoid cyst for a condition with very few reported cases worldwide.
PubMed: 38600914
DOI: 10.1016/j.crwh.2024.e00603 -
Current Opinion in Obstetrics &... Aug 2024Given the current political climate and the release of an updated version of the World Professional Association for Transgender Health's guidelines, this review assesses... (Review)
Review
PURPOSE OF REVIEW
Given the current political climate and the release of an updated version of the World Professional Association for Transgender Health's guidelines, this review assesses recent updates in the care of transgender and gender diverse (TGD) patients, specifically related to care provided by gynecologists.
RECENT FINDINGS
The number of people identifying as TGD and pursuing gender affirming care is increasing. Contraception for these patients is underdiscussed and high rates of pelvic pain and irregular bleeding were identified. Rates of regret are low following gender affirming surgeries, and studies have repeatedly shown their benefits for gender dysphoria. A minimally invasive approach is recommended for gender affirming hysterectomy, and the decision to proceed with bilateral salpingo-oophorectomy should be based on shared decision making. Surgical techniques include ensuring an adequate margin when taking the infundibulopelvic ligament, and consideration for two-layer vaginal cuff closure.
SUMMARY
Gynecologists play a key role in the care of TGD patients. Recent reviews have found extensive gaps in our knowledge, including a lack of guidelines for cancer prevention, effects of testosterone on benign conditions, and the long-term effects of bilateral salpingo-oophorectomy on health outcomes for patients on testosterone.
Topics: Humans; Female; Male; Transgender Persons; Minimally Invasive Surgical Procedures; Hysterectomy; Sex Reassignment Surgery; Gynecology; Gender Dysphoria; Salpingo-oophorectomy; Gender-Affirming Care
PubMed: 38597457
DOI: 10.1097/GCO.0000000000000956