Treatment strategies in patients with gynecological sarcoma: Results of the prospective intergroup real-world registry for gynecological sarcoma in Germany (REGSA-NOGGO RU1).

OBJECTIVE: Gynecological sarcomas account for 3% of all gynecological malignancies and are associated with a poor prognosis. Due to the rarity and heterogeneity of gynecological sarcomas there is still no consensus on optimal therapeutic strategies. This study's objective was to describe the treatment strategies used in patients with gynecological sarcomas in the primary course of disease. METHODS: The German prospective registry for gynecological sarcoma (REGSA) is the largest registry for gynecological sarcomas in Germany, Austria and Switzerland. Primary inclusion criteria for REGSA are histological diagnosis of sarcoma of the female genital tract, sarcoma of the breast or uterine smooth muscle tumors of uncertain malignant potential (STUMP). We evaluated data of the REGSA registry on therapeutic strategies used for primary treatment from August 2015 to February 2021. RESULTS: A total of 723 patients from 120 centers were included. Data on therapeutic strategies for primary treatment were available in 605 cases. Overall, 580 (95.9%) patients underwent primary surgery, 472 (81.4%) of whom underwent only hysterectomy. Morcellation was reported in 11.4% (n=54) of all hysterectomies. A total of 42.8% (n=202) had no further surgical interventions, whereas an additional salpingo-ophorectomy was performed in 54% (n=255) of patients. An additional lymphadenectomy was performed in 12.7% (n=60), an omentectomy in 9.5% (n=45) and intestinal resection in 6.1% (n=29) of all patients. Among 448 patients with available information, 21.4% (n=96) received chemo- or targeted therapies, more commonly as single-agent treatment than as drug combinations. Information about anti-hormonal treatment was available for 423 patients, among which 42 (9.9%) received anti-hormonal treatment, 23 (54.8%) of whom with low-grade endometrial stroma sarcomas. For radiotherapy, data of 437 patients were available, among which 29 (6.6%) patients underwent radiotherapy. CONCLUSION: Our study showed that treatment of patients with gynecologic sarcomas is heterogeneous. Further trials are needed along with more information on treatment modalities, therapy response and patient-reported outcomes to implement new treatment strategies.

Rates of severe complications in patients undergoing colorectal surgery for deep endometriosis-a retrospective multicenter observational study.

INTRODUCTION: Surgical experience and hospital procedure volumes have been associated with the risk of severe complications in expert centers for endometriosis in France. However, little is known about other certified units in Central European countries. MATERIAL AND METHODS: This retrospective observational study included 937 women who underwent surgery for colorectal endometriosis between January 2018 and January 2020 in 19 participating expert centers for endometriosis. All women underwent complete excision of colorectal endometriosis by rectal shaving, discoid or segmental resection. Postoperative severe complications were defined as grades III-IV of the Clavien-Dindo classification system including anastomotic leakage, fistula, pelvic abscess and hematoma. Surgical outcomes of centers performing less than 40 (group 1), 40-59 (group 2) and ≥60 procedures (group 3) over a period of 2 years were compared. RESULTS: The overall complication rate of grade III and IV complications was 5.1% (48/937), with rates of anastomotic leakage, fistula formation, abscess and hemorrhage in segmental resection, discoid resection and rectal shaving, respectively, as follows: anastomotic leakage 3.6% (14/387), 1.4% (3/222), 0.6% (2/328); fistula formation 1.6% (6/387), 0.5% (1/222), 0.9%; (3/328); abscess 0.5% (2/387), 0% (0/222) and 0.6% (2/328); hemorrhage 2.1% (8/387), 0.9% (2/222) and 1.5% (5/328). Higher overall complication rates were observed for segmental resection (30/387, 7.8%) than for discoid (6/222, 2.7%, P = 0.015) or shaving procedures (12/328, 3.7%, P = 0.089). No significant correlation was observed between the number of procedures performed and overall complication rates (rSpearman  = -0.115; P = 0.639) with a high variability of complications in low-volume centers (group 1). However, an intergroup comparison revealed a significantly lower overall severe complication rate in group 3 than in group 2 (2.9% vs 6.9%; P = 0.017) without significant differences between other groups. CONCLUSIONS: A high variability in complication rates does exist in centers with a low volume of activity. Major complications may decrease with an increase in the volume of activity but this effect cannot be generally applied to all institutions and settings.

[S2k guidelines for the diagnosis and treatment of endometriosis-Recommendations for pathology]. / S2k-Leitlinie Diagnostik und Therapie der Endometriose ­ Anforderungen an die Pathologie.

The present article summarises the recommendations for the handling, histopathological workup, diagnostics and reporting in surgical pathology of biopsies and resection specimens in patients with the clinical diagnosis of endometriosis. In addition to practical aspects of pathology, the guidelines also take into account the clinical requirements for histopathology for the optimal diagnosis and therapy of the patients.Based on the definition of endometriosis of the corpus uteri (adenomyosis uteri) most commonly used in the pathological literature, this was defined in the guidelines as the detection of the endometriosis focus in the myometrium at a distance from the endomyometrial border of a medium-sized visual field (100× magnification), which in metric units corresponds to around 2.5 mm. In bowel resection specimens, the status of the resection margins had to be documented within the histopathological report.Also mentioned are the requirements for the reporting of carcinomas associated with endometriosis, including the immunohistochemical evaluation of steroid hormone receptors and mismatch repair proteins.

The #Enzian classification: A comprehensive non-invasive and surgical description system for endometriosis.

Advances in preoperative diagnostics as well as in surgical techniques for the treatment of endometriosis, especially for deep endometriosis, call for a classification system, that includes all aspects of the disease such as peritoneal endometriosis, ovarian endometriosis, deep endometriosis, and secondary adhesions. The widely accepted revised American Society for Reproductive Medicine classification (rASRM) has certain limitations because of its incomplete description of deep endometriosis. In contrast, the Enzian classification, which has been implemented in the last decade, has proved to be the most suitable tool for staging deep endometriosis, but does not include peritoneal or ovarian disease or adhesions. To overcome these limitations, a comprehensive classification system for complete mapping of endometriosis, including anatomical location, size of the lesions, adhesions and degree of involvement of the adjacent organs, that can be used with both diagnostic and surgical methods, has been created through a consensus process and will be described in detail-the #Enzian classification.

Surgical versus clinical staging prior to primary chemoradiation in patients with cervical cancer FIGO stages IIB-IVA: oncologic results of a prospective randomized international multicenter (Uterus-11) intergroup study.

OBJECTIVE: Revised staging of patients with locally advanced cervical cancer is based on clinical examination, imaging, and potential surgical findings. A known limitation of imaging techniques is an appreciable rate of understaging. In contrast, surgical staging may provide more accurate information on lymph node involvement. The aim of this prospective study was to evaluate the impact of pre-treatment surgical staging, including removal of bulky lymph nodes, on disease-free survival in patients with locally advanced cervical cancer. METHODS: Uterus-11 was a prospective international multicenter study including patients with locally advanced cervical cancer who were randomized 1:1 to surgical staging (experimental arm) or clinical staging (control arm) followed by primary platinum-based chemoradiation. Patients with histologically proven squamous cell carcinoma, adenocarcinoma, or adenosquamous cancer International Federation of Gynecology and Obstetrics (FIGO) 2009 stage IIB-IVA underwent gynecologic examination and pre-treatment imaging including abdominal computed tomography (CT) and/or abdominal magnetic resonance imaging (MRI). Patients had chest imaging (any of the following: X-ray, CT, or PET-CT). The primary endpoint was disease-free survival and the secondary endpoint was overall survival. An ad hoc analysis was performed after trial completion for cancer-specific survival. Randomization was conducted from February 2009 to August 2013. RESULTS: A total of 255 patients (surgical arm, n=130; clinical arm, n=125) with locally advanced cervical cancer were randomized. Of these, 240 patients were eligible for analysis. The two groups were comparable with respect to patient characteristics. The surgical approach was transperitoneal laparoscopy in most patients (96.6%). Laparoscopic staging led to upstaging in 39 of 120 (33%) patients. After a median follow-up of 90 months (range 1-123) in both arms, there was no difference in disease-free survival between the groups (p=0.084). For patients with FIGO stage IIB, surgical staging is superior to clinical staging with respect to disease-free survival (HR 0.51, 95% CI 0.30 to 0.86, p=0.011). In the post-hoc analysis, surgical staging was associated with better cancer-specific survival (HR 0.61, 95% CI 0.40 to 0.93, p=0.020). CONCLUSION: Our study did not show a difference in disease-free survival between surgical and clinical staging in patients with locally advanced cervical cancer. There was a significant benefit in disease-free survival for patients with FIGO stage IIB and, in a post-hoc analysis, a cancer-specific survival benefit in favor of laparoscopic staging. The high risk of distant metastases in both arms emphasizes the need for further evaluation.

Assessing practical laparoscopic training in certified Training Centers of the Gynecological Endoscopy Working Group (AGE) of the German Society of Gynecology and Obstetrics (DGGG).

PURPOSE: This study was performed to assess the practical laparoscopic training in Gynecological Endoscopy Working Group (AGE) certified Training Centers (TC) and evaluate the possible implementation for a manual dexterity skills-training within the Minimal Invasive Surgery (MIC) certification process. MATERIAL AND METHODS: An online questionnaire was developed and the link provided for the heads of the AGE TC. The questionnaire comprised topics on TC organization, practical training performance and perspectives for future training and demographic data. RESULTS: Response rate was 78.9% (15/19) of AGE TC. Grasping for the basic and suturing exercises for the advanced curricula, respectively, are thought to be of highest value (each 1.0 ± 0, on a scale from 1 = very valuable to 6 = not at all valuable). Most valuable parameter in assessing training was thought to be pressure/tension with 1.80 ± 1.08 The most valuable training capacity was considered for box training under supervision (1.27 ± 0.59) and feed-back box training with direct evaluation of various surgical skills (1.40 ± 0.63). Supervised box training was also thought to have the most positive influence on surgical performance (1.33 ± 0.49). The majority of respondents (86.7%) were qualified with the highest MIC certification and additional 66.7% were sub-specialized Gynecological Oncologists. CONCLUSION: The AGE certified TC offer a structured curriculum with emphasis on practical training. The results of this questionnaire and the additional respondents comments on value and future perspectives/changes of practical training support the concept and the implementation of a skills-training to the AGE MIC concept.

Perioperative morbidity and rate of upstaging after laparoscopic staging for patients with locally advanced cervical cancer: results of a prospective randomized trial.

OBJECTIVE: The International Federation of Gynecology and Obstetrics (FIGO) staging for cervical cancer is based on clinical examination. Previous studies have demonstrated significant upstaging with surgical staging. However, no randomized trial has ever shown a survival benefit when radiation combined with chemoradiation (RCTX) is modified according to surgical staging. The objective of the study was to evaluate the feasibility and outcomes of surgical staging prior to radical RCTX treatment among patients with locally advanced cervical cancer in the setting of a larger, prospective, randomized study (the Uterus-11 study of the German Gynecologic Oncology Group). STUDY DESIGN: Between 2009 and 2013, 255 patients with advanced cervical cancer (FIGO IIB-IVA) were randomized to surgical staging and RCTX (arm A) or RCTX (arm B). RCTX in both arms included pelvic external beam radiotherapy with weekly cisplatin at 40 mg/m(2) and brachytherapy. Extended-field radiation was performed in cases of confirmed paraaortic metastases. RESULTS: One hundred thirty patients were randomized to surgical staging; 121 were eligible for this analysis. The mean patient age was 47.2 years, and the mean body mass index was 26.2 kg/m(2); the FIGO stages were IIB, IIIA, IIIB, and IVA in 85 (70.2%), 4 (3.3%), 29 (24%), and 3 (2.5%) patients, respectively. Arm A and arm B were similar with respect to Karnofsky performance status, histology, comorbidities, and lymphovascular space involvement. The surgical approach was transperitoneal laparoscopy in nearly all patients (93.4%), with no operative mortality. One patient (0.8%) had a conversion to laparotomy; 2 patients had more than 500 mL blood loss; the early postoperative complication rate was 7.3%. A mean of 19 pelvic and 17 paraaortic nodes were removed, with means of 2.4 and 1.3 positive nodes, respectively. RCTX began between 7 and 21 days after surgery. Operative staging led to upstaging in 40 of 121 (33%). CONCLUSION: Surgical staging in patients with locally advanced cervical cancer is safe and does not delay primary RCTX in a randomized study.

Surgical Treatment of Deep Endometriosis.

In deep endometriosis (DE), clusters of endometrium-like cells penetrate more than 5 mm below the peritoneum: The affected organs and tissue structures can eventuate in an alteration of the anatomy with eliminated organ boundaries, which in some cases can pose a real surgical challenge, even for experienced surgeons. A comprehensive description of the different manifestations of the disease can be found in the #Enzian classification. Since the operation is usually the foundation for the successful treatment of DE, what is important are conclusive indications, appropriate preoperative preparation and, above all, appropriate experience on the part of the surgical team. This article aims to provide a review of the surgical options that are currently available.

Laparoscopic Fertility-Sparing Surgery for Early Ovarian Malignancies.

The demand for fertility-sparing surgery (FSS) has increased in the last decade due to increased maternal age, increased incidence of ovarian malignancies in younger patients, and technical advances in surgery. Data on oncological safety and fertility outcomes of patients with ovarian cancer after laparoscopic FSS are sparse, but some retrospective studies have shown that open FSS may be offered to selected patients. We assessed the role of minimally invasive FSS in comparison with radical surgery (RS) in terms of oncological safety and reproductive outcomes after FSS in this multicenter study. Eighty patients with FIGO stage I/II ovarian cancer treated with laparoscopic FSS or RS between 01/2000 and 10/2018 at the participating centers (comprehensive gynecological cancer centers with minimally invasive surgical expertise) were included in this retrospective analysis of prospectively kept data. Case-control (n = 40 each) matching according to the FIGO stage was performed. Progression-free survival [150 (3-150) and 150 (5-150) months; p = 0.61] and overall survival [36 (3-150) and 50 (1-275) months; p = 0.65] did not differ between the FSS and RS groups. Eight (25.8%) women became pregnant after FSS, resulting in seven (22.5%) deliveries; three (37.5%) patients conceived after in vitro fertilization, and five (62.5%) conceived spontaneously. Laparoscopic FSS seems to be applicable and oncologically safe for patients with early-stage ovarian cancer, with adequate fertility outcomes.

Disseminated peritoneal leiomyomatosis with endometriosis.

Herein is described the case of a 41-year-old woman with disseminated peritoneal leiomyomatosis with distinct endometriosis. The pathogenesis of both conditions is as yet unclear; however, the 2 main hypotheses are discussed. Metaplastic origin from the secondary müllerian system has been suggested, as well as metastatic development. Inasmuch as spontaneous regression is likely, and the course of the disease can be influenced by hormonal withdrawal, operative measures could be refined to ensure the correct diagnosis and benignity.

Sarcoma of the Uterus. Guideline of the DGGG, OEGGG and SGGG (S2k-Level, AWMF Registry No. 015/074, April 2021).

Purpose This is an official guideline, published and coordinated by the Germany Society for Gynecology and Obstetrics (Deutsche Gesellschaft für Gynäkologie und Geburtshilfe, DGGG). Because of their rarity and heterogeneous histopathology, uterine sarcomas are challenging in terms of their clinical management and therefore require a multidisciplinary approach. To our knowledge, there are currently no binding evidence-based recommendations for the appropriate management of this heterogeneous group of tumors. Methods This S2k guideline was first published in 2015. The update published here is once again the result of the consensus of a representative interdisciplinary committee of experts who were commissioned by the Guidelines Committee of the DGGG to carry out a systematic search of the literature on uterine sarcomas. Members of the participating professional societies achieved a formal consensus after a structured consensus process. Recommendations 1.1 Epidemiology, classification, staging of uterine sarcomas. 1.2 Symptoms, general diagnostic workup, general pathology or genetic predisposition to uterine sarcomas. 2. Management of leiomyosarcomas. 3. Management of low-grade endometrial stromal sarcomas. 4. Management of high-grade endometrial stromal sarcoma and undifferentiated uterine sarcomas. 5. Management of adenosarcomas. 6. Rhabdomyosarcomas of the uterus in children and adolescents. 7. Follow-up of uterine sarcomas. 8. Management of morcellated uterine sarcomas. 9. Information provided to patients.

Risk-reducing salpingo-oophorectomy in BRCA1 and BRCA2 mutation carriers.

BACKGROUND: Risk-reducing salpingo-oophorectomy (RRSO) is often recommended to carriers of deleterious breast cancer gene 1/2 (BRCA1/2) mutations in order to reduce their breast cancer risk by 50% and their ovarian cancer risk by approximately 95%. To evaluate the acceptance, timing, histopathology findings and follow-up results we retrospectively analyzed a cohort of BRCA1/2 mutation carriers who underwent risk-reducing salpingo-oophorectomies. METHODS: Between 1996 and 2009, 306 women who tested positive for a BRCA1 or BRCA2 mutation were counseled for preventive options. RRSO was recommended to all mutation carriers at age 40 or 5 years prior to the earliest occurrence of ovarian cancer in the family. Data from 175 BRCA mutation carriers (92 BRCA1 and 83 BRCA2), who decided to undergo a RRSO, were analyzed. Data were collected from study entry until recent follow-up. RESULTS: Fifty-seven percent of BRCA mutation carriers opted for RRSO. Mean age at time of surgery was 47 years. Overall, one occult carcinoma of the fallopian tube was detected at the time of surgery in a 57-year-old woman and one primary peritoneal carcinoma occurred 26 months after RRSO in a 59-year-old woman. CONCLUSION: Risk-reducing salpingo-oophorectomy is widely accepted. Recommendation of surgery at the age of 40 seems to be safe and the frequency of extraovarian primary peritoneal carcinoma after surgery is low.

Diagnosis and Treatment of Endometriosis. Guideline of the DGGG, SGGG and OEGGG (S2k Level, AWMF Registry Number 015/045, August 2020).

Aims The aim of this official guideline published and coordinated by the German Society of Gynaecology and Obstetrics (DGGG) in cooperation with the Austrian Society for Gynaecology and Obstetrics (OEGGG) and the Swiss Society for Gynaecology and Obstetrics (SGGG) was to provide consensus-based recommendations for the diagnosis and treatment of endometriosis based on an evaluation of the relevant literature. Methods This S2k guideline represents the structured consensus of a representative panel of experts with different professional backgrounds commissioned by the Guideline Committee of the DGGG, OEGGG and SGGG. Recommendations Recommendations on the epidemiology, aetiology, classification, symptomatology, diagnosis and treatment of endometriosis are given and special situations are discussed.

Helical tomotherapy in cervical cancer patients: simultaneous integrated boost concept: technique and acute toxicity.

PURPOSE: To evaluate the acute toxicity of simultaneous integrated boost (SIB) technique for dose escalation with helical tomotherapy (HT) in patients with locally advanced cervical cancer. PATIENTS AND METHODS: 20 patients (FIGO IB1 pN1-IIIB) underwent primary chemoradiation. Prior to chemoradiation, a laparoscopic pelvic and para-aortic lymphadenectomy was performed. A boost region was defined using titanium clips during staging for planning target volume (PTV-B). Patients were treated with five weekly fractions of 1.8 Gy to a total dose of 50.4 Gy to the tumor region and the pelvic (para-aortic) lymph node region (PTV-A), and five weekly fractions of 2.12 Gy to a total dose of 59.36 Gy to the PTV-B. Chemotherapy consisted of weekly cisplatin 40 mg/m(2). 19 patients underwent brachytherapy. Dose-volume histograms were evaluated and acute gastrointestinal (GI), genitourinary (GU), and hematologic toxicity were documented (CTCAE v3.0). RESULTS: Pelvic and para-aortic lymph node metastases were confirmed in nine and four patients, respectively. Five patients refused laparoscopic staging. The mean volume of PTV-A and PTV-B was 1,570 ± 404 cm(3) and 341 ± 125 cm(3), respectively. The mean dose to the bladder, rectum, and small bowel was 47.85 Gy, 45.76 Gy, and 29.71 Gy, respectively. No grade 4/5 toxicity was observed. Grade 2/3 hematologic toxicity occurred in 50% of patients and 5% experienced grade 3 diarrhea. There was no grade 3 GU toxicity. 19 patients underwent curettage 6-9 weeks after chemoradiation without any evidence of tumor. CONCLUSION: The concept of SIB for dose escalation in patients with locally advanced cervical cancer is feasible with a low rate of acute toxicity. Whether dose escalation can translate into improved outcome will be assessed after a longer follow-up.

Recommendations for the surgical treatment of endometriosis. Part 2: deep endometriosis.

STUDY QUESTION: How should surgery for endometriosis be performed? SUMMARY ANSWER: This document provides recommendations covering technical aspects of different methods of surgery for deep endometriosis in women of reproductive age. WHAT IS KNOWN ALREADY: Endometriosis is highly prevalent and often associated with severe symptoms. Yet compared to equally prevalent conditions, it is poorly understood and a challenge to manage. Previously published guidelines have provided recommendations for (surgical) treatment of deep endometriosis, based on the best available evidence, but without technical information and details on how to best perform such treatment in order to be effective and safe. STUDY DESIGN SIZE DURATION: A working group of the European Society for Gynaecological Endoscopy (ESGE), ESHRE and the World Endometriosis Society (WES) collaborated on writing recommendations on the practical aspects of surgery for treatment of deep endometriosis. PARTICIPANTS/MATERIALS SETTING METHODS: This document focused on surgery for deep endometriosis and is complementary to a previous document in this series focusing on endometrioma surgery. MAIN RESULTS AND THE ROLE OF CHANCE: The document presents general recommendations for surgery for deep endometriosis, starting from preoperative assessments and first steps of surgery. Different approaches for surgical treatment are discussed and are respective of location and extent of disease; uterosacral ligaments and rectovaginal septum with or without involvement of the rectum, urinary tract or extrapelvic endometriosis. In addition, recommendations are provided on the treatment of frozen pelvis and on hysterectomy as a treatment for deep endometriosis. LIMITATIONS REASONS FOR CAUTION: Owing to the limited evidence available, recommendations are mostly based on clinical expertise. Where available, references of relevant studies were added. WIDER IMPLICATIONS OF THE FINDINGS: These recommendations complement previous guidelines on management of endometriosis and the recommendations for surgical treatment of ovarian endometrioma. STUDY FUNDING/COMPETING INTERESTS: The meetings of the working group were funded by ESGE, ESHRE and WES. Dr Roman reports personal fees from ETHICON, PLASMASURGICAL, OLYMPUS and NORDIC PHARMA, outside the submitted work; Dr Becker reports grants from Bayer AG, Volition Rx, MDNA Life Sciences and Roche Diagnostics Inc. and other relationships or activities from AbbVie Inc., and Myriad Inc, during the conduct of the study; Dr Tomassetti reports non-financial support from ESHRE, during the conduct of the study; and non-financial support and other were from Lumenis, Gedeon-Richter, Ferring Pharmaceuticals and Merck SA, outside the submitted work. The other authors had nothing to disclose. TRIAL REGISTRATION NUMBER: na.

Updated Opinion of the Uterus Commission of the Gynecological Oncology Working Group (AGO) and the Gynecological Endoscopy Working Group (AGE) of the German Society of Gynecology and Obstetrics (DGGG) on the Randomized Study Comparing Minimally Invasive with Abdominal Radical Hysterectomy for Early-stage Cervical Cancer (LACC).

In this opinion on the randomized study comparing minimally invasive with abdominal radical hysterectomy for early-stage cervical cancer (LACC), the Uterus Commission of the Gynecological Oncology Working Group (AGO) and the Gynecological Endoscopy Working Group (AGE) of the Germany Society of Gynecology and Obstetrics (DGGG) state that, based on their examination of the published data, patients with FIGO stage IA1 (with LVSI), IA2 or IB1 cervical cancer must be informed about the results of this LACC study prior to making a decision on the route for radical hysterectomy.

Sarcoma of the Uterus. Guideline of the DGGG and OEGGG (S2k Level, AWMF Register Number 015/074, February 2019).

Aims This is an official guideline published and coordinated by the German Society of Gynecology and Obstetrics (DGGG) and the Austrian Society of Gynecology and Obstetrics (OEGGG). Because of their rarity and heterogeneous histopathology, uterine sarcomas are challenging in terms of how they should be managed clinically, and treatment requires a multidisciplinary approach. To our knowledge, there are currently no binding evidence-based recommendations for the appropriate management of this heterogeneous group of tumors. Methods This S2k guideline was first published in 2015. The update published here is the result of the consensus of a representative interdisciplinary group of experts who carried out a systematic search of the literature on uterine sarcomas in the context of the guidelines program of the DGGG, OEGGG and SGGG. Members of the participating professional societies achieved a formal consensus after a moderated structured consensus process. Recommendations The consensus-based recommendations and statements include the epidemiology, classification, staging, symptoms, general diagnostic work-up and general pathology of uterine sarcomas as well as the genetic predisposition to develop uterine sarcomas. Also included are statements on the management of leiomyosarcomas, (low and high-grade) endometrial stromal sarcomas and undifferentiated uterine sarcomas and adenosarcomas. Finally, the guideline considers the follow-up and morcellation of uterine sarcomas and the information provided to patients.

Uterine Adenosarcoma.

Uterine adenosarcoma is a rare malignancy. It is defined as a biphasic tumor composed of both sarcomatous stroma and benign epithelium. While the sarcomatous component usually is a low-grade homologous uterine sarcoma, the epithelium most often consists of endometrium-like cells. If the sarcomatous part occupies more than 25% of the tumor volume, the situation is referred to as sarcomatous overgrowth - accounting for about 10% of cases. While adenosarcoma usually may be considered a tumor of low malignant potential, the sarcomatous overgrowth most often presents as high-grade sarcoma and is associated with aggressive clinical behavior. Adenosarcomas stage I without sarcomatous overgrowth have a rather good prognosis, with a 5-year overall survival up to 80%. For treatment, complete surgical removal is advocated. Adjuvant chemotherapy and radiotherapy are not defined. Recurrences should again be treated surgically, attempting to achieve complete tumor resection. While the optimum medical treatment for relapsed and metastasized adenosarcomas has yet to be found, chemotherapy and endocrine therapy are potential options.

Uterine Carcinosarcomas - Diagnosis and Management.

Uterine carcinosarcomas are rare tumors that account for less than 5% of all uterine malignancies. These tumors (previously called malignant mixed Müllerian tumors) are dedifferentiated carcinomas that comprise carcinomatous and sarcomatous elements and arise from a single malignant clone. They are considered a high-risk variant of endometrial adenocarcinoma because carcinosarcomas share more similarities in epidemiology, risk factors, and clinical behavior with endometrial carcinoma than with uterine sarcomas. The clinical features, diagnosis, staging, and treatment of uterine carcinosarcoma will be discussed in this review.

Recommendations for the Surgical Treatment of Endometriosis. Part 1: Ovarian Endometrioma.

STUDY QUESTION: What does this document on the surgical treatment of endometriosis jointly prepared by the European Society for Gynaecological Endoscopy (ESGE), ESHRE, and the World Endometriosis Society (WES) provide? SUMMARY ANSWER: This document provides recommendations covering technical aspects of different methods of surgery for endometriomas in women of reproductive age. WHAT IS ALREADY KNOWN: Endometriomas (ovarian endometriotic cysts) are a commonly diagnosed form of endometriosis, owing to the relative ease and accuracy of ultrasound diagnosis. They frequently present a clinical dilemma as to whether and how to treat them when found during imaging or incidentally during surgery. Previously published guidelines have provided recommendations based on the best available evidence, but without technical details on the management of endometriosis. STUDY DESIGN SIZE DURATION: A working group of ESGE, ESHRE and WES collaborated on writing recommendations on the practical aspects of endometrioma surgery. PARTICIPANTS/MATERIALS SETTING METHODS: This document focused on endometrioma surgery. Further documents in this series will provide recommendations for surgery of deep and peritoneal endometriosis. MAIN RESULTS AND THE ROLE OF CHANCE: The document presents general recommendations for surgery of endometrioma, and specific recommendations for cystectomy, ablation by laser or by plasma energy, electrocoagulation and a combination of these techniques applied together or with an interval between them. LIMITATIONS REASONS FOR CAUTION: Owing to the limited evidence available, recommendations are mostly based on clinical expertise. WIDER IMPLICATIONS OF THE FINDINGS: These recommendations complement previous guidelines on the management of endometriosis. STUDY FUNDING/COMPETING INTERESTS: The meetings of the working group were funded by ESGE, ESHRE and WES. C.B. declares to be a member of the independent data monitoring committee for a clinical study by ObsEva, and receiving research grants from Bayer, Roche Diagnostics, MDNA Life Sciences, and Volition. E.S. received honoraria for provision of training to healthcare professionals from Ethicon, Olympus and Gedeon Richter. The other authors declare that they have no conflict of interest. TRIAL REGISTRATION NUMBER: NA.