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Since the publication of the updated German guideline in 2015, the recommendations for performing pelvic lymphadenectomy (LAE) in patients with vulvar cancer (VSCC) have changed considerably. The guideline recommends surgical lymph node staging in all patients with a higher risk of pelvic lymph node involvement. However, the current data do not allow the population at risk to be clearly defined, therefore, the indication for pelvic lymphadenectomy is still not clear. There are currently two published German patient populations who had pelvic LAE which can be used to investigate both the prognostic effect of histologically verified pelvic lymph node metastasis and the relation between inguinal and pelvic lymph node involvement. A total of 1618 patients with primary FIGO stage ≥ IB VSCC were included in the multicenter AGO CaRE-1 study (1998 – 2008), 70 of whom underwent pelvic LAE. During a retrospective single-center evaluation carried out at the University Medical Center Hamburg-Eppendorf (UKE), a total of 514 patients with primary VSCC treated between 1996 – 2018 were evaluated, 21 of whom underwent pelvic LAE. In both cohorts, around 80% of the patients who underwent pelvic LAE were inguinally node-positive, with a median number of three affected groin lymph nodes. There were no cases of pelvic lymph node metastasis without inguinal lymph node metastasis in either of the two cohorts. Between 33 – 35% of the inguinal node-positive patients also had pelvic lymph node metastasis; the median number of affected groin lymph nodes in these patients was high (> 4), and the maximum median diameter of the largest inguinal metastasis was > 40 mm in both cohorts. Pelvic lymph node staging and pelvic radiotherapy is therefore probably not necessary for the majority of node-positive patients with VSCC, as the relevant risk of pelvic lymph node involvement was primarily found in node-positive patients with high-grade disease. More, ideally prospective data collections are necessary to validate the relation between inguinal and pelvic lymph node involvement.

Summary The first German interdisciplinary S3-guideline on the diagnosis, therapy and follow-up of patients with endometrial cancer was published in April 2018. Funded by German Cancer Aid as part of an Oncology Guidelines Program, the lead coordinators of the guideline were the German Society of Gynecology and Obstetrics (DGGG) and the Gynecological Oncology Working Group (AGO) of the German Cancer Society (DKG). Purpose Using evidence-based, risk-adapted therapy to treat low-risk women with endometrial cancer avoids unnecessarily radical surgery and non-useful adjuvant radiotherapy and/or chemotherapy. This can significantly reduce therapy-induced morbidity and improve the patientʼs quality of life as well as avoiding unnecessary costs. For women with endometrial cancer and a high risk of recurrence, the guideline defines the optimal extent of surgical radicality together with the appropriate chemotherapy and/or adjuvant radiotherapy if required. An evidence-based optimal use of different therapeutic modalities should improve the survival rates and quality of life of these patients. This S3-guideline on endometrial cancer is intended as a basis for certified gynecological cancer centers. The aim is that the quality indicators established in this guideline will be incorporated in the certification processes of these centers. Methods The guideline was compiled in accordance with the requirements for S3-level guidelines. This includes, in the first instance, the adaptation of source guidelines selected using the DELBI instrument for appraising guidelines. Other consulted sources included reviews of evidence, which were compiled from literature selected during systematic searches of literature databases using the PICO scheme. In addition, an external biostatistics institute was commissioned to carry out a systematic search and assessment of the literature for one part of the guideline. Identified materials were used by the interdisciplinary working groups to develop suggestions for Recommendations and Statements, which were then subsequently modified during structured consensus conferences and/or additionally amended online using the DELPHI method, with consent between members achieved online. The guideline report is freely available online. Recommendations Part 2 of this short version of the guideline presents recommendations for the therapy of endometrial cancer including precancers and early endometrial cancer as well as recommendations on palliative medicine, psycho-oncology, rehabilitation, patient information and healthcare facilities to treat endometrial cancer. The management of precancers of early endometrial precancerous conditions including fertility-preserving strategies is presented. The concept used for surgical primary therapy of endometrial cancer is described. Radiotherapy and adjuvant medical therapy to treat endometrial cancer and uterine carcinosarcomas are described. Recommendations are given for the follow-up care of endometrial cancer, recurrence and metastasis. Palliative medicine, psycho-oncology including psychosocial care, and patient information and rehabilitation are presented. Finally, the care algorithm and quality assurance steps for the diagnosis, therapy and follow-up of patients with endometrial cancer are outlined.

Basierend auf den aktuellen Publikationen zur Tumorgenese, Diagnostik, Therapie und Nachsorge wurde die aktuelle, interdisziplinär und interprofessionell erstellte S3-Leitlinie aktualisiert. Im vorliegenden Artikel werden praxisrelevante Änderungen diskutiert.Besonders wird auf Aspekte des Screenings, Patienteninformation, Prävention, Pathologie, prognostische Faktoren, Pathologie, Operation, Rezidivtherapie sowie Nachsorge und Langzeitüberleben eingegangen.

2.1 Target audience The guideline was compiled with the aim of providing high-risk groups with advice on diagnostics, surgical and systemic therapy in early and advanced stages of disease together with the treatment of rare histological subtypes. A lot of emphasis has been placed on follow-up care, rehabilitation, palliative therapy and psycho-oncological counselling. The recommendations are for physicians working both in hospitals and outpatient clinics, nursing staff and other medical partners involved in treating patients with malignant ovarian tumours. As it also covers the topics ‘Screening’ and ‘Follow-up’, registered physicians working in their own practice are also an important target audience of this guideline. It is additionally intended to offer guidance to affected patients and persons seeking more information as well as providing a basis for the gynaecological cancer centres currently being set up in Germany. For the first time, scientific medical societies in Switzerland and Austria were also consulted, expanding the scope of this guideline.

Hintergund/Zielsetzung: Abhangig vom Lymphknotenstatus entwickeln 15 – 40% der Patientinnen mit Vulvakarzinom ein Lokalrezidiv. Ein isoliertes Rezidiv im Bereich der Vulva gilt dabei als wenig relevant fur die weitere Prognose. Methoden: Die AGO-CaRE-1 Studie ist eine retrospektive Datensammlung zu Behandlungsstrategien und Prognosefaktoren des Vulvakarzinoms. Insgesamt wurden 1618 Patientinnen mit vulvarem Plattenepithelkarzinom FIGO Stadium ≥1B, behandelt an 29 deutschen Zentren 1998 – 2008, erfasst. Diese Subgruppenanalyse untersucht Patientinnen, die im Krankheitsverlauf ein isoliertes Lokalrezidiv entwickelten. Ergebnisse: 1249/1618 (77,2%) Patientinnen erhielten ein operatives Staging der Leiste und hatten einen bekannten Nodalstatus. Die meisten zeigten lokal begrenzte Primartumore [1124/1249 (90,0%) pT1b/pT2], die R0 reseziert wurden [1022/1249 (81,8%)]. 447/1249 (35,8%) Patientinnen waren nodalpositiv. Bei 324 (25,9%) wurde eine adjuvante Therapie durchgefuhrt. Das mediane Follow-up betrug 39,4 Monate. 360 Patientinnen (28,8%) entwickelten ein Rezidiv; davon 193 (53,6%) isoliert im Bereich der Vulva nach medianen 17,1 Monaten. Initial erhielten 38 (19,7%) dieser Patientinnen eine Radiotherapie der Vulva. Die Therapie des Lokalrezidives umfasste die alleinige Operation in 104 (53,9%), eine Operation und Radiotherapie in 40 (20,7%) und eine Radio(chemo)therapie in 14 (7,3%) Fallen [19 (9,8%) erhielten keine Rezidivtherapie und bei 16 (8,3%) war die Therapie unbekannt]. 58 (30,1%) entwickelten ein zweites Rezidiv in den folgenden Lokalisationen: 40 (20,7%) Vulva, 15 (7,8%) Leiste, 7 (3,6%) Becken, 12 (6,2%) Fernmetastasen, 2 (1,0%) unbekannt (multiple Nennung moglich). Die 1-Jahres-Rezidivfreie-Uberlebensrate nach Lokalrezidiv lag bei 58,5%. Das 3- und 5-Jahres-Gesamtuberleben betrug 76,7% bzw. 66,9% verglichen mit 85,9% und 80,6% bei Patientinnen ohne Lokalrezidiv. Zusammenfassung: Entgegen der allgemeinen Annahme zeigen AGO-CaRE-1 Patientinnen mit Lokalrezidiv eine beeintrachtigte Prognose. Ein wesentlicher Anteil der Betroffenen entwickelt ein Zweitrezidiv nach initial isoliertem Lokalrezidiv.

The role of persistent organochlorine compounds in the development of breast cancer is controversially discussed. Used as biocides or technical adjuvants for miscellaneous applications, organochlorines have been released into the environment for years. Due to their highly lipophilic properties, they have been able to accumulate in the food chain leading to a long lasting presence in human tissues. The evidence that organochlorines might be involved in the carcinogenesis of breast cancer is based on their complex toxicologic properties: organochlorines are able to influence estrogen-mediated and other endocrine processes; they induce microsomal enzyme systems with consecutive formation of genotoxic metabolites and activate the Her2-protooncogene product, c-neu tyrosine kinase. Several epidemiological studies have investigated the correlation between organochlorine exposure and the incidence of breast cancer. Although the majority of authors did not detect a positive correlation, the results remain inconclusive. Due to the common retrospective study design as well as the selection of the control population, the validity of some studies is questionable; due to the wide variation of tested compounds, many studies are hard to compare. In addition, genetically determined and individually varying sensitivity to organochlorines, synergistic interactions of several compounds, and evidence for the association of organochlorine exposure with poor prognosis in case of malignant transformation complicate the interpretation of many results. However, considering the available data, the involvement of organochlorine compounds in the carcinogenesis of breast cancer can not be excluded and therefore needs further investigation.

Fallopian tube carcinoma is the least common type of all gynecologic malignancies. Its low incidence led to therapeutic concepts derived from smaller retrospective studies with data collected over long time periods, resulting in a poorly defined management of this disease. As a result, every clinician is forced to decide on the basis of a low level of evidence. Alternatively, treatment of fallopian tube cancer today follows concepts derived from large prospective randomised trials in ovarian cancer. In comparison to ovarian cancer, fallopian tube carcinoma tends to be more often diagnosed as being of low FIGO stage at time of primary diagnosis, probably due to earlier symptoms. Together with increased retroperitoneal lymph node involvement, specific tumorbiological factors may be reasonable for that. However, despite these differences both malignancies share biological characteristics as well as clinical appearances. In keeping with standard treatment of ovarian carcinoma, recent results from retrospective trials with the largest collectives ever confirm the importance of cytoreductive surgery and platinum sensitivity for the treatment of fallopian tube cancer. Furthermore, strikingly similar frequencies of BRCA-1 and BRCA-2 mutations and patterns of genomic alterations in these tumour entities have recently suggested a common molecular pathogenesis. In summary of existing experiences, treatment of fallopian tube cancer may follow concepts derived from large prospective randomised trials in ovarian cancer.

HIPEC is offered to patients with ovarian, fallopian tube or primary peritoneal cancer at some hospitals. Altogether, there is still no evidence that HIPEC leads to an improvement of prognosis in any gynecologic tumor, neither in primary therapy nor in treatment of relapse. The available data indicate an increased complication rate which might negatively impact the benefit-risk balance of this procedure. In addition, standard treatment with proven efficacy might be withheld due to application of unproven methods. The use of HIPEC outside of well designed, prospective and controlled clinical trials is therefore disregarded.Die HIPEC wird Patientinnen mit einer durch ein Ovarial-, Tuben-, oder primären Peritonealkarzinom bedingten Peritonealkarzinose an einigen Kliniken angeboten. Insgesamt gibt es jedoch keinen Nachweis, dass bei einem gynäkologischen Tumor durch Einsatz der HIPEC weder in der Primär- noch in der Rezidivsituation eine Verbesserung der Prognose erreicht werden kann. Aufgrund der derzeitigen Datenlage kann durch eine erhöhte Komplikationsrate auch eine Verschlechterung der Prognose nicht ausgeschlossen werden, insbesondere wenn hierdurch den Patientinnen eine erwiesenermaßen wirksame Standardtherapie vorenthalten wird. Daher wird von einer Durchführung der HIPEC außerhalb von prospektiven kontrollierten Studien abgeraten.

Hamartomas can occur in different areas of the breast, but they are rarely found in the breast. Myoid hamartomas with smooth muscle cells of the type described here are particularly unusual. The pathogenesis of this benign entity with its tendency to growth and recurrence is not clear. Excision is the therapy of choice. Capillary hemangiomas are rare vascular malformations of the breast which, in contrast to cavernous hemangiomas, usually remain clinically occult. It is important to differentiate these benign findings from malignant angiosarcoma. The possible heterogeneities between myoid hamartoma and capillary hemangioma using current breast imaging methods for the differential diagnosis (high-resolution ultrasound, duplex sonography, shear wave elastography, digital mammography, minimally invasive intervention) are discussed together with an overview of the literature.Hamartome können in unterschiedlichen Körperregionen auftreten, sind in der Brust aber selten. Insbesondere die hier beschriebene Variante eines myoiden Hamartoms, welches glatte Muskelzellen enthält, ist außergewöhnlich. Die Pathogenese des gelegentlich wachstumsprogredienten und rezidivierenden myoiden Hamartoms ist nicht geklärt. Therapie der Wahl stellt die Exzision dar. Zu den raren vaskulären Neubildungen in der Mamma gehören die hier präsentierten kapillären Hämangiome, die klinisch im Gegensatz zu den kavernösen Hämangiomen meist inapparent bleiben. Zu differenzieren sind diese Befunde vom malignen Angiosarkom. In dieser Arbeit werden die möglichen Heterogenitäten des myoiden Hamartoms und der kapillären Hämangiome in der modernen bildgebenden Mammadiagnostik und ihre Differenzialdiagnosen (hochauflösende Sonografie, Duplex-Sonografie, Scherwellenelastografie, digitale Mammografie, minimalinvasive Abklärungsmethoden) zusammen mit einer Literaturübersicht ausführlich diskutiert.