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Zusammenfassung Hintergrund Keimstrangstroma(SCST)- und Keimzelltumoren (MOGCT) sind seltene ovarielle Neoplasien, die häufig bei Jugendlichen oder prämenopausalen Patientinnen auftreten. Die Prognose ist in allen Stadien gut, weshalb psychisches Wohlbefinden, Lebensqualität, Fertilitätserhalt und Kinderwunsch zu relevanten Themen der Langzeitüberlebenden werden. Ziel der Arbeit Untersuchung der Prävalenz der fertilitätserhaltenden Therapie bei Patientinnen mit ovariellen Keimstrangstromatumoren und Keimzelltumoren und die Erfassung von Zusammenhängen mit Angst, Depression, Lebensqualität und Sexualität nach Therapie. Material und Methoden Die Current-Ovarian-geRM-cell-and-Sex-Cord-stromal-Tumour-Treatment-strategies(CORSETT)-Studie ist eine multizentrische, retro- und prospektiv beobachtende Kohortenstudie der Arbeitsgemeinschaft für Gynäkologische Onkologie (AGO) der Deutschen Krebsgesellschaft. Patientinnen nach Keimstrangstroma- oder Keimzelltumorbehandlung wurden gebeten, Fragebögen zu beantworten (Hospital Anxiety and Depression Scale [HADS], Sexual Activity Questionnaire [SAQ], European Organisation for Research and Treatment of Cancer Core Quality of Life Questionnaire [EORTC QLQ-C30]). Im Fokus dieser Analyse standen der Stellenwert der fertilitätserhaltenden Operation sowie die Ausprägung und potenziellen Zusammenhänge mit Sexualität, Lebensqualität, Schmerz, Angst und Depressivität. Ergebnisse Von 355 eingeschlossenen Patientinnen hatten 152 Patientinnen die Fragebögen ausgefüllt und wurden in die Analysen eingeschlossen. 85 Patientinnen hiervon (56 %) wurden fertilitätserhaltend operiert. 34 % der Patientinnen hatten klinisch relevante Angst und Depressivität. Patientinnen nach fertilitätserhaltender Operation waren signifikant häufiger sexuell aktiv, hatten mehr sexuelles Verlangen und litten seltener unter Angst und Depressivität als Patientinnen ohne fertilitätserhaltende operative Therapie. Das Ausmaß von Angst und Depressivität korrelierte mit dem Schmerzniveau, unabhängig vom zeitlichen Abstand zur Krebsdiagnose. Diskussion Die fertilitätserhaltende operative Therapie sollte, wann immer onkologisch vertretbar, den Patientinnen mit SCST und MOGCT angeboten werden, um den Krebsüberlebenden bestmögliche Lebensqualität zu ermöglichen. Ein wiederholtes Screening auf Angst, Depressivität, Schmerzen und Probleme in der Sexualität sollte anhand von standardisierten Fragebögen bei allen Patientinnen durchgeführt werden.

Introduction The intention of this study was to evaluate the level of anxiety and depression of malignant ovarian germ cell (MOGCT) and sex cord stromal tumors (SCST) survivors and to identify possible alterable cofactors. Methods CORSETT was an observational, multicenter, mixed retrospective/prospective cohort study of the AGO Studygroup. Women who had been diagnosed with MOGCTs and SCSTs between 2001 and 2011 were asked to complete the Hospital Anxiety and Depression Scale (HADS) to evaluate distress. Predictors of distress (type of surgery, chemotherapy, time since diagnosis, recurrence, second tumor, pain) were investigated using multivariate linear regression analysis. Results 150 MOGCT and SCST patients with confirmed histological diagnosis completed the questionnaire median seven years after diagnosis. They had a HADS total score ≥ 13 indicating severe mental distress in 34% of cases. Patients after fertility-conserving surgery had lower probability of severe mental distress than those without fertility-conserving treatment (β = − 3.1, p = 0.04). Pain was associated with the level of distress in uni- and multivariate analysis (coef 0.1, p Discussion Severe mental distress was frequent in patients with MOGCT and SCST and the level of pain was associated with the level of distress. Fertility conserving therapy, however, was associated with less mental distress. Screening and treatment of pain and depression is required to improve mental well-being in survivors of MOGCT and SCST.

The intention of this study was to evaluate the level of anxiety and depression of malignant ovarian germ cell (MOGCT) and sex cord stromal tumors (SCST) survivors and to identify possible alterable cofactors.CORSETT was an observational, multicenter, mixed retrospective/prospective cohort study of the AGO Studygroup. Women who had been diagnosed with MOGCTs and SCSTs between 2001 and 2011 were asked to complete the Hospital Anxiety and Depression Scale (HADS) to evaluate distress. Predictors of distress (type of surgery, chemotherapy, time since diagnosis, recurrence, second tumor, pain) were investigated using multivariate linear regression analysis.150 MOGCT and SCST patients with confirmed histological diagnosis completed the questionnaire median seven years after diagnosis. They had a HADS total score ≥ 13 indicating severe mental distress in 34% of cases. Patients after fertility-conserving surgery had lower probability of severe mental distress than those without fertility-conserving treatment (β = - 3.1, p = 0.04). Pain was associated with the level of distress in uni- and multivariate analysis (coef 0.1, p 0.01, coef. Beta 0.5).Severe mental distress was frequent in patients with MOGCT and SCST and the level of pain was associated with the level of distress. Fertility conserving therapy, however, was associated with less mental distress. Screening and treatment of pain and depression is required to improve mental well-being in survivors of MOGCT and SCST.

Despite recent advances in the treatment of ovarian cancer (OC), long-term remissions remain scarce. For a targeted approach, prognostic markers are indispensable for predicting survival and treatment response. Given their association with multiple hallmarks of cancer, histamine receptors (HR) are emerging as promising candidates. Here, we investigate their expression pattern and prognostic value in OC.Specimens of 156 epithelial OC patients were collected during cytoreductive surgery at the Department of Obstetrics and Gynecology, LMU, between 1990 and 2002 and combined in a tissue microarray. Immunohistochemical staining of the HR H1, H2, H3 and H4 was quantified by an immunoreactive score and linked with clinico-pathological data by Spearman's correlation. Via ROC curve analysis, optimal cut-off values for potential prognostic markers were defined. Overall survival (OS) was visualized in Kaplan-Maier curves and significances determined by log-rank testing. A Cox regression model was applied for multivariate analysis.HR H3 and H4 expression was restricted to the cytosol of OC cells, while H1 was also present in the nucleus. A significant association between HR H1, H3 and H4 expression with several clinico-pathological parameters was revealed. In addition, HR H1 and H3 expression correlated positively, HR H4 expression negatively with OS. In addition, HR H3 was identified as independent prognostic marker for OS. HR H2 expression had no prognostic value.HR H1, H3 and H4 could serve as potential predictors for OS of OC patients. Further research is warranted to elucidate their pathophysiologic role and their predictive and therapeutic potential in OC.

The aim of this study was to analyse the course of adrenomedullin (ADM) and endothelin-1 (ET-1) levels in patients with vasodilatory shock after cardiac surgery and to explore differences compared to patients after uncomplicated coronary artery bypass graft (CABG) surgery. ADM and ET-1 are involved in the vasomotor response during vasodilatory shock.We included 32 patients with vasodilatory shock (study group) and 10 patients after uncomplicated CABG surgery (control group). Daily measurements of MR-proADM and CT-proET-1 (stable surrogate markers for ADM and ET-1) were collected during the first 7 postoperative days.MR-proADM and CT-proET-1 levels were significantly elevated in the study group when compared to the control group. In addition, the course of both biomarkers was significantly different in the study versus control group. Higher levels of both biomarkers were associated with organ dysfunction (higher maximum multiple organ dysfunction score, acute kidney injury).Significantly higher levels of MR-proADM and CT-proET-1 and a different course of both biomarkers were observed in patients with vasodilatory shock after cardiac surgery and seemed to be associated with organ dysfunction.

Background In Europe, dietary management of isovaleric acidemia (IVA) may vary widely. There is limited collective information about dietetic management. Aim To describe European practice regarding the dietary management of IVA, prior to the availability of the E-IMD IVA guidelines (E-IMD 2014). Methods A cross-sectional questionnaire was sent to all European dietitians who were either members of the Society for the Study of Inborn Errors of Metabolism Dietitians Group (SSIEM-DG) or whom had responded to previous questionnaires on dietetic practice (n = 53). The questionnaire comprised 27 questions about the dietary management of IVA. Results Information on 140 patients with IVA from 39 centres was reported. 133 patients (38 centres) were given a protein restricted diet. Leucine-free amino acid supplements (LFAA) were routinely used to supplement protein intake in 58% of centres. The median total protein intake prescribed achieved the WHO/FAO/UNU [2007] safe levels of protein intake in all age groups. Centres that prescribed LFAA had lower natural protein intakes in most age groups except 1 to 10 y. In contrast, when centres were not using LFAA, the median natural protein intake met WHO/FAO/UNU [2007] safe levels of protein intake in all age groups. Enteral tube feeding was rarely prescribed. Conclusions This survey demonstrates wide differences in dietary practice in the management of IVA across European centres. It provides unique dietary data collectively representing European practices in IVA which can be used as a foundation to compare dietary management changes as a consequence of the first E-IMD IVA guidelines availability., 0, SCOPUS: ar.j, info:eu-repo/semantics/published

Tumor stage, residual postoperative tumor, histological type and grading are considered among the main prognostic parameters in the consensus-based recommendations in the new S3 guidelines for diagnosis, treatment and clinical follow-up of malignant tumours of the ovary. Based on the 2014 update of the WHO Classification of Tumours of the Female Reproductive Organs this article summarizes the most significant changes. For example now the same TNM and FIGO classification applies for tumours of the ovary, peritoneum or fallopian tube. Noninvasive implants of serous borderline tumours are now named implants. In contrast, invasive implants are regarded as low-grade serous carcinoma. By presenting the current background information, we want to provide a basis for discussion, regarding more detailed consensus recommendations for pathologists and clinicians in the future.

The present article summarises the relevant aspects of the S3 guidelines on endometrioid carcinomas. The recommendations include the processing rules of fractional currettings as well as for hysterectomy specimens and lymph node resections (including sentinel lymph nodes). Besides practical aspects, the guidelines consider the needs of the clinicians for appropriate surgical and radiotherapeutic treatment of the patients. Carcinosarcomas are assigned to the endometrial carcinoma as a special variant. For the first time, an algorithmic approach for evaluation of the tumour tissue for Lynch syndrome is given. Prognostic factors based on morphologic findings are summarised.

WOS: 000442229500021, PubMed ID: 30101073, Background: In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe. Methods: We sent a cross sectional, survey monkey (R) questionnaire to European health professionals working in IMD. It contained 31 open and multiple-choice questions. The results were analysed according to different geographical regions. Results: Ninety-five centres from 21 countries responded. Over 60% of centres commenced diet in infants by age 10 days, with 58% of centres implementing newborn screening by day 3 post birth. At diagnosis, infant hospital admission occurred in 61% of metabolic centres, mainly in Eastern, Western and Southern Europe. Breastfeeding fell sharply following diagnosis with only 30% of women still breast feeding at 6 months. 53% of centres gave pre-measured Phe-free infant formula before each breast feed and 23% alternated breast feeds with Phe-free infant formula. With standard infant formula feeds, measured amounts were followed by Phe-free infant formula to satiety in 37% of centres (n = 35/95), whereas 44% (n = 42/95) advised mixing both formulas together. Weaning commenced between 17 and 26 weeks in 85% centres, >= 26 weeks in 12% and < 17 weeks in 3%. Discussion: This is the largest European survey completed on PKU infant feeding practices. It is evident that practices varied widely across Europe, and the practicalities of infant feeding in PKU received little focus in the PKU European Guidelines (2017). There are few reports comparing different feeding techniques with blood Phe control, Phe fluctuations and growth. Controlled prospective studies are necessary to assess how different infant feeding practices may influence longer term feeding development., Vitaflo, We thank Vitaflo for supporting the publication cost of this paper.

WOS: 000423688500002, PubMed ID: 29370874, Background: In fructose 1,6 bisphosphatase (FBPase) deficiency, management aims to prevent hypoglycaemia and lactic acidosis by avoiding prolonged fasting, particularly during febrile illness. Although the need for an emergency regimen to avoid metabolic decompensation is well established at times of illness, there is uncertainty about the need for other dietary management strategies such as sucrose or fructose restriction. We assessed international differences in the dietary management of FBPase deficiency. Methods: A cross-sectional questionnaire (13 questions) was emailed to all members of the Society for the Study of Inborn Errors of Metabolism (SSIEM) and a wide database of inherited metabolic disorder dietitians. Results: Thirty-six centres reported the dietary prescriptions of 126 patients with FBPase deficiency. Patients' age at questionnaire completion was: 1-10y, 46% (n = 58), 11-16y, 21% (n = 27), and >16y, 33% (n = 41). Diagnostic age was: 16y, 2% (n = 2). Seventy-five per cent of centres advocated dietary restrictions. This included restriction of: high sucrose foods only (n = 7 centres, 19%); fruit and sugary foods (n = 4, 11%); fruit, vegetables and sugary foods (n = 13, 36%). Twenty-five per cent of centres (n = 9), advised no dietary restrictions when patients were well. A higher percentage of patients aged >16y rather than 16y, 85% (n = 35/41). Patients classified as having a normal fasting tolerance increased with age from 30% in 1-10y, to 36% in 11-16y, and 58% in >16y, but it was unclear if fasting tolerance was biochemically proven. Twenty centres (56%) routinely prescribed uncooked cornstarch (UCCS) to limit overnight fasting in 47 patients regardless of their actual fasting tolerance (37%). All centres advocated an emergency regimen mainly based on glucose polymer for illness management. Conclusions: Although all patients were prescribed an emergency regimen for illness, use of sucrose and fructose restricted diets with UCCS supplementation varied widely. Restrictions did not relax with age. International guidelines are necessary to help direct future dietary management of FBPase deficiency., ERDF - Programa Operacional Competitividade e Internacionalizacao - COMPETE [POCI-01-0145-FEDER-007746]; FCT - Fundacao para a Ciencia e a Tecnologia within CINTESIS, RD Unit [UID/IC/4255/2013], This article was supported by ERDF through the operation POCI-01-0145-FEDER-007746 funded by the Programa Operacional Competitividade e Internacionalizacao - COMPETE2020 and by National Funds through FCT - Fundacao para a Ciencia e a Tecnologia within CINTESIS, R&D Unit (reference UID/IC/4255/2013).

Tumor stage, residual postoperative tumor, histological type and grading are considered among the main prognostic parameters in the consensus-based recommendations in the new S3 guidelines for diagnosis, treatment and clinical follow-up of malignant tumours of the ovary. Based on the 2014 update of the WHO Classification of Tumours of the Female Reproductive Organs this article summarizes the most significant changes. For example now the same TNM and FIGO classification applies for tumours of the ovary, peritoneum or fallopian tube. Noninvasive implants of serous borderline tumours are now named implants. In contrast, invasive implants are regarded as low-grade serous carcinoma. By presenting the current background information, we want to provide a basis for discussion, regarding more detailed consensus recommendations for pathologists and clinicians in the future.

Background Obese patients are often not fully dosed when receiving adjuvant chemotherapy for breast cancer, which might influence the inferior outcomes seen in this patient population. Current guidelines recommend full, weight-based dosing of chemoherapy for obese patients. The aim of the present study was to assess toxicity and deliverable dose intensity in patients treated with adjuvant chemotherapy for breast cancer. Patients and Methods We conducted a single-center retrospective case review on patients treated with adjuvant chemotherapy for early breast cancer at our institution from 2009 to 2012. Data were extracted from electronic patient records and side effect were graded according to CTC AE 3.0 in the source data. Statistics: Chi-Square, Fisher's exact test, MANOVA and repeated measures ANOVA. Software: SPSS 18. The study received approval from the local ethics comitee. Results We included 187 patients (185 female, 2 male) who received a total of 924 courses of chemotherapy with full eight-based dosing with either 6 courses of FEC-T (n = ,%) or TC (n = ,%). There were no differences accross BMI categories for age, gender, endocrine receptor expression, grading or her2-status. Obese patients had more unplanned admission (p = 0.008), infections (grade 3-4, p = 0.005, all grades, p = 0.025) and diarrhoe (all grades, p = 0.033) compared to normal-weight patients. This was not the case for overweight patients (p = 0.063, 0.322, 0.199, 0.107, 0.065). Obese and overweight patients had more constipation than normal-weight patients (all grades, p = 0.025 and 0.006, respectively). There were no signficant differences across weight categories for neutropenic fever, delivered dose intensity, dose reduction and premature discontinuation of therapy. Discussion Obese and overweight patients can be treated with full dose intensity and do not suffer from excessive serious complications compared to normal-weight patients. However, due to partly increased toxicity seen in obese patients, adequate supportive therapy is of particular importance. Citation Information: Cancer Res 2013;73(24 Suppl): Abstract nr P3-12-12.

Histopathological assessment of the tumor grade and cell type is central to the management and prognosis of various gynecological malignancies. Conventional grading systems for squamous carcinomas and adenocarcinomas of the vulva, vagina and cervix are poorly defined. For endometrioid tumors of the female genital tract as well as for mucinous endometrial, ovarian and seromucinous ovarian carcinomas, the 3‑tiered FIGO grading system is recommended. For uterine neuroendocrine tumors the grading system of the gastrointestinal counterparts has been adopted. Uterine leiomyosarcomas are not graded. Endometrial stromal sarcomas are divided into low and high grades, based on cellular morphology, immunohistochemical and molecular findings. A chemotherapy response score was established for chemotherapeutically treated high-grade serous pelvic cancer. For non-epithelial ovarian malignancies, only Sertoli-Leydig cell tumors and immature teratomas are graded. At this time molecular profiling has no impact on the grading of tumors of the female genital tract.

Free asymmetric dimethylarginine (ADMA) is a competitive inhibitor of the nitric oxide synthases (NOS). Suppression of nitric oxide (NO) synthesis increases the risk of atherosclerosis. Nevertheless, in the condition of oxidative stress, NOS blockade by ADMA may exert protective effects. Protein metabolism is altered in patients with phenylketonuria (PKU) on dietary treatment and as shown recently, oxidative stress is high in PKU. Since free ADMA concentrations are determined by both protein metabolism and oxidative stress we hypothesized, that free ADMA levels may be elevated in PKU patients.Sixteen patientswith PKU on dietary treatment (mean age 10.1 ± 5.2 yrs), and 91 healthy children (mean age 11.6 ± 3.7 yrs) participated in a cross sectional study.ADMA, total homocysteine (tHcy) and blood glucose were lower and the L-arginine/ADMA ratio was higher in PKU patients compared to controls. No significant correlation was present between phenylalanine (Phe) concentrations, protein intake, and lipid profile, history of cardiovascular disease or ADMA.In contrast to our hypothesis, ADMAwas lower and the L-arginine/ADMA ratio was higher in PKU patients. Therefore, in PKU patients, the regulating function of ADMA on NO synthesis is altered and may thus contribute to oxidative stress.

Eine 42-jahrige OP-Schwester verletzte sich mit einem benutzten Skalpell am linken Ellenbogen. Nach zunachst leitliniengerechter Versorgung der Wunde erfolgte eine antiseptische Spulung mit Octenisept®. Die Patientin entwickelte im weiteren Verlauf eine starke Rotung, Uberwarmung und Druckschmerzhaftigkeit im Bereich der Stichverletzung. Therapieversuche mit Antibiotika oder Cortison unter der Annahme einer infektiosen oder allergisch-toxischen Genese waren erfolglos. Einen Monat nach dem Unfallereignis wurde die Indikation zur operativen Exzision des schmerzhaften und entzundlich veranderten Gewebes gestellt. Die histopathologische Untersuchung von Haut- und Subkutangewebe zeigte das Bild einer Weichgewebsnekrose mit ausgepragter resorptiv-histiozytarer Entzundung, welche sich durch eine Ansammlung von Schaumzellen um die ausgepragte Fettgewebsnekrose darstellte. Zusatzlich fand sich eine bereits chronisch-granulierende, fibrosierende Entzundung. Das Geschehen ist wahrscheinlich auf die fehlerhafte Anwendung des topischen Antiseptikums Octenisept® zuruckzufuhren, das bei der Spulung der Wunde ohne Abflussmoglichkeit tief ins Gewebe eingebracht wurde. Ursachlich fur das Geschehen durfte der Sachverhalt sein, dass Octenidindihydrochlorid nicht verstoffwechselt werden und entsprechende entzundliche Reaktionen im subkutanen Gewebe verursachen kann. Unmittelbar nach der operativen Exzision des veranderten Gewebes war die Schmerzsymptomatik der Patientin deutlich rucklaufig, der weitere Verlauf gestaltete sich komplikationslos.