Das Klippel-Trenaunay-Syndrom (KTS) ist eine sehr selten auftretende, angeborene Erkrankung, die durch ein flaches Hämangiom der Haut (port-winestain), am häufigsten am Rumpf, eine Knochen- und Weichteilhypertrophie der Extremitäten sowie eine Fehlbildung von Venen und Lymphgefäßen charakterisiert ist. Betroffen ist in der Regel nur eine Extremität, weitaus häufiger eine untere als obere [1] [2] [3] . Das Syndrom manifestiert sich bereits kurz nach der Geburt oder in früher Kindheit [4]. Beide Geschlechter sind gleichermaßen betroffen, es bestehen keine Rassenunterschiede. Die Hypertrophie der Extremität nimmt allmählich zu und kann bis zu einem lokalen Gigantismus führen. Dieses Syndrom wurde erstmals 1900 von den französischen Ärzten Klippel und Trenaunay beschrieben. Häufig wird es mit dem Parkes-Weber-Syndrom verwechselt und fälschlicherweise als Klippel-Trenaunay-Weber-Syndrom bezeichnet. Die Bezeichnung Parkes-Weber-Syndrom gilt für eine ähnliche Kombination aus Symptomen, doch zusätzlich besteht hier eine deutliche ,arteriovenöse Malformation (arteriovenöse Fistel ([Tab. 1]). Diese Klassifizierung wird von den meisten Autoren angenommen.
The aim of this retrospective study is to present our experience with the management of injuries to the vascular system at the Trauma Centre, University Hospital in Plzeň. We show the most frequent mechanisms of injury, diagnostic pitfalls, therapeutic options and evaluate both the short-term and long-term results of surgical treatment and interventional radiology procedures.We evaluated a group of patients with a serious vascular injury who were treated at the Traima Centre during the five-year period from January 2006 to December 2010. The group included 82 patients, 59 men and 23 women, with the average age of 34.5 years. We assessed the success rate of vascular reconstruction, primary and secondary graft patency and mortality and morbidity rates. Recovery of function in the injured extremities and return of the patient to normal daily activities were considered the most important parameters. In 28 patients (34%) vascular injury was due to polytrauma, in nine (11%) it was part of a combined injury, and 45 patients (55 %) sustained monotrauma. Most of the patients (68; 83%) were managed surgically. The most frequent procedures included direct suture of a lesion, resection and replacement of an injured artery or bypass grafting. In 13 patients we used interventional radiology procedures (in most cases it was for thoracic endovascular aortic repair). In one patient we were able to use a conservative treatment.The 30-day mortality rate was 8.5%, i.e., seven patients died of polytrauma. The morbidity rate was 35% (29 patients). The most frequent complications included respiratory failure in polytrauma (13%), transient leg/arm swelling (6%) and wound infection (4%). Primary and secondary graft patencies were 100% and 95.5%, respectively. Vascular reconstruction failed in three patients and, in two of them, we had to carry out limb amputation.From the viewpoint of vascular surgery, even very serious injuries of upper and lower extremities can be managed, if possible associated injuries of the skeletal and nerve structures allow for it. The key to success is good co-operation of the orthopaedic trauma surgeon, vascular surgeon and neurosurgeon.
The objective of this study was to assess the robustness of a novel test bolus (TB)-based computed tomographic angiography (CTA) contrast-enhancement-prediction (CEP) algorithm by retrospectively quantifying the systematic and random errors between the predicted and true enhancements.All local institutional review boards approved this retrospective study, in which a total of 72 (3 × 24) anonymized cardiac CTA examinations were collected from 3 hospitals. All patients (46 men; median age, 62 years [range, 31-81 years]) underwent a TB scan and a cardiac CTA according to local scan and injection protocols. For each patient, a shorter TB signal and TB signals with lower temporal resolution were derived from the original TB signal. The CEP algorithm predicted the enhancement in the descending aorta (DAo) on the basis of the TB signals in the DAo, the injection protocols and kilovolt settings, as well as population-averaged blood circulation characteristics. The true enhancement was extracted with a region of interest along the DAo centerline. For each patient, the errors in timing and amplitude were calculated; differences between the hospitals were assessed using the 1-way analysis of variance (P < 0.05) and variations between the TB signals were assessed using the within-subject standard deviation.No significant differences were found between the 3 hospitals for any of the TB signals. With errors in the amplitude and timing of 0.3% ± 15.6% and -0.2 ± 2.0 seconds, respectively, no clinically relevant systematic errors existed. Shorter- and coarser-time-sampled TB signals introduced a within-subject standard deviation of 4.0% and 0.5 seconds, respectively.This TB-based CEP algorithm has no systematic errors in the timing and amplitude of predicted enhancements and is robust against coarser-time-sampled and incomplete TB scans.
The authors present the case of a 57-year-old woman with a very rare extragenital malignant retroperitoneal Müllerian carcinosarcoma invading the inferior vena cava. Tumor resection with partial resection of the vena cava wall and resection of metastases in the pelvic area is described. The authors further discuss diagnostic options of metastases of this tumour and the recommended adjuvant chemotherapy.extragenital Müllerian carcinosarcoma malignant mixed Müllerian tumour - diagnosis therapy.
INTRODUCTION: Patients in advanced stages of non-small cell lung carcinomas (NSCLC), who and unsuited for targeted biological therapy because of lack of actionable molecular predictors are frequently treated by anti-angiogenic therapy. The effectiveness of such therapy is primarily relying on imaging techniques including CT or hybrid PET/CT evaluating a combination of morphological factors (dimensions and volume) and, more recently, also functional parameters including the metabolic activity, tumor vascularization etc. The ability to track the course of the disease at the same time all of these parameters is prerequisite to enable timely monitoring and therapy change in case of an early detection of resistance. This study was aimed at utility of circulating-tumor DNA (ctDNA) as an tool for monitoring of therapy response. and assessment of phenotypic parameters of the tumor.PATIENTS AND METHODS: A total of 50 patients with confirmed Stage IV lung adenocarcinomas showing negativity on ALK, BRAF, EGFR, RET, ROS1 and MET predictors were prospectively enrolled into the study. Patients were treated under a standard protocol by a combination of paclitaxes/carboplatin/bevacizumab and followed by dual PET/CT. For each patient cytology tissue sample was subjected to test for a panel of somatic mutations. The found mutations were subsequently detected in ctDNA in plasma extracted from peripheral blood collected prior to therapy start and then in 1-month intervals during the therapy. The occurrence and quantity of ctDNA were correlated with the objective therapy response (RECIST) and to the functional imaging parameters of metabolic activity and vascularization.RESULTS: ctDNA was initially positive in 29 patients. ctDNA levels closely reflected the response to the therapy with complete or partial remission expressed as reduction or absence of ctDNA, while disease stabilization or progression signaled by persisting or increasing ctDNA levels. There was a borderline correlation between ctDNA and vascularization evaluated by dual PET/CT (p=0.055).CONCLUSION: In a subset of patients ctDNA can readily serve as a surrogate marker for semi-continuous monitoring of the effect of anti-angiogenic therapy. Work supported by Czech Ministry of Health project AZV 17-30748A.Citation Format: Marek Minarik, Martin Svaton, Barbora Belsanova, Anastasiya Semyakina, Jan Baxa, Ondrej Fiala, Milos Pesek, Lucie Benesova. Application of a serial liquid biopsy ctDNA assay for monitoring efficacy of anti-angiogenic lung cancer therapy [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2019; 2019 Mar 29-Apr 3; Atlanta, GA. Philadelphia (PA): AACR; Cancer Res 2019;79(13 Suppl):Abstract nr 412.
