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Einleitung: Im LTR werden Daten von Patienten mit malignen und benignen Lebertumoren im Kindesalter erfasst. Ziel ist es, die Erkenntnisse und Therapieempfehlungen insbesondere auch zu den gutartigen Tumoren hinsichtlich Epidemiologie, Pathogenese und Therapie zu erweitern. Ebenso dient das Register[zum vollständigen Text gelangen Sie über die oben angegebene URL], 133. Kongress der Deutschen Gesellschaft für Chirurgie

Fetal and neonatal renal tumors are rare. Nevertheless, in-depth understanding of their features can lead to early recognition and appropriate treatment, ultimately benefiting outcome. Despite the many obvious similarities, important distinctions exist between these tumors and their counterparts in older children. Likewise, some important distinctions exist between fetal tumors on the one hand and neonatal tumors on the other. In this article, we review the pertinent features of fetal and neonatal renal tumors and specifically point out the important individualities in their clinical management.

Background The treatment of Wilms Tumor is integrated into clinical trials since the 1970's. In contrast to the National Wilms Tumor Study Group (NWTSG) the SIOP trials and studies largely focus on the issue of preoperative therapy to facilitate surgery of a shrunken tumor and to treat metastasis as early as possible. Patients and methods In the SIOP 93-01/GPOH trial and study 1 020 patients with a newly diagnosed renal tumor were registered. 847 of them had a histological proven Wilms Tumor, of whom 637 were unilateral localized, and 173 tumors had an other histology [40 congenital mesoblastic nephroma (CMN), 51 clear cell sarcoma (CCSK), 24 rhabdoid tumor (RTK) and 58 other tumors]. Preoperative chemotherapy in benign tumors was given to 1.3 % of the patients. The main objective of the trial was the randomized question, if the postoperative two drug chemotherapy for stage I in intermediate risk or anaplasia can be reduced from conventional 3 courses to an experimental 1 course without loss of efficacy. Results 519 patients with unilateral nonmetastatic Wilms did receive preoperative chemotherapy. The histology in this group of patients was of intermediate risk in 469 (90 %) patients, 14 (3 %) tumors were low risk and 36 (7 %) high risk. The stage distribution of the tumors was stage I in 315 (61 %), stage II N- in 126 (24 %), stage II N+ in 25 (5 %) and stage III in 36 (7 %) patients. In 17 (3 %) patients the tumor stage remained unclear. Tumor volume was measured in 487 patients before and in 402 after preoperative chemotherapy. The median tumor volume did shrink from 353 to 126 ml. The amount of volume reduction depends on the histological subtype. The event free survival (EFS) after 5 years was 91 % for all patients with unilateral Wilms tumor without distant metastasis. Randomisation was done in 43.7 % for stage I patients and there was no difference in EFS for both treatment arms (90 versus 91 %). The EFS is identical for patients with stage I and II N- (0.92), as well as for stage II N+ and III (0.82). The tumor volume after chemotherapy is a prognostic factor for intermediate risk tumors with the exception of epithelial and stromal predominant tumors. These two subtypes often present as large tumors, they do not shrink during preoperative chemotherapy but they still have an excellent prognosis. On the other hand the prognosis of patients with blastemal predominant subtype after preoperative chemotherapy is worse than in any other patient group of intermediate risk tumors. There are less blastemal predominant tumors compared to primary surgery, but they are chemotherapeutic resistant selected by the preoperative chemotherapy. Conclusion Patients with unilateral Wilms tumor without metastasis have an excellent prognosis. The post-operative chemotherapy in stage I can be reduced to 4 weeks without worsening treatment outcome. The reduction of the tumor volume could be identified as a helpful marker for stratification of post-operative treatment. Post-chemotherapy blastemal predominant subtype of Wilms tumor has to be classified as high risk tumor. Focal anaplasia has a better prognosis than diffuse anaplasia and will be classified as intermediate risk tumor.

Purpose To analyse the surgical and oncological outcome of minimally invasive surgery (MIS) for tumor nephrectomy in Wilms tumor (WT) patients. Methods WT patients from the SIOP 2001 trial, undergoing MIS for tumor nephrectomy were analyzed with regard to demographic characterization, surgical specifications, complications, and outcome. Results There were 24 children matching the inclusion criteria. Median age at operation was 40.35 months (14.3–65.4). All patients received preoperative chemotherapy. Median tumor volume was 177.5 ml at diagnosis (46.5–958) and 73.0 ml at surgery (3.8–776). There was one surgical complication (splenic injury), no intraoperative tumor rupture occurred. Abdominal stage was I in 14, II in 7, and III in 3 patients. Adequate lymph node sampling was performed in only 2 patients. One local relapse occurred. Event-free survival was 23/24, overall survival was 24/24, median follow up was 47 months (2–114). Conclusions We present the largest series so far of minimally invasive nephrectomies for nephroblastoma based on a multinational trial. Treatment results were comparable to those of open surgery; however, experience of operating surgeons was generally high. Discipline of lymph node sampling was inadequate. Based on this analysis a prospective study on MIS in nephroblastoma is planned by the SIOP Renal Tumor Study Group.

Einleitung: Das international neuroblastoma risk group staging system (INRGSS) dient der prätherapeutischen Risikostratifizierung von Neuroblastom-Patienten (Monclair et al, 2009). Wir führten eine retrospektive Auswertung zwischen dem Vorliegen von IDRF und dem Outcome von chirurgisch behandelten[for full text, please go to the a.m. URL], 130. Kongress der Deutschen Gesellschaft für Chirurgie

Hintergrund & Fragestellung: Der M. Hunter (Mukopolysaccharidose Typ II) ist eine x-chromosomal vererbte Erkrankung, bei der es durch einen Enzymmangel von Iduronat-2-Sulfatase durch fortschreitende Akkumulation von Glykosaminoglykanen in zahlreichen Organsystemen zu spezifischen Krankheitssymptomen[for full text, please go to the a.m. URL], Süddeutsche Tage der Kinder- und Jugendmedizin; 61. Jahrestagung der Süddeutschen Gesellschaft für Kinder- und Jugendmedizin und der Süddeutschen Gesellschaft für Kinderchirurgie und dem Berufsverband für Kinder- und Jugendärzte – Landesverband Bayern

Einleitung: Die Resektion von Hepatoblastomen stellt nach wie vor eine große Herausforderung dar, postoperative Komplikationen sind dabei häufig. Ziel dieser Analyse war es, den Einfluss solcher Komplikationen auf die Prognose zu untersuchen. Methoden: Im Rahmen der GPOH-Studie HB99 wurden[for full text, please go to the a.m. URL], Süddeutsche Tage der Kinder- und Jugendmedizin; 61. Jahrestagung der Süddeutschen Gesellschaft für Kinder- und Jugendmedizin und der Süddeutschen Gesellschaft für Kinderchirurgie und dem Berufsverband für Kinder- und Jugendärzte – Landesverband Bayern

Accumulated experimental evidence indicates that Hedgehog (Hh) signaling regulates cell proliferation and specification in a variety of organs during embryonic development. However, abnormal activation of this pathway in postnatal tissues has been linked to a large number of human cancers. With respect to the liver, it is known that Hh signaling not only influences bipotential precursor cells capable of pancreas and liver development, but is also implicated in the pathogenesis of liver tumors such as hepatoblastoma, hepatocellular and cholangiocellular carcinoma, if aberrantly activated. Blockade of Hh signaling by several specific inhibitors has been proven to successfully inhibit tumor growth of various Hh-associated cancers in vitro and in preclinical mouse models, and recent clinical data suggest that the implementation of novel anticancer therapeutics based on Hh interference into commonly accepted regimens are within reach. Thus, it is highly probable that Hh targeted therapies could be used for the treatment of Hh-dependent liver cancers in the future.

Während paraneoplastische Syndrome im Erwachsenenalter eine allgemein bekannte Differentialdiagnose unklarer Endokrinopathien sind, stellen sie im pädiatrischen Patientenkollektiv eine äußerste Rarität dar. Noch bemerkenswerter ist eine endokrine Aktivität primärer[for full text, please go to the a.m. URL], 60. Jahrestagung der Norddeutschen Gesellschaft für Kinder- und Jugendmedizin