TY  - JOUR
AU  - von Frowein, J.*
AU  - Hauck, S.M.
AU  - Kappler, R.*
AU  - Pagel, P.*
AU  - Fleischmann, K.K.*
AU  - Magg, T.*
AU  - Cairo, S.*
AU  - Roscher, A.*
AU  - von Schweinitz, D.*
AU  - Schmid, I.*
C1  - 54487
C2  - 45580
CY  - 111 River St, Hoboken 07030-5774, Nj Usa
SP  - S366-S366
TI  - miR-492 regulates metastatic Ppoperties of hepatoblastoma via CD44.
JO  - Pediatr. Blood Cancer
VL  - 65
PB  - Wiley
PY  - 2018
SN  - 1545-5009
ER  - 

TY  - JOUR
AU  - Hensel, T.*
AU  - Giorgi, C.*
AU  - Becker-Dettling, F.*
AU  - Calzada-Wack, J.
AU  - Neff, F.
AU  - Schmidt, O.*
AU  - Schäfer, B.*
AU  - Burdach, S.*
AU  - Richter, G.*
C1  - 50232
C2  - 42135
CY  - Hoboken
SP  - S20-S21
TI  - Combined targeting of the EWS/ETS Ttanscriptional program by BET bromodomain and PI3K pathway inhibition blocks tumorigenicity and increases apoptosis in ewing sarcoma.
JO  - Pediatr. Blood Cancer
VL  - 63
PB  - Wiley-blackwell
PY  - 2016
SN  - 1545-5009
ER  - 

TY  - JOUR
AU  - Nathrath, M.
AU  - Smida, J.
AU  - Baumhoer, D.
AU  - Kovac, M.*
C1  - 47117
C2  - 39210
SP  - S164
TI  - The genomic landscape in osteosarcoma.
JO  - Pediatr. Blood Cancer
VL  - 62
PY  - 2015
SN  - 1545-5009
ER  - 

TY  - JOUR
AU  - Bozkurt, C.*
AU  - Trippel, F.*
AU  - Kanmaz, T.*
AU  - Acarli, K.*
AU  - Leuschner, I.*
AU  - Sahin, G.*
AU  - Schwarzmayr, T.
AU  - von Schweinitz, D.*
AU  - Strom, T.M.
AU  - Kappler, R.*
C1  - 42908
C2  - 35804
SP  - S198-S199
TI  - De novo mutation of Rb1 in monozygotic twins with hepatoblastoma.
JO  - Pediatr. Blood Cancer
VL  - 61
PY  - 2014
SN  - 1545-5009
ER  - 

TY  - JOUR
AB  - BACKGROUND: Malignant ectomesenchymoma (MEM) is a soft tissue tumor with heterologous rhabdomyoblastic components believed to arise from pluripotent migratory neural crest cells. To date merely 50 cases have been published and the knowledge about the course of disease and optimal treatment is limited. METHODS: Six patients with MEM were registered 1996-2009. The diagnosis was confirmed according to current criteria. Their treatment and outcome was analyzed. RESULTS: The median age of the three females and three males was 0.6 years (range, 0.2-13.5). The mesenchymal component in all tumors was rhabdomyosarcoma (RMS), the neural component ganglioneuroblastoma/neuroblastoma (n = 5) and peripheral primitive neuroectodermal tumor in one case. Five patients presented with localized, one with metastatic disease. All but one patient received multiagent chemotherapy during their initial treatment. The tumors of 4/5 patients with localized MEM were at least grossly resected at best surgery; the patient without gross resection was additionally irradiated. Three of four evaluable tumors responded well to induction chemotherapy. All patients achieved a first complete remission (CR), but three recurrences (two local, one systemic) occurred. The individual with metastatic MEM did not survive, but all five patients with localized MEM are currently alive in CR with a median follow-up of 5 years (range: 2.1-13.7). CONCLUSIONS: Risk-factors and outcome of MEM appear to be comparable with other highly malignant pediatric soft tissue sarcoma when a multimodal treatment strategy including chemotherapy and adequate local treatment is pursued. We propose that treatment of patients with MEM be done according to pediatric protocols similar to other rhabdomyosarcoma-like soft tissue sarcoma.
AU  - Dantonello, T.M.*
AU  - Leuschner, I.*
AU  - Vokuhl, C.*
AU  - Gfroerer, S.*
AU  - Schuck, A.*
AU  - Kube, S.*
AU  - Nathrath, M.
AU  - Bernbeck, B.*
AU  - Kaatsch, P.*
AU  - Pal, N.*
AU  - Ljungman, G.*
AU  - Bielack, S.S.*
AU  - Klingebiel, T.*
AU  - Koscielniak, E.*
C1  - 11022
C2  - 30467
SP  - 224-229
TI  - Malignant ectomesenchymoma in children and adolescents: Report from the Cooperative Weichteilsarkom Studiengruppe (CWS).
JO  - Pediatr. Blood Cancer
VL  - 60
IS  - 2
PB  - Wiley-Blackwell
PY  - 2013
SN  - 1545-5009
ER  - 

TY  - JOUR
AB  - BACKGROUND: Recurrences in primary localized alveolar rhabdomyosarcoma (RMA) are common. Post-relapse survival is poor. We evaluated prognostic factors including relapse treatment in patients with recurrent RMA. METHODS: Relapses occurred in 115/235 patients with nonmetastatic RMA treated in four consecutive CWS-trials after achievement of a complete remission. Sufficient information about post-relapse treatment and outcome could be obtained in 99 patients and was retrospectively analyzed. RESULTS: Nine of 99 patients received no salvage therapy and died after a median of 2 months. The remaining 90 patients received multimodal relapse treatment including mandatory chemotherapy. Recurrences were grossly resected in 39 patients; 57 patients received radiation. At a median follow-up from relapse of 8 years, 20 patients were alive and disease-free (5-year post-relapse survival [PROS] 21.3 ± 8). All surviving patients apart from a single individual had an isolated, circumscribed recurrence. Sixteen of 20 survivors were treated with adequate local relapse therapy (ALRT, i.e., either complete resection or gross resection + radiation). Survival in the subgroup of 27 individuals with circumscribed recurrences and ALRT was significantly better (PROS 53.7 ± 19) compared with disseminated recurrences and/or tumors treated without ALRT. Absence of primary lymph node involvement, circumscribed relapses, ALRT, and achievement of a second CR were identified as independent favorable risk factors. CONCLUSION: Post-relapse survival for primary localized RMA is generally poor. However, certain patient groups differed significantly in their likelihood of survival and 50% of patients with circumscribed relapses treated with ALRT survived. These findings may form the basis for an evidence-based risk-stratification for recurrent disease including relapse treatment. Pediatr Blood Cancer 2013;60:1267-1273. © 2013 Wiley Periodicals, Inc.
AU  - Dantonello, T.M.*
AU  - Int-Veen, C.*
AU  - Schuck, A.*
AU  - Seitz, G.*
AU  - Leuschner, I.*
AU  - Nathrath, M.
AU  - Schlegel, P.G.*
AU  - Kontny, U.*
AU  - Behnisch, W.*
AU  - Veit-Friedrich, I.*
AU  - Kube, S.*
AU  - Hallmen, E.*
AU  - Kazanowska, B.*
AU  - Ladenstein, R.*
AU  - Paulussen, M.*
AU  - Ljungman, G.*
AU  - Bielack, S.S.*
AU  - Klingebiel, T.*
AU  - Koscielniak, E.*
AU  - Cooperative Weichteilsarkom Studiengruppe (CWS)*
C1  - 26118
C2  - 32078
SP  - 1267-1273
TI  - Survival following disease recurrence of primary localized alveolar rhabdomyosarcoma.
JO  - Pediatr. Blood Cancer
VL  - 60
IS  - 8
PB  - Wiley-Blackwell
PY  - 2013
SN  - 1545-5009
ER  -