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01-10-2014 | Bone and Soft Tissue Sarcomas

Challenges in the Local Treatment of Large Abdominal Embryonal Rhabdomyosarcoma

Authors: Tobias M. Dantonello, MD, Helmut Lochbühler, MD, Andreas Schuck, MD, Stefanie Kube, MD, Jan Godzinski, MD, Erik Sköldenberg, MD, Gustaf Ljungman, MD, Daniel Kosztyla, Dipl-Inform, Iris Veit-Friedrich, CDM, Erika Hallmen, CDM, Simone Feuchtgruber, CDM, Ruediger Wessalowski, MD, Markus Franke, MD, Stefan S. Bielack, MD, Thomas Klingebiel, MD, Ewa Koscielniak, MD

Published in: Annals of Surgical Oncology | Issue 11/2014

Abstract

Background

Embryonal rhabdomyosarcoma is the most common pediatric soft tissue sarcoma. The best local treatment in large, nonmetastatic primary unresected nongenitourinary embryonal rhabdomyosarcoma of the abdomen (LARME) is however unclear.

Methods

We analyzed patients with LARME treated in four consecutive CWS trials. All diagnoses were confirmed by reference reviews. Treatment included multiagent chemotherapy and local treatment of the primary tumor with surgery and/or radiotherapy. The impact of primary debulking surgery (PDS) also was studied.

Results

One hundred patients <21 years with a median age of 4 years had LARME. Sixty-one of them had a tumor >10 cm in diameter at diagnosis. PDS was performed in 19 of 100 children. The outcomes of patients with PDS were similar to those of the other patients. In 36 children, the tumor was resected after induction chemotherapy; 60 RME were irradiated. The toxic effects of radiochemotherapy were not significantly increased compared with the nonirradiated patients. With a median follow-up of 10 years, the 5-year EFS and OS were 52 ± 10 and 65 ± 9 %, respectively. Significant risk factors in multivariate analysis were age >10 years; no achievement of complete remission; and inadequate secondary local treatment, defined as incomplete secondary resection or no radiation.

Conclusions

Children with LARME have a fair prognosis, despite an often huge tumor size and unfavorable primary site, if the tumors can either be resected or irradiated following induction chemotherapy. PDS was only performed in a small subgroup. Radiation performed concomitantly with chemotherapy did not increase the acute toxicity significantly.

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Title: Challenges in the Local Treatment of Large Abdominal Embryonal Rhabdomyosarcoma
Authors: Tobias M. Dantonello, MD
Helmut Lochbühler, MD
Andreas Schuck, MD
Stefanie Kube, MD
Jan Godzinski, MD
Erik Sköldenberg, MD
Gustaf Ljungman, MD
Daniel Kosztyla, Dipl-Inform
Iris Veit-Friedrich, CDM
Erika Hallmen, CDM
Simone Feuchtgruber, CDM
Ruediger Wessalowski, MD
Markus Franke, MD
Stefan S. Bielack, MD
Thomas Klingebiel, MD
Ewa Koscielniak, MD
Publication date: 01-10-2014
Publisher: Springer US
Published in: Annals of Surgical Oncology / Issue 11/2014
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI: https://doi.org/10.1245/s10434-014-3551-7

At a glance: The STEP trials

Springer Medicine

Challenges in the Local Treatment of Large Abdominal Embryonal Rhabdomyosarcoma

Abstract

Background

Methods

Results

Conclusions

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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