uu.seUppsala University Publications
Change search
ReferencesLink to record
Permanent link

Direct link
Challenges in the Local Treatment of Large Abdominal Embryonal Rhabdomyosarcoma
Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany.
Department of Pediatric Surgery, Olgahospital, Klinikum Stuttgart, Germany.
Department of Radiotherapy, University of Muenster, Münster, Germany.
Pediatrics 5, Olgahospital, Klinikum Stuttgart, Germany.
Show others and affiliations
2014 (English)In: Annals of Surgical Oncology, ISSN 1068-9265, E-ISSN 1534-4681, Vol. 21, no 11, 3579-3586 p.Article in journal (Refereed) Published
Abstract [en]


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.


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.


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.


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.

Place, publisher, year, edition, pages
2014. Vol. 21, no 11, 3579-3586 p.
National Category
Cancer and Oncology Surgery
URN: urn:nbn:se:uu:diva-229164DOI: 10.1245/s10434-014-3551-7ISI: 000342144900036PubMedID: 25047462OAI: oai:DiVA.org:uu-229164DiVA: diva2:735940
Available from: 2014-08-04 Created: 2014-08-04 Last updated: 2014-10-29Bibliographically approved

Open Access in DiVA

No full text

Other links

Publisher's full textPubMed

Search in DiVA

By author/editor
Sköldenberg, ErikLjungman, Gustaf
By organisation
Paediatric SurgeryPediatrics
In the same journal
Annals of Surgical Oncology
Cancer and OncologySurgery

Search outside of DiVA

GoogleGoogle Scholar
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

Altmetric score

Total: 334 hits
ReferencesLink to record
Permanent link

Direct link