TY  - JOUR
A1  - Urla, Cristian
A1  - Warmann, Steven
A1  - Sparber-Sauer, Monika
A1  - Schuck, Andreas
A1  - Leuschner, Ivo
A1  - Klingebiel, Thomas
A1  - Blumenstock, Gunnar
A1  - Seitz, Guido
A1  - Koscielniak, Ewa
A1  - Fuchs, Jörg
T1  - Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS)
T2  - BMC cancer
N2  - Background: Biliary rhabdomyosarcoma (RMS) is the most common biliary tumor in children. The management of affected patients contains unique challenges because of the rarity of this tumor entity and its critical location at the porta hepatis, which can make achievement of a radical resection very difficult.
Methods: In a retrospective chart analysis we analysed children suffering from biliary RMS who were registered in three different CWS trials (CWS-96, CWS-2002P, and SoTiSaR registry).
Results: Seventeen patients (12 female, 5 male) with a median age of 4.3 years were assessed. The median follow-up was 42.2 months (10.7–202.5). The 5-year overall (OS) and event free survival (EFS) rates were 58% (45–71) and 47% (34–50), respectively. Patients > 10 years of age and those with alveolar histology had the worst prognosis (OS 0%). Patients with botryoid histology had an excellent survival (OS 100%) compared to those with non-botryoid histology (OS 38%, 22–54, p = 0.047). Microscopic complete tumor resection was achieved in almost all patients who received initial tumor biopsy followed by chemotherapy and delayed surgery.
Conclusion: Positive predictive factors for survival of children with biliary RMS are age ≤ 10 years and botryoid tumor histology. Primary surgery with intention of tumor resection should be avoided.
KW  - Rhabdomyosarcoma
KW  - Biliary tree
KW  - CWS Studiengruppe
KW  - Treatment
KW  - Outcome
Y1  - 2019
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/52999
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-529993
SN  - 1471-2407
N1  - Open Access: This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
IS  - 1, Art. 945
SP  - 1
EP  - 8
PB  - BioMed Central ; Springer
CY  - London ; Berlin ; Heidelberg
ER  -