Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS)
Abstract 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...
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| Language: | English |
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BMC
2019-10-01
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| Series: | BMC Cancer |
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| Online Access: | http://link.springer.com/article/10.1186/s12885-019-6172-5 |
| _version_ | 1828889761824112640 |
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| author | Cristian Urla Steven W. Warmann Monika Sparber-Sauer Andreas Schuck Ivo Leuschner Thomas Klingebiel Gunnar Blumenstock Guido Seitz Ewa Koscielniak Jörg Fuchs |
| author_facet | Cristian Urla Steven W. Warmann Monika Sparber-Sauer Andreas Schuck Ivo Leuschner Thomas Klingebiel Gunnar Blumenstock Guido Seitz Ewa Koscielniak Jörg Fuchs |
| author_sort | Cristian Urla |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-13T12:47:11Z |
| format | Article |
| id | doaj.art-7700dde475834d419e11429c97baac66 |
| institution | Directory Open Access Journal |
| issn | 1471-2407 |
| language | English |
| last_indexed | 2024-12-13T12:47:11Z |
| publishDate | 2019-10-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cancer |
| spelling | doaj.art-7700dde475834d419e11429c97baac662022-12-21T23:45:28ZengBMCBMC Cancer1471-24072019-10-011911810.1186/s12885-019-6172-5Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS)Cristian Urla0Steven W. Warmann1Monika Sparber-Sauer2Andreas Schuck3Ivo Leuschner4Thomas Klingebiel5Gunnar Blumenstock6Guido Seitz7Ewa Koscielniak8Jörg Fuchs9Department of Pediatric Surgery and Pediatric Urology, University Children’s Hospital TuebingenDepartment of Pediatric Surgery and Pediatric Urology, University Children’s Hospital TuebingenKlinikum Stuttgart, Zentrum für Kinder-, Jugend- und Frauenmedizin, Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology and Immunology)Klinikum Ingolstadt, Prostatakarzinom ZentrumDepartment of Pediatric Pathology, University Hospital Schleswig-HolsteinDepartment of Pediatric Hematology and Oncology, University Hospital FrankfurtDepartment of Clinical Epidemiology and Applied Biometry, University Hospital of TuebingenDepartment of Pediatric Surgery, University Children’s Hospital, BaldingerstrKlinikum Stuttgart, Zentrum für Kinder-, Jugend- und Frauenmedizin, Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology and Immunology)Department of Pediatric Surgery and Pediatric Urology, University Children’s Hospital TuebingenAbstract 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.http://link.springer.com/article/10.1186/s12885-019-6172-5RhabdomyosarcomaBiliary treeCWS StudiengruppeTreatmentOutcome |
| spellingShingle | Cristian Urla Steven W. Warmann Monika Sparber-Sauer Andreas Schuck Ivo Leuschner Thomas Klingebiel Gunnar Blumenstock Guido Seitz Ewa Koscielniak Jörg Fuchs Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS) BMC Cancer Rhabdomyosarcoma Biliary tree CWS Studiengruppe Treatment Outcome |
| title | Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS) |
| title_full | Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS) |
| title_fullStr | Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS) |
| title_full_unstemmed | Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS) |
| title_short | Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS) |
| title_sort | treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree experience of the cooperative weichteilsarkom studiengruppe cws |
| topic | Rhabdomyosarcoma Biliary tree CWS Studiengruppe Treatment Outcome |
| url | http://link.springer.com/article/10.1186/s12885-019-6172-5 |
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