Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS).
Adolescent
Antineoplastic Combined Chemotherapy Protocols
/ therapeutic use
Biliary Tract
/ pathology
Biliary Tract Neoplasms
/ drug therapy
Biopsy
Child
Child, Preschool
Combined Modality Therapy
Disease-Free Survival
Female
Follow-Up Studies
Humans
Infant
Kaplan-Meier Estimate
Male
Postoperative Complications
Recurrence
Retrospective Studies
Rhabdomyosarcoma
/ drug therapy
Biliary tree
CWS Studiengruppe
Outcome
Rhabdomyosarcoma
Treatment
Journal
BMC cancer
ISSN: 1471-2407
Titre abrégé: BMC Cancer
Pays: England
ID NLM: 100967800
Informations de publication
Date de publication:
14 Oct 2019
14 Oct 2019
Historique:
received:
11
02
2019
accepted:
20
09
2019
entrez:
16
10
2019
pubmed:
16
10
2019
medline:
27
2
2020
Statut:
epublish
Résumé
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. 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). 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. 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.
Sections du résumé
BACKGROUND
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
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
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
CONCLUSIONS
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.
Identifiants
pubmed: 31610788
doi: 10.1186/s12885-019-6172-5
pii: 10.1186/s12885-019-6172-5
pmc: PMC6791000
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
945Références
J Pediatr Surg. 1982 Oct;17(5):508-11
pubmed: 7175637
Cancer. 1985 Aug 1;56(3):575-81
pubmed: 4005814
J Clin Oncol. 1999 Dec;17(12):3706-19
pubmed: 10577842
Eur J Cancer. 2013 Jan;49(1):185-93
pubmed: 22901831
J Pediatr Surg. 1971 Aug;6(4):474-9
pubmed: 5563889
Pediatr Transplant. 2014 Aug;18(5):E146-51
pubmed: 24815678
J Clin Oncol. 2016 May 20;34(15):1772-9
pubmed: 27022116
J Pediatr Surg. 2000 Feb;35(2):309-16
pubmed: 10693686
Ann Surg. 2004 Aug;240(2):205-13
pubmed: 15273542
Pediatr Radiol. 1998 Feb;28(2):101-8
pubmed: 9472056
Pediatr Blood Cancer. 2008 Apr;50(4):739-45
pubmed: 18286501
Int J Radiat Oncol Biol Phys. 2010 May 1;77(1):210-6
pubmed: 19695791
Cancer. 1992 Nov 15;70(10):2557-67
pubmed: 1482503
Wien Klin Wochenschr. 2005 Mar;117(5-6):196-209
pubmed: 15875759
World J Gastroenterol. 2008 Aug 14;14(30):4823-5
pubmed: 18720547