Medizinische Universität Graz - Research portal
Selected Publication:
SHR Neuro Cancer Cardio Lipid Metab Microb
Benesch, M; Leuschner, I; Wardelmann, E; Thielen, M; Schmid, I; Kontny, U; Ebetsberger, G; Frey, E; Graf, N; Schneider, DT; Kremens, B; Amann, G; Urban, C; Schlemmer, M; Quehenberger, F; Klingebiel, T; Dantonello, T; Koscielniak, E.
Gastrointestinal stromal tumours in children and young adults: A clinicopathologic series with long-term follow-up from the database of the Cooperative Weichteilsarkom Studiengruppe (CWS).
Eur J Cancer. 2011; 47(11):1692-1698
Doi: 10.1016/j.ejca.2011.03.014
Web of Science
PubMed
FullText
FullText_MUG
Google Scholar
- Leading authors Med Uni Graz
- Benesch Martin
- Co-authors Med Uni Graz
- Quehenberger Franz
- Urban Ernst-Christian
- Altmetrics:
- Dimensions Citations:
- Plum Analytics:
- Scite (citation analytics):
- Abstract:
- Background: Studies on gastrointestinal stromal tumours (GIST) in the paediatric population are limited to case reports or small case series. Patients and methods: We conducted a retrospective study to describe the long-term outcome of children and adolescents with GIST registered in the database of the Cooperative Weichteilsarkom Studiengruppe (CWS). Results: Sixteen patients (female, n = 11) were identified. Median age at diagnosis was 13.5 years. In four female patients presence of thoracic masses in addition to GIST led to the diagnosis of complete or incomplete Carney triad. Three female patients had metastatic disease at diagnosis, the remaining thirteen GIST were localised. The stomach was the most common primary site of the tumour, followed by the small bowel and colon/abdomen. All patients underwent tumour resection. Receptor tyrosine kinase inhibitors (RTKI) were administered in five patients. With a median follow-up of 96 months all patients are alive, nine of them in first CR. Four female patients developed local or distant recurrence; three of them achieved second CR and one a PR. Two individuals have extensive progressive (n = 1) or stable (n = 1) disease. Estimated progression-free survival at 5 years is 0.63 (95%CI: 0.50-0.86). Conclusions: Although long-term overall survival is favourable, approximately 30 percent of patients develop disease progression. International cooperation in registration, tissue collection and molecular studies are required to obtain reliable data on the clinical course of these rare tumours in the paediatric population. Biological studies are a prerequisite for initiation of studies with RTKI. (C) 2011 Elsevier Ltd. All rights reserved.
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent -
- Child -
- Child, Preschool -
- Databases, Factual -
- Disease Progression -
- Female -
- Follow-Up Studies -
- Gastrointestinal Stromal Tumors - diagnosis
- Humans -
- Infant -
- Male -
- Neoplasm Metastasis -
- Recurrence -
- Registries -
- Retrospective Studies -
- Treatment Outcome -
- Find related publications in this database (Keywords)
- Gastrointestinal stromal tumours
- Children
- Adolescents
- Carney triad