Affiliation:
1. Pediatric Oncology, Hospital of the Goethe University Frankfurt Centre
for Paediatrics and Adolescent Medicine, Frankfurt am Main,
Germany
2. Department of Pathology, Section Pediatric Pathology, University Bonn,
Germany
Abstract
Abstract
BackgroundThe outcome of children with refractory or relapsed soft tissue
sarcoma (STS) is extremely poor. Whereas larger clinical trials evaluated
specific treatment modalities, real-life data on individual multimodal
therapeutic strategies, given alone or in combination, are scarce.
Patients and Methods We retrospectively analyzed the clinical course of 18
pediatric patients with progression of or relapsed STS treated between 2008 and
2018 in our institution.
Results A total of 18 patients (median age 12.4 years) suffered from
progression or relapse of alveolar (n=7), embryonal (n=5),
undifferentiated (n=2) rhabdomyosarcoma or desmoplastic small round cell
tumor (n=4). 14 patents had an initial stage IV disease. All but one
patient died. Median survival was 12.5 months. Shortest survival was seen in
patients with systemic progression of the disease, longest in patients with
local relapse. Patients with an Oberlin score<2 at the time of relapse
had a significant longer time of survival than those with a score≥2. No
significant advantage of a specific therapeutic modality was observed.
Discussion We critically analyzed the clinical course in the real-life
setting, in which various treatment options were applied to an individual
patient according to the best of available data. We observed that some patients
died within a short period of time despite multiple treatment modalities, which
underlines the need for better prognostic parameters.
Conclusion In addition to well characterized clinical factors such as
local or systemic relapse, the Oberlin score could be helpful in counselling
patients and their families for choosing the best strategy of care.
Subject
Pediatrics, Perinatology and Child Health