In:
Klinische Pädiatrie, Georg Thieme Verlag KG
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.
Type of Medium:
Online Resource
ISSN:
0300-8630
,
1439-3824
Language:
English
Publisher:
Georg Thieme Verlag KG
Publication Date:
2023
detail.hit.zdb_id:
2039110-9
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