Progression and Relapse of Pediatric Soft Tissue Sarcoma: Individualized Approach of Treatment – Experience from a Major Pediatric Cancer Center in Europe

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.

Zusammenfassung

Hintergrund Die Prognose von Kindern mit refraktärem oder rezidiviertem Weichteilsarkom (STS) ist extrem schlecht. Während in prospektiven Studien der Benefit von spezifischen Behandlungsmodalitäten untersucht wurde, finden sich in der Literatur kaum Daten zu individuellen Therapiestrategien, die allein oder in Kombination verabreicht wurden.

Patienten und Methoden Wir analysierten retrospektiv den Verlauf aller pädiatrischen Patienten mit Progress oder Rezidiv eines STS, die zwischen 2008 und 2018 in unserer Klinik behandelt wurden.

Ergebnisse Insgesamt betreuten wir 18 Patienten (medianes Alter 12,4 Jahre) mit einem Progress/Rezidiv eines alveolären (n=7), embryonalen (n=5), oder undifferenzierten (n=2) Rhabdomyosarkoms oder eines desmoplastischen kleinen Rundzelltumors (n=4). 14 Patienten hatten initial ein Stadium IV. Bis auf einen Patienten verstarben alle Kinder, wobei die mediane Überlebenszeit 12,5 Monate betrug. Die kürzeste Überlebenszeit hatten Kinder mit systemischem Progress, die längste Kinder mit Lokalrezidiv. Patienten mit einem Oberlin-Score<2 zum Zeitpunkt des Rezidivs hatten eine signifikant längere Überlebenszeit als Patienten mit einem Score≥2. Kein bestimmtes therapeutisches Vorgehen zeigte einen signifikanten Vorteil.

Diskussion Die kritische Analyse im “real-life setting” zeigt, dass einige Patienten trotz intensivster kombinierter Therapiemethoden innerhalb kurzer Zeit verstarben, was die Notwendigkeit besserer prognostischer Parameter unterstreicht.

Schlussfolgerung Neben bereits gut charakterisierten klinischen Faktoren könnte der Oberlin-Score bei der Beratung von Patienten hinsichtlich der Wahl des besten therapeutischen Vorgehens hilfreich sein.

Key words

soft tissue sarcoma - pediatric - relapse - progression - child - outcome

Schlüsselwörter

Weichteilsarkom - Kinder - Rezidiv - Progress - Prognose

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