Ewing Sarcoma of the Hand or Foot

Abstract

Purpose:

Ewing Sarcoma (ES) of the hand or foot is a rare clinical condition. Due to the critical site, it is of major importance to choose an optimal procedure for local control in terms of outcome and function. Local therapeutic options for these patients range from: surgery (OP), surgery followed by radiotherapy (OP & RT), or radiotherapy (RT) alone.

Patients and Methods:

Data from 80 patients with ES of the hand or foot were analyzed. All patients received chemotherapy according to the protocols of the Cooperative Ewing Sarcoma Study Group (CESS) from 1991 to 2009 (EICESS-92 and EURO-E.W.I.N.G.99). Local therapy consisted of: OP in 39%, OP & RT in 44%, and RT in 12%. In 5% of the patients no local therapy (noL) was performed. Primary endpoint of our study was the event-free-survival (EFS).

Results:

The 3-year overall EFS was 62% (95%CI 0.50–0.72). Patients with localized disease had a significantly better outcome with an EFS of 77% (95%CI 0.63–0.86), compared to patients with primary disseminated disease with an EFS of 30% (95%CI 0.14–0.49; p<0.001). In comparing local treatment modalities, no significant difference was observed. The 3-year EFS for OP was 61% (95% CI 0.40–0.76), for OP & RT 66% (95%CI 0.47–0.79) and for RT only 70% (95%CI 0.32–0.89) (p=0.253). Patients who did not receive local treatment had an unfavourable prognosis (3-year EFS=0.25; 95%CI 0.01–0.67; p=0.024). A multivariate analysis which included local treatment modality and known prognosticators, showed that primary dissemination was the only significant prognostic factor.

Conclusion:

Ewing sarcoma of the hand or foot is associated with a favourable outcome. Our data analysed a limited group of patients and thus did not provide a clear indication for a preferred local treatment modality.

Zusammenfassung

Hintergrund:

Ewing Sarkome (ES) treten selten an Hand oder Fuß auf. Aufgrund des Tumorsitzes ist für die Patienten die Wahl der lokaltherapeutischen Modalität von besonderer Bedeutung. Die lokaltherapeutischen Optionen bes­tehen in Operation (OP), Operation und Bestrahlung (OP&RT) oder alleiniger Bestrahlung (RT).

Patienten und Methoden:

In einer retrospektiven Studie analysierten wir von 80 Patienten mit ES an Hand oder Fuß. Alle Patienten erhielten eine Behandlung im Rahmen der Protokolle der kooperativen Ewing Sarkom Studien Gruppe (CESS), EICESS-92 und EURO E.W.I.N.G.-99. Die Lokaltherapie war in 39% die OP, in 44% OP & RT und in 12% die RT. In 5% wurde keine Lokaltherapie (noL) durchgeführt. Endpunkt der Analyse war das Ereignis-freie Überleben (EFS).

Ergebnisse:

Das EFS nach 3 Jahren lag bei 62% (95%CI 0,50–0,72). Patienten mit lokalisierter Erkrankung hatten mit 77% (95%CI 0,63–0,86) 3yEFS eine bessere Prognose als Patienten mit primär disseminierter Erkrankung mit einem 3yEFS von 30% (95%CI 0,14–0,49; p<0,001). Nach der jeweiligen lokaltherapeutischen Moda­lität lag das 3yEFS bei 61% (95% CI 0,40–0,76) nach Operation, bei 66% (95%CI 0,47–0,79) nach Operation in Kombination mit Strahlentherapie und bei 70% (95%CI 0,32–0,89) nach Strahlentherapie. Patienten die keine Lokaltherapie erhielten, hatten ein 3yEFS von 25% (95%CI 0,01–0,67; p=0,024). Eine multivariate Analyse unter Einschluss der lokaltherapeutischen Modalitäten und bekannter Risikofaktoren zeigt als alleinig signifikanten prognostischen Faktor den primären Metastasierungsstatus.

Schlussfolgerungen:

Das Ewing Sarkom an Hand oder Fuß hat eine gute Prognose.

Key words

Ewing Sarcoma - hand - foot - local therapy - prognosis

Schlüsselwörter

Ewing Sarkom - Hand - Fuß - Lokaltherapie - Prognose

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