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Klin Padiatr 2012; 224(06): 348-352
DOI: 10.1055/s-0032-1327607
Original Article
© Georg Thieme Verlag KG Stuttgart · New York

Ewing Sarcoma of the Hand or Foot

A Retrospective Analysis of 80 Patients Treated According to the Clinical Trial EICESS-92 and Euro-E.W.I.N.G.99Ewing Sarkom der Hände oder FüßeEine retrospektive Analyse von 80 Patienten der Studien EICESS-92 und EURO-E.W.I.N.G. 99
D. Froeb
2   FB5, Westfalian Wilhelms University Muenster, Muenster, Germany
,
A. Ranft
3   Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany
,
T. Boelling
4   Centre for Radiotherapy, Radiotherapy, Osnabrueck, Germany
,
M. Paulussen
5   Vestische Kinder- und Jugendklinik Datteln, Witten Herdecke University, Datteln, Germany
,
S. Klco-Brosius
3   Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany
,
H. Jürgens
6   2 EURO-E.W.I.N.G. Trial centre, University Children’s Hospital Muenster, Muenster, Germany
,
U. Dirksen
1   Klinik und Poliklinik für Kinder- und Jugendmedizin, Ped. Hematologie und Onkologie, University Hospital Münster, Münster, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
09 November 2012 (online)

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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
 

  • References

  • 1 Adkins CD, Kitaoka HB, Seidl RK et al. Ewing’s Sarcoma of the Foot. Clin Orthop Rel Res 1997; 343: 173-182
    PubMedGoogle Scholar
  • 2 Anakwenze OA, Parker WL, Wold LE et al. Ewing’s sarcoma of the hand. J of Hand Surg Eur 2009; 34: 35-39
    PubMedGoogle Scholar
  • 3 Applebaum MA, Worch J, Matthay KK et al. Clinical features and outcomes in patients with extraskeletal ewing sarcoma. Cancer 2011; 117: 3027-3032
    CrossrefPubMedGoogle Scholar
  • 4 Arai Y, Kun LE, Brooks MT et al. Ewing’s sarcoma: Local tumor control and patterns of failure following limited-volume radiation therapy. Int J Radiat Oncol Biol Phys 1991; 21: 1501-1508
    CrossrefPubMedGoogle Scholar
  • 5 Bacci G, Toni A, Avella M et al. Long-term results in 144 localized Ewing’s sarcoma patients treated with combined therapy. Cancer 1989; 63: 1477-1486
    CrossrefPubMedGoogle Scholar
  • 6 Bacci G, Ferrari S, Mercuri M et al. Multimodal Therapy for the Treatment of Nonmetastatic Ewing Sarcoma of Pelvis. J Pediatr Hematol Oncol 2003; 25: 118-124
    CrossrefPubMedGoogle Scholar
  • 7 Baraga JJ, Amrami KK, Swee RG et al. Radiographic features of Ewing’s sarcoma of the bones of the hands and feet. Skeletal Radiol 2001; 30: 121-126
    CrossrefPubMedGoogle Scholar
  • 8 Baumhoer D. Tumours of the hand: a review on histology of bone malignancies. J of Hand Surg Eur 2010; 35: 354-361
    PubMedGoogle Scholar
  • 9 Bölling T, Schuck A, Pape H et al. Register for the evaluation of side effects after radiation in childhood and adolescence – first results. Klin Pädiatr 2007; 219: 139-145
    Article in Thieme ConnectPubMedGoogle Scholar
  • 10 Burgert Jr EO, Nesbit ME, Garnsey LA et al. Multimodal therapy for the management of non pelvic, localized Ewing’s sarcoma of bone: Intergroup study IESS-II. J Clin Oncol 1990; 8: 1514-1524
    PubMedGoogle Scholar
  • 11 Biswas G, Parashar A, Ghosh S et al. Extra-osseous Ewing’s sarcoma of the thumb. J Hand Surg Eur 2008; 33: 667-669
    PubMedGoogle Scholar
  • 12 Casadei R, Magnani M, Biagini R et al. Prognostic factors in Ewing’s sarcoma of the foot. Clin Orthop Relat Res 2004; 420: 230-238
    CrossrefPubMedGoogle Scholar
  • 13 Cotterill SJ, Ahrends S, Paulussen M et al. Prognostic factors in Ewing tumor of bone: Analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study Group. J Clin Oncol 2000; 8: 3108-3114
    PubMedGoogle Scholar
  • 14 Daecke W, Ahrens S, Juergens H et al. Ewing’s sarcoma and primitive neuroectodermal tumor of hand and forearm. Experience of the Cooperative Ewing’s Sarcoma Study Group. J Cancer Res Clin Oncol 2005; 131: 219-225
    CrossrefPubMedGoogle Scholar
  • 15 DeLaney TF, Trofimov AV, Engelsman M et al. Advanced-technology radiation therapy in the management of bone and soft tissue sarcomas. Cancer Control 2005; 12: 27-35
    PubMedGoogle Scholar
  • 16 Dirksen U, Jürgens H. Approaching Ewing sarcoma. Future Oncol 2010; 6: 1155-1162 Review
    CrossrefPubMedGoogle Scholar
  • 17 Escobedo EM, Bjorkengren AG, Moore SG. Ewing’s sarcoma of the hand. AJR Am J Roentgenol 1992; 159: 101-102
    CrossrefPubMedGoogle Scholar
  • 18 Elomaa I, Blomqvist CP, Saeter G et al. Five-year results in Ewing’s sarcoma. The Scandinavian Sarcoma Group experience with the SSG IX protocol. Eur J Cancer 2000; 36: 875-880
    CrossrefPubMedGoogle Scholar
  • 19 Ewing J. Diffuse endothelioma of bone. Proc N Y Pathol Soc 1921; 21: 17-24
    PubMedGoogle Scholar
  • 20 Exner GU, Jacob HA, Middendorp J. Reconstruction of the first and second metatarsals with free microvascular fibular bone graft after resection of a Ewing sarcoma. J Pediatr Orthop B 1998; 7: 239-242
    CrossrefPubMedGoogle Scholar
  • 21 Fröhlich B, Ahrens S, Burdach S et al. High-dosage chemotherapy in primary metastasized and relapsed Ewing’s sarcoma. (EI)CESS. Klin Padiatr 1999; 211: 284-290
    Article in Thieme ConnectPubMedGoogle Scholar
  • 22 Fulda S. Cell Death and Survival Signaling in Oncogenesis. Klin Pädiatr 2010; 222: 340-344
    Article in Thieme ConnectPubMedGoogle Scholar
  • 23 Goodwin MI. A case of metatarsal Ewing’s sarcoma. Acta Orthop Scand 1990; 61: 187-188
    PubMedGoogle Scholar
  • 24 Haeusler J, Ranft A, Boelling T et al. The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES). Cancer 2010; 116: 443-450
    CrossrefPubMedGoogle Scholar
  • 25 Hense HW. Factors associated with tumor volume and primary metastases in Ewing tumors result from the (EI(CESS) ) studies. Ann Oncol 1999; 1073-1077
    PubMedGoogle Scholar
  • 26 Johnstone PA, Wexler LH, Venzon DJ et al. Sarcomas of the hand and foot: analysis of local control and functional result with combined modality therapy in extremity preservation. Int J Radiat Oncol Biol 1994; 29: 735-745
    PubMedGoogle Scholar
  • 27 Juergens C, Weston C, Lewis I et al. Safety assessment of intensive induction with vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) in the treatment of Ewing tumors in the EURO-E.W.I.N.G. 99 clinical trial. Pediatr Blood Cancer 2006; 47: 22-29
    CrossrefPubMedGoogle Scholar
  • 28 Kovar H, Alonso J, Aman P et al. The first European interdisciplinary Ewing sarcoma research summit. Front Oncol 2012; 2: 54
    PubMedGoogle Scholar
  • 29 Ladenstein R, Pötschger U, Le Deley MC et al. Primary disseminated multifocal Ewing sarcoma: results of the Euro-EWING 99 trial. J Clin Oncol 2010; 28: 3284-3291
    CrossrefPubMedGoogle Scholar
  • 30 Leeson MC, Smith MJ. Ewing’s sarcoma of the foot. Foot Ankle 1989; 10: 147-151
    CrossrefPubMedGoogle Scholar
  • 31 Mathieu G, Mascard E, Wicart P et al. Forefoot reconstruction after lesser intermediate metatarsal bone resection for aggressive or malignant tumors in children: report of three cases. Foot Ankle Int 2007; 28: 1011-1016
    CrossrefPubMedGoogle Scholar
  • 32 Nesbit Jr ME, Gehan EA, Burgert Jr EO et al. Multimodal therapy for the management of primary, nonmetastetic Ewing’s sarcoma of bone: a long-term follow-up of the first intergroup study. J Clin Oncol 1990; 8: 1664-1674
    PubMedGoogle Scholar
  • 33 Oberlin O, Ledeley MC, Ngyenbu B et al. Prognostic factors in localized Ewing’s tumours and peripheral neuroectodermal tumours: the third study of the French Society of Paediatric Oncology (EW88 study). British Journal of Cancer 2001; 85: 1646-1654
    CrossrefPubMedGoogle Scholar
  • 34 Ozaki T, Hashizume H, Kawai A et al. Ewing’s sarcoma of the hand: magnetic resonance images and treatment. J Hand Surg Am 1995; 20: 441-444
    CrossrefPubMedGoogle Scholar
  • 35 Paulussen M, Ahrens S, Braun-Munzinger G et al. EICESS 92 (European Intergroup Cooperative Ewing’s Sarcoma Study) – preliminary results. Klin Padiatr 1999; 211: 276-283
    Article in Thieme ConnectPubMedGoogle Scholar
  • 36 Paulussen M, Craft AW, Lewis I et al. Results of the EICESS-92 Study: Two Randomized Trials of Ewing’s Sarcoma Treatment – Cyclophosphamide Compared With Ifosfamide in Standard-Risk Patients and Assessment of Benefit of Etoposide Added to Standard Treatment in High-Risk Patients. J Clin Oncol 2008; 26: 4385-4393
    CrossrefPubMedGoogle Scholar
  • 37 Pieper S, Ranft A, Braun-Munziger G et al. Ewing tumors over the age of forty: A retrospective analysis of 47 patients treated according to the international clinical trial EICESS 92 and EURO-E.W.I.N.G. 99. Onkologie 2008; 31: 657-663
    PubMedGoogle Scholar
  • 38 Potratz J, Dirksen U, Jürgens H et al. Ewing Sarcoma: Clinical State of the art. Pediatr Hematol Oncol 2012; 29: 1-11 Review
    CrossrefPubMedGoogle Scholar
  • 39 Pritchard DJ, Dahlin DC, Dauphine RT et al. Ewing’s Sarcoma: A clinicopathological and statistical analysis of patients surviving five years or longer. J Bone Joint Surg 57A 1975; 10-16
    PubMedGoogle Scholar
  • 40 Schuck A, Ahrens S, Paulussen M et al. Local therapy in localized Ewing tumors: results of 1 058 patients treated in the CESS 81, CESS 86, and EICESS 92 trials. Int J Radiat Oncol Biol Phys 2003; 55: 168-177
    CrossrefPubMedGoogle Scholar