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Weichteilsarkome im Kindesalter

Diagnostik und Therapie

Soft tissue sarcomas of childhood

Diagnosis and therapy

  • Leitthema
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Zusammenfassung

Bei Weichteilsarkomen (WTS) handelt es sich um eine seltene, in ihrer Klinik und Biologie sehr heterogene Gruppe maligner Tumoren. Das häufigste bei Kindern auftretende WTS ist das Rhabdomyosarkom (RMS). Die multizentrischen Therapieoptimierungsstudien, hier auch die Cooperative Weichteilsarkom Studie CWS der GPOH, haben entscheidend zur Verbesserung der Prognose von Kindern mit WTS beigetragen. Die Reihenfolge wie auch die Durchführung der einzelnen Therapiemodalitäten (Chirurgie, Chemotherapie, Radiotherapie) hängt von der Tumorgröße, Histologie, Lokalisation und dem Alter des Patienten ab. Die rezidivfreie Überlebensrate nach 5 Jahren für alle Patienten mit „RMS-artigen“ Tumoren (RMS, EES, PNET, SySA) beträgt 50–80% (RMS 70%, EES/PNET 50%, SySa 70–80%).

Abstract

Soft tissue sarcomas (STS) account for approximately 7% of malignant neoplasms in children. The heterogeneity of STS makes diagnosis and therapy particularly difficult and should be reserved for specialised centers with expertise in treating oncological disease in children. As a result of large multi-center STS studies, such as the North-American Intergroup Rhabdomyosarcoma Study, the German CWS, Italian GCI and SIOP MMT the identification of more effective treatment strategies and an improvement in prognosis have been made in the last 30 years. Prognostic variables like tumor size, site, histology, und age were identified and criteria were established for the use of preoperative chemotherapy and radiotherapy, primary and delayed second-look surgery. When treated with a combination of chemotherapy and local therapy, STS showed an event free survival of between 50–80% (RMS 70%, EES/PNET circa 50%, synovial sarcomas 70–80%).

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Literatur

  1. Ackermann B, Tröger A, Glouchkova L, Körholz D, Göbel U, Dilloo D (2004) Characterization of CD34+ progenitor-derived dendritic cells pulsed with tumor cell lysate for a vaccination strategy in children with malignant solid tumors and a poor prognosis. Klin Pädiatr 216:176–182

    Google Scholar 

  2. Alava ED (2000) Molecular biology of the Ewing‚s Sarcoma/primitive neuroectodermal tumor family. J Clin Oncol 18:204–213

    PubMed  Google Scholar 

  3. Barr F (1997) Molecular genetics and pathogenesis of rahbdomyosarcoma. J Pediatr Hematol/Oncol 19:483–491

    Google Scholar 

  4. Bisogno G, Ferrari A, Bergeron C (2005) The IVADo regimen-A pilot study with ifosfamide, vincristine, actinomycin D, and doxorubicin in children with metastatic soft tissue sarcoma. Cancer 103:1719–1724

    Article  PubMed  Google Scholar 

  5. Bramwell V, Rouesse J, Steward W (1994) Adjuvant CYVADIC chemotherapy for adult soft tissue sarcoma--reduced local recurrence but no improvement in survival: a study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. J Clin Oncol 12:1137–1149

    PubMed  Google Scholar 

  6. Breneman JC, Lyden E, Pappo AS (2003) Prognostic factors and clinical outcomes in children and adolescents with metastatic rhabdomyosarcoma--a report from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol 21:78–84

    Article  PubMed  Google Scholar 

  7. Carli M, Colombatti R, Oberlin O (1999) High-dose melphalan with autologous stem-cell rescue in metastatic rhabdomyosarcoma. J Clin Oncol 17:2796–2803

    PubMed  Google Scholar 

  8. Carli M, Passone E, Perilongo G, Bisogno G (2003) Ifosfamide in pediatric solid tumors. Oncology 65:99–104

    Article  PubMed  Google Scholar 

  9. Carli M, Sotti G, Alaggio R, Stevens MCG (2004) Soft Tissue Sarcomas. In: Pinkerton R PP, Pieters R, ed. Pediatric Oncology. London: Arnold

  10. Carli M, Colombatti R, Oberlin O (2004) European intergroup studies (MMT4–89 and MMT4–91) on childhood metastatic rhabdomyosarcoma: final results and analysis of prognostic factors. J Clin Oncol 22:4787–4794

    Article  PubMed  Google Scholar 

  11. Coindre JM, Nguyen BB, Bonichon F, de Mascarel I, Trojani M (1988) Histopathologic grading in spindle cell soft tissue sarcomas. Cancer 61:2305–2309

    PubMed  Google Scholar 

  12. Crist WM, Anderson JR, Meza JL (2001) Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J Clin Oncol 19:3091–3102

    PubMed  Google Scholar 

  13. Crist W, Gehan EA, Ragab AH (1995) The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 13:610–630

    PubMed  Google Scholar 

  14. Joensuu H, Roberts PJ, Sarlomo-Rikala M (2001) Effect of the tyrosine kinase inhibitor STI571 in a patient with a metastatic gastrointestinal stromal tumor. N Engl J Med 344:1052–1056

    PubMed  Google Scholar 

  15. Kampe CE, Rosen G, Eilber F (1994) Synovial sarcoma. A study of intensive chemotherapy in 14 patients with localized disease. Cancer 72:2161–2169

    Google Scholar 

  16. Kawaguchi S, Wada T, Ida K (2005) Phase I vaccination trial of SYT-SSX junction peptide in patients with disseminated synovial sarcoma. J Transl Med 3:1

    Article  PubMed  Google Scholar 

  17. Kawai A, Woodruff J, Healey JH, Brennan MF, Antonescu CR, Ladanyi M (1998) SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med 338:153–160

    Article  PubMed  Google Scholar 

  18. Koscielniak E, Dilloo D (2002) Definition of the high risk patients: the EBMT-CWS experience and CWS proposal for a therapeutic strategy for high risk sarcoma patients. Bone Marrow Transplant 30:33–34

    Google Scholar 

  19. Koscielniak E, Gross-Wieltsch U, Treuner J (2005) Graft-versus-Ewing sarcoma effect and long-term remission induced by haploidentical stem-cell transplantation in a patient with relapse of metastatic disease. J Clin Oncol 23:242–24419

    Article  PubMed  Google Scholar 

  20. Koscielniak E, Harms D, Henze G (1999) Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86. J Clin Oncol 17:3706–3719

    PubMed  Google Scholar 

  21. Koscielniak E, Klingebiel TH, Peters C (1997) Do patients with metastatic and recurrent rhabdomyosarcoma benefit from high-dose therapy with hematopoietic rescue? Report of the German/Austrian Pediatric Bone Marrow Transplantation Group. Bone Marrow Transplant 19:227–231

    Article  PubMed  Google Scholar 

  22. Koscielniak E, Rodary C, Flamant F (1992) Metastatic rhabdomyosarcoma and histologically similar tumors in childhood: a retrospective European multi-center analysis. Med Pediatr Oncol 20:209–214

    PubMed  Google Scholar 

  23. Ladenstein R, Hartmann O, Koscielniak E, Philip T (2004) Megatherapy with stem cell rescue in solid tumors. In: Pinkerton R PP, Pieters R, ed. Paediatric Onclogy. London: Arnold; 539–569

  24. Ladenstein R, Treuner J, Koscielniak E (1993) Synovial sarcoma of childhood and adolescence. Report of the German CWS-81 study. Cancer 71:3647–3655

    PubMed  Google Scholar 

  25. Misawa A HH, Tsuchiya K, Iehara T, Sawada T, Sugimoto T (2003) Regression of refractory rhabdomyosarcoma after allogeneic stem-cell transplantation. Pediatr Hematol Oncol 151–15525

  26. Newton WA Jr GE, Webber BL et al. (1995) Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects—an Intergroup Rhabdomysoarcoma Study. Cancer 76:1073–1085

    PubMed  Google Scholar 

  27. Okcu MF, Despa S, Choroszy M (2001) Synovial sarcoma in children and adolescents: thirty three years of experience with multimodal therapy. Med Pediatr Oncol 37:90–96

    Article  PubMed  Google Scholar 

  28. Raney RB (2005) Synovial sarcoma in young people: background, prognostic factors, and therapeutic questions. J Pediatr Hematol Oncol 27:207–211

    Article  PubMed  Google Scholar 

  29. Rosen G, Forscher C, Lowenbraun S (1994) Synovial sarcoma. Uniform response of metastases to high dose ifosfamide. Cancer 73:2506–2511

    PubMed  Google Scholar 

  30. Rubin BP, Schuetze SM, Eary JF (2002) Molecular targeting of platelet-derived growth factor B by imatinib mesylate in a patient with metastatic dermatofibrosarcoma protuberans. J Clin Oncol 20:3586–3591

    Article  PubMed  Google Scholar 

  31. Schramm A, Apostolov O, Sitek B, Pfeiffer K, Stühler K, Meyer HE, Havers W, Eggert A (2003) Proteomics: Techniques and applications in cancer research. Klin Pädiatr 215:293–297

  32. Schulte JH, Schramm A, Pressel T, Klein-Hitpass L, Kremens B, Eils J, Havers W, Eggert A (2003) Microarray-analysis: A new approach to study the molecular mechanisms of thermo-chemotherapy. Klin Pädiatr 215:298–302

    Google Scholar 

  33. Siebenrock KA, Hertel R, Ganz R (2000) Unexpected resection of soft-tissue sarcoma. More mutilating surgery, higher local recurrence rates, and obscure prognosis as consequences of improper surgery. Arch Orthop Trauma Surg 120:65–69

    PubMed  Google Scholar 

  34. Staege MS, Hattenhorst UE, Neumann I, Hutter C, Foja S, Burdach S (2003) DNA-Microarrays as tools for the identification of tumor specific gene expression profiles: Applications in tumor biology, diagnosis and therapy. Klin Pädiatr 215:135–138

    Google Scholar 

  35. Stegmeier S, Leuschner I, Aakcha-Rudel E, Münch P, Kazanowska B, Bekassy A, Treuner J, Koscielniak E (2004) Identification of various exon combinations of the ews/fli1 translocation: An optimized PT-PCR method for paraffin embedded tissue. A report by the CWS-Study Group. Klin Pädiatr 216:315–322

    Google Scholar 

  36. Stevens MC, Rey A, Bouvet N (2005) Treatment of nonmetastatic rhabdomyosarcoma in childhood and adolescence: third study of the International Society of Paediatric Oncology--SIOP Malignant Mesenchymal Tumor 89. J Clin Oncol 23:2618–2628

    Article  PubMed  Google Scholar 

  37. Treuner J (2003) Interim Report of the CWS-96 Study: Results of the tretament for soft tissue sarcomas in childhood

  38. Wessalowski R, Schneider DT, Mils O et al. (2003) An approach for cure: PEI-chemotherapy and regional deep hyperthermia in children and adolescents with unresectable malignant tumors. Klin Pädiatr 215:303–309

    Google Scholar 

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Authors and Affiliations

  1. Olgahospital Stuttgart

    E. Koscielniak

  2. Universitätskinderklinik Frankfurt

    T. Klingebiel

  3. Universität Münster

    A. Schuck

  4. Kindertumorregister Kiel

    I. Leuschner

  5. Olgahospital, Bismarckstraße 8, 70 176, Stuttgart

    E. Koscielniak

Authors
  1. E. Koscielniak
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  2. T. Klingebiel
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  3. A. Schuck
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  4. I. Leuschner
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Correspondence to E. Koscielniak.

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Koscielniak, E., Klingebiel, T., Schuck, A. et al. Weichteilsarkome im Kindesalter. Onkologe 11, 1047–1053 (2005). https://doi.org/10.1007/s00761-005-0928-z

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  • DOI: https://doi.org/10.1007/s00761-005-0928-z

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