Advertisement

Weichteiltumoren bei Kindern und Jugendlichen

  • Jörg FuchsEmail author
Living reference work entry
Part of the Springer Reference Medizin book series (SRM)

Zusammenfassung

Weichteiltumoren stellen eine komplexe Gruppe verschiedener Tumoren im Kindesalter mit >20 verschiedenen Entitäten dar. Prozentual machen sie ca. 5–8 % aller kindlichen Tumoren aus. Sarkome sind maligne Tumoren, die von mesenchymalen Zellen ausgehen. Grundsätzlich kann man bei den Weichteilsarkomen zwischen den „RMS-artigen Weichteilsarkomen“ und den „nicht-RMS-artigen Weichteilsarkomen“ unterscheiden. Das Rhabdomyosarkom ist mit einem Anteil von 60–70 % der häufigste maligne Weichteiltumor (Perez et al. 2011, Rhabdomyosarcoma in children: a SEER population based study. J Surg Res 170:e243–e251). Die Klassifikation ist jedoch aufgrund der vielfältigen Tumorentitäten schwierig und durch neue molekulargenetische Erkenntnisse in der Tumoranalytik einer ständigen Modifikation unterworfen. Wahrscheinlich wird es in naher Zukunft eine neue molekulargenetische Klassifikation der Weichteiltumoren geben. Epidemiologisch kommen die Tumoren bei Knaben und der weißen Bevölkerung etwas häufiger vor. Hinsichtlich der Altersverteilung gibt es eine bimodale Distribution mit einem Erkrankungsgipfel zwischen 2 und 6 sowie 10 und 18 Jahren. Die Behandlung erfolgt in Abhängigkeit von der Tumorentität, der Tumorausdehnung und der Lokalisationen sowie dem Alter des Patienten. Grundsätzlich gilt es bei jedem Weichteiltumor im Kindesalter eine maligne Erkrankung auszuschließen. In den letzten 30 Jahren konnten durch internationale und nationale Therapieoptimierungsstudien die Überlebensrate der Kinder deutlich verbessert werden. Diese positiven Ergebnisse basieren auf einer interdisziplinären Kooperation zwischen den Fachgebieten Kinderonkologie, Radiologie, Pathologie, Strahlentherapie und Chirurgie.

Literatur

  1. Bisogno G, De RC, Gamboa Y et al (2008) Improved survival for children with parameningeal rhabdomyosarcoma: results from the AIEOP soft tissue sarcoma committee. Pediatr Blood Cancer 50:1154–1158CrossRefGoogle Scholar
  2. Bisogno G, Pastore G, Perilongo G et al (2012) Long-term results in childhood rhabdomyosarcoma: a report from the Italian Cooperative Study RMS 79. Pediatr Blood Cancer 58:872–876CrossRefGoogle Scholar
  3. Breneman J, Meza J, Donaldson SS et al (2012) Local control with reduced-dose radiotherapy for low-risk rhabdomyosarcoma: a report from the Children’s Oncology Group D9602 study. Int J Radiat Oncol Biol Phys 83:720–726CrossRefGoogle Scholar
  4. Carli M, Ferrari A, Mattke A et al (2005) Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol 23:8422–8430CrossRefGoogle Scholar
  5. Chargari C, Haie-Meder C, Guerin F et al (2017) Brachytherapy combined with surgery for conservative treatment of children with bladder neck and/or prostate rhabdomyosarcoma. Int J Radiat Oncol Biol Phys 98:352–359CrossRefGoogle Scholar
  6. Chisholm JC, Marandet J, Rey A et al (2011) Prognostic factors after relapse in nonmetastatic rhabdomyosarcoma: a nomogram to better define patients who can be salvaged with further therapy. J Clin Oncol 29:1319–1325CrossRefGoogle Scholar
  7. Chisholm JC, Merks JHM, Casanova M et al (2017) Open-label, multicentre, randomised, phase II study of the EpSSG and the ITCC evaluating the addition of bevacizumab to chemotherapy in childhood and adolescent patients with metastatic soft tissue sarcoma (the BERNIE study). Eur J Cancer 83:177–184CrossRefGoogle Scholar
  8. Combs SE, Behnisch W, Kulozik AE et al (2007) Intensity Modulated Radiotherapy (IMRT) and Fractionated Stereotactic Radiotherapy (FSRT) for children with head-and-neck-rhabdomyosarcoma. BMC Cancer 7:177CrossRefGoogle Scholar
  9. Crist WM, Anderson JR, Meza JL et al (2001) Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J Clin Oncol 19:3091–3102CrossRefGoogle Scholar
  10. Dantonello TM, Int-Veen C, Harms D et al (2009) Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults. J Clin Oncol 27:1446–1455CrossRefGoogle Scholar
  11. Dasgupta R, Rodeberg DA (2012) Update on rhabdomyosarcoma. Semin Pediatr Surg 21:68–78CrossRefGoogle Scholar
  12. Dasgupta R, Fuchs J, Rodeberg D (2016) Rhabdomyosarcoma. Semin Pediatr Surg 25:276–283CrossRefGoogle Scholar
  13. De CF, Dall’igna P, Bisogno G et al (2009) Sentinel node biopsy in pediatric soft tissue sarcomas of extremities. Pediatr Blood Cancer 52:51–54CrossRefGoogle Scholar
  14. Donaldson SS, Meza J, Breneman JC et al (2001) Results from the IRS-IV randomized trial of hyperfractionated radiotherapy in children with rhabdomyosarcoma – a report from the IRSG. Int J Radiat Oncol Biol Phys 51:718–728CrossRefGoogle Scholar
  15. Fernandez-Pineda I, Spunt SL, Parida L et al (2011) Vaginal tumors in childhood: the experience of St. Jude Children’s Research Hospital. J Pediatr Surg 46:2071–2075CrossRefGoogle Scholar
  16. Ferrari A, Orbach D, Sultan I et al (2012) Neonatal soft tissue sarcomas. Semin Fetal Neonatal Med 17:231–238CrossRefGoogle Scholar
  17. Frassica FJ, Khanna JA, Mccarthy EF (2000) The role of MR imaging in soft tissue tumor evaluation: perspective of the orthopedic oncologist and musculoskeletal pathologist. Magn Reson Imaging Clin N Am 8:915–927PubMedGoogle Scholar
  18. Fuchs J (2012) Solide Tumoren im Kindesalter; Grundlagen, Diagnostik, Therapie. Schattauer, StuttgartGoogle Scholar
  19. Fuchs J, Dantonello TM, Blumenstock G et al (2014) Treatment and outcome of patients suffering from perineal/perianal rhabdomyosarcoma: results from the CWS trials – retrospective clinical study. Ann Surg 259:1166–1172CrossRefGoogle Scholar
  20. Fuchs J, Paulsen F, Bleif M et al (2016) Conservative surgery with combined high dose rate brachytherapy for patients suffering from genitourinary and perianal rhabdomyosarcoma. Radiother oncol 121:262–267CrossRefGoogle Scholar
  21. Haie-Meder C, Martelli H, Dumas I et al (2011) Brachytherapy in paediatric sarcoma. Bull Cancer 98:549–557PubMedGoogle Scholar
  22. Hayes-Jordan A (2012) Recent advances in non-rhabdomyosarcoma soft-tissue sarcomas. Semin Pediatr Surg 21:61–67CrossRefGoogle Scholar
  23. Hayes-Jordan A, Anderson PM (2011) The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol 23:385–389CrossRefGoogle Scholar
  24. Hayes-Jordan A, Andrassy R (2009) Rhabdomyosarcoma in children. Curr Opin Pediatr 21:373–378CrossRefGoogle Scholar
  25. Hayes-Jordan A, Stoner JA, Anderson JR et al (2008) The impact of surgical excision in chest wall rhabdomyosarcoma: a report from the Children’s Oncology Group. J Pediatr Surg 43:831–836CrossRefGoogle Scholar
  26. Hayes-Jordan A, Green H, Ludwig J et al (2012) Toxicity of hyperthermic intraperitoneal chemotherapy (HIPEC) in pediatric patients with sarcomatosis/carcinomatosis: early experience and phase 1 results. Pediatr Blood Cancer 59:395–397CrossRefGoogle Scholar
  27. Heinzelmann F, Thorwarth D, Lamprecht U et al (2011) Comparison of different adjuvant radiotherapy approaches in childhood bladder/prostate rhabdomyosarcoma treated with conservative surgery. Strahlenther Onkol 187:715–721CrossRefGoogle Scholar
  28. Klingebiel T, Boos J, Beske F et al (2008) Treatment of children with metastatic soft tissue sarcoma with oral maintenance compared to high dose chemotherapy: report of the HD CWS-96 trial. Pediatr Blood Cancer 50:739–745CrossRefGoogle Scholar
  29. Koscielniak E, Morgan M, Treuner J (2002) Soft tissue sarcoma in children: prognosis and management. Paediatr Drugs 4:21–28CrossRefGoogle Scholar
  30. Magnusson S, Gisselsson D, Wiebe T et al (2012) Prevalence of germline TP53 mutations and history of Li-Fraumeni syndrome in families with childhood adrenocortical tumors, choroid plexus tumors, and rhabdomyosarcoma: a population-based survey. Pediatr Blood Cancer 59:846–853CrossRefGoogle Scholar
  31. Malempati S, Hawkins DS (2012) Rhabdomyosarcoma: review of the Children’s Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studies. Pediatr Blood Cancer 59:5–10CrossRefGoogle Scholar
  32. Martelli H, Oberlin O, Rey A et al (1999) Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: a report from the study committee of the international society of pediatric oncology. J Clin Oncol 17:2117–2122CrossRefGoogle Scholar
  33. Martelli H, Haie-Meder C, Branchereau S et al (2009) Conservative surgery plus brachytherapy treatment for boys with prostate and/or bladder neck rhabdomyosarcoma: a single team experience. J Pediatr Surg 44:190–196CrossRefGoogle Scholar
  34. Newton WA Jr, Gehan EA, Webber BL et al (1995) Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification – an intergroup rhabdomyosarcoma study. Cancer 76:1073–1085CrossRefGoogle Scholar
  35. Oberlin O, Rey A, Sanchez De TJ et al (2012) Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatric Oncology MMT95 study. J Clin Oncol 30:2457–2465CrossRefGoogle Scholar
  36. Parham DM, Webber BL, Jenkins JJ 3rd et al (1995) Nonrhabdomyosarcomatous soft tissue sarcomas of childhood: formulation of a simplified system for grading. Mod Pathol 8:705–710PubMedGoogle Scholar
  37. Paulino AC (2004) Treatment options for children with nonrhabdomyosarcoma soft tissue sarcoma. Expert Rev Anticancer Ther 4:247–256CrossRefGoogle Scholar
  38. Perez EA, Kassira N, Cheung MC et al (2011) Rhabdomyosarcoma in children: a SEER population based study. J Surg Res 170:e243–e251CrossRefGoogle Scholar
  39. Raney B, Anderson J, Jenney M et al (2006) Late effects in 164 patients with rhabdomyosarcoma of the bladder/prostate region: a report from the international workshop. J Urol 176:2190–2194CrossRefGoogle Scholar
  40. Reinhard H, Reinert J, Beier R et al (2008) Rhabdoid tumors in children: prognostic factors in 70 patients diagnosed in Germany. Oncol Rep 19:819–823PubMedGoogle Scholar
  41. Rodeberg DA, Paidas CN, Lobe TL et al (2002) Surgical principles for children/adolescents with newly diagnosed rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children’s Oncology Group. Sarcoma 6:111–122CrossRefGoogle Scholar
  42. Rodeberg DA, Anderson JR, Arndt CA et al (2011) Comparison of outcomes based on treatment algorithms for rhabdomyosarcoma of the bladder/prostate: combined results from the Children’s Oncology Group, German Cooperative Soft Tissue Sarcoma Study, Italian Cooperative Group, and International Society of Pediatric Oncology Malignant Mesenchymal Tumors Committee. Int J Cancer 128:1232–1239CrossRefGoogle Scholar
  43. Schalow EL, Broecker BH (2003) Role of surgery in children with rhabdomyosarcoma. Med Pediatr Oncol 41:1–6CrossRefGoogle Scholar
  44. Seitz G, Dantonello TM, Int-Veen C et al (2011) Treatment efficiency, outcome and surgical treatment problems in patients suffering from localized embryonal bladder/prostate rhabdomyosarcoma: a report from the Cooperative Soft Tissue Sarcoma trial CWS-96. Pediatr Blood Cancer 56:718–724CrossRefGoogle Scholar
  45. Seitz G, Dantonello TM, Kosztyla D et al (2014) Impact of hemiscrotectomy on outcome of patients with embryonal paratesticular rhabdomyosarcoma: results from the Cooperative Soft Tissue Sarcoma Group Studies CWS-86, 91, 96 and 2002P. J Urol 192:902–907CrossRefGoogle Scholar
  46. Seitz G, Fuchs J, Sparber-Sauer M et al (2016) Improvements in the treatment of patients suffering from bladder-prostate rhabdomyosarcoma: a report from the CWS-2002P trial. Ann Surg Oncol 23:4067–4072CrossRefGoogle Scholar
  47. Sobin LH (2009) UICC:TNM classification of malignant tumors. Wiley, New JerseyGoogle Scholar
  48. Sparber-Sauer M, Von Kalle T, Seitz G et al (2017a) The prognostic value of early radiographic response in children and adolescents with embryonal rhabdomyosarcoma stage IV, metastases confined to the lungs: a report from the Cooperative Weichteilsarkom Studiengruppe (CWS). Pediatr Blood Cancer 64:1–7CrossRefGoogle Scholar
  49. Sparber-Sauer M, Seitz G, Kirsch S, Vokuhl C, Leuschner I, Dantonello TM, Scheer M, von Kalle T, Ljungman G, Bielack SS, Klingebiel T, Fuchs J, Koscielniak E, CWS Study Group (2017b) The impact of local control in the treatment of type II/III pleuropulmonary blastoma. Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS). J Surg Oncol 115(2)164–172.  https://doi.org/10.1002/jso.24416. Epub 2017 Jan 19CrossRefPubMedGoogle Scholar
  50. Walter F, Czernin J, Hall T et al (2012) Is there a need for dedicated bone imaging in addition to 18F-FDG PET/CT imaging in pediatric sarcoma patients? J Pediatr Hematol Oncol 34:131–136CrossRefGoogle Scholar
  51. Walterhouse DO, Meza JL, Breneman JC et al (2011) Local control and outcome in children with localized vaginal rhabdomyosarcoma: a report from the Soft Tissue Sarcoma committee of the Children’s Oncology Group. Pediatr Blood Cancer 57:76–83CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Deutschland, ein Teil von Springer Nature 2019

Authors and Affiliations

  1. 1.Abteilung für Kinderchirurgie und KinderurologieUniversitätsklinik für Kinder- und JugendmedizinTübingenDeutschland

Personalised recommendations