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.
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Fuchs, J. (2019). Weichteiltumoren bei Kindern und Jugendlichen. In: von Schweinitz, D., Ure, B. (eds) Kinderchirurgie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-53390-1_90-1
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