Zusammenfassung
Hintergrund
Maligne fibrohistiozytäre und myofibrozytäre Tumore stellen eine heterogene Gruppe mesenchymaler Neoplasien dar, die in der Kutis und Subkutis auftreten.
Diagnose
Die Diagnose dieser seltenen Tumore wird durch eine ausgeprägte morphologische Vielfalt erschwert. In diesem Update werden relevante Aspekte der ausgewählten Entitäten Dermatofibrosarcoma protuberans, Desmoidtumor, atypisches Fibroxanthom, pleomorphes dermales Sarkom und Myxofibrosarkom entsprechend der WHO-Klassifikation von 2013 diskutiert. Die Klinik dieser Tumore ist oft uncharakteristisch; die histologische Aufarbeitung gilt daher als Goldstandard zur Diagnosesicherung. Hierbei nehmen die Immunhistochemie und die molekulare Diagnostik einen stetig größer werdenden Stellenwert ein.
Therapie
Die Therapie der Wahl stellt bei lokal behandelbaren Tumoren die komplette chirurgische Exzision dar. Chemotherapie, Radiatio sowie zunehmend auch die zielgerichtete Therapie mit Signaltransduktionsinhibitoren kommen bei inoperablen und fortgeschrittenen Tumoren zur Anwendung.
Abstract
Background
Malignant fibrohistiocytic tumors are a heterogeneous group of mesenchymal neoplasms that may occur in the skin and subcutaneous tissues.
Diagnosis
Diagnosis of these tumors may be difficult, as they are rare, and a wide morphological diversity of types and subtypes has been described. In this update, relevant aspects of selected entities like dermatofibrosarcoma protuberans, desmoid tumor, atypical fibroxanthoma, pleomorphic dermal sarcoma, and myxofibrosarcoma are discussed according to the WHO classification of 2013. The typical clinical feature of these tumors is their mostly asymptomatic appearance. For diagnosis, the histologic workup is therefore the key feature; herein immunohistochemistry as well as molecular diagnostics become increasingly important.
Therapy
The primary treatment for locally resectable tumors is complete surgical removal; chemotherapy, radiation, and targeted therapies with kinase inhibitors are available for inoperable and metastatic disease.
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Einhaltung ethischer Richtlinien
Interessenkonflikt. B. Aigner, S. Ugurel, S. Kaddu und J.C. Becker geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.
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Aigner, B., Ugurel, S., Kaddu, S. et al. Kutane Sarkome. Hautarzt 65, 614–622 (2014). https://doi.org/10.1007/s00105-013-2738-3
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DOI: https://doi.org/10.1007/s00105-013-2738-3