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Kindliche Tumoren mit Spindelzellmorphologie

Pediatric tumors with spindle cell morphology

  • Schwerpunkt: Sarkome
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Zusammenfassung

Spindelzellige Tumoren des Kindesalters sind seltene Läsionen mit heterogenem morphologischen Bild und klinischen Verlauf, wobei das Spektrum von benignen Tumoren, Läsionen mit intermediärer Malignität bis hin zu malignen Tumoren reicht. Klinisch macht die Beurteilung dieser Tumoren immer wieder Schwierigkeiten, da zum einen sehr schnell wachsende Läsionen benignen Tumoren entsprechen und langsam wachsende Tumoren maligne Entitäten darstellen können. Aufgrund der Seltenheit dieser Entitäten, der sich oft ähnelnden Morphologie sowie der teils uncharakteristischen immunhistochemischen Profile bereiten diese Tumoren auch den Pathologen immer wieder diagnostische Schwierigkeiten. Im Rahmen dieser Übersicht sollen neben der Morphologie und den spezifischen immunhistochemischen Aspekten auch auf die molekularen Veränderungen eingegangen werden. Zudem werden auch die neu beschriebenen Translokationen einiger Tumorentitäten besprochen, da sie teilweise auch therapeutische Optionen implizieren.

Abstract

Spindle cell tumors in childhood are rare lesions with a heterogeneous morphological picture and clinical course, ranging from benign lesions to fully malignant tumors. The clinical assessment of these tumors is often challenging since some of them show fast growth dynamics but are utterly benign, while a subset of slow-growing tumors can represent malignant entities. Due to the rarity of these tumors as well as the overlapping morphology and the often uncharacteristic immunohistochemical profiles, the pathologic diagnosis is often also difficult. This review gives an overview of some of the more common pediatric spindle cell tumors. In addition to the morphological features and immunohistochemical aspects, specific molecular changes are discussed. Here, some of the newly described translocations that may imply therapeutic options, are presented.

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  1. Kindertumorregister der GPOH, Sektion Kinderpathologie, Institut für Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 10/Haus 4, 24105, Kiel, Deutschland

    C. Vokuhl

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Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Vokuhl, C. Kindliche Tumoren mit Spindelzellmorphologie. Pathologe 40, 381–392 (2019). https://doi.org/10.1007/s00292-019-0602-7

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