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Spindelzelliges Rhabdomyosarkom des Erwachsenen

Eine neue Entität im Spektrum maligner mesenchymaler Tumoren des Weichgewebes

Spindle cell rhabdomyosarcoma in adults

A new entity in the spectrum of malignant mesenchymal tumors of soft tissues

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Zusammenfassung

Rhabdomyosarkome (RMS) sind seltene Sarkome des Weichgewebes mit einer Differenzierung entsprechend der Skelettmuskulatur. Histologisch werden embryonale, alveoläre und pleomorphe Subtypen unterschieden. Während im Kindes- und Jugendalter – der Altersgruppe, in der RMS zu den häufigsten Sarkomen gehören – embryonale und alveoläre Subtypen dominieren, sind bei erwachsenen Patienten RMS extrem selten. In dieser Altersgruppe entstehen bevorzugt pleomorphe RMS. Spindelzellige und sklerosierende RMS des Erwachsenen sind im Unterschied zu morphologisch vergleichbaren Tumoren im Kindesalter hochmaligne Sarkome und müssen differenzialdiagnostisch von spindelzelligen malignen Melanomen, spindelzelligen Karzinomen und einer Reihe spindelzelliger Sarkome anderer Differenzierungsrichtung unterschieden werden. Spindelzellige und sklerosierende RMS entstehen bevorzugt in der Kopf-Hals-Region und den Extremitäten und bestehen aus atypischen Spindelzellen mit eingestreuten Rhabdomyoblasten. Da spindelzellige RMS zellärmere Areale mit einem kollagenfaserreichen, sklerosierten Stroma enthalten können, und in einigen Fällen sklerosierender RMS auch zellreichere Tumorareale vorhanden sind, kann man von einem morphologischen Spektrum einer distinkten Formvariante im Spektrum der RMS des Erwachsenenalters ausgehen.

Abstract

Rhabdomyosarcomas (RMS) are rare soft tissue sarcomas showing a skeletal muscle differentiation. Histologically, embryonal, alveolar and pleomorphic subtypes are distinguished. RMS represent one of the most frequent sarcomas in children and adolescents, and in this age group embryonal and alveolar subtypes predominate. RMS in adults is extremely rare, and the pleomorphic subtype is most frequently seen. Spindle cell and sclerosing RMS in adults are very rare and, unlike the morphologically comparable tumors in children and adolescents, clinically aggressive neoplasms, which need to be distinguished from spindle cell malignant melanoma, spindle cell sarcomatoid carcinoma and from a number of spindle cell sarcomas of varying lines of differentiation. Spindle cell and sclerosing RMS in adults arise predominantly in the head and neck region and on the extremities, and are composed of atypical spindled cells and scattered rhabdomyoblasts. Since spindle cell RMS may contain areas of lower cellularity with abundant sclerosing stroma, and sclerosing RMS may show focal areas of increased cellularity, it is most likely that both forms represent a morphological spectrum of a single RMS variant.

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Mentzel, T. Spindelzelliges Rhabdomyosarkom des Erwachsenen. Pathologe 31, 91–96 (2010). https://doi.org/10.1007/s00292-009-1249-6

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