Zusammenfassung
Gemäß den neuesten Richtlinien der Weltgesundheitsorganisation (WHO) sollte die Diagnose eines malignen fibrösen Histiozytoms (MFH) nur für Läsionen mit Tumorzellen ohne Nachweis einer bestimmten Ausdifferenzierung gestellt werden. Somit handelt sich es sich um eine Ausschlussdiagnose, weshalb die Bezeichnung hochgradig undifferenziertes pleomorphes Sarkom (UPS) vorzuziehen ist. Weichteilsarkome können mit einer Inzidenz von ca. 20 Fällen/1 Mio. Einwohner/Jahr in allen Körperteilen auftreten. Weichteiltumoren des Penis machen etwa 5 % sämtlicher Tumoren des Penis aus, die Inzidenz der Penissarkome wird auf ca. 0,6–1 Fälle/100.000 Patienten geschätzt. In der Literatur wurden bisher nur 7 Fälle beschrieben. Diese Kasuistik beschreibt den Fall eines 61-jährigen kaukasischen Mannes mit einer schmerzlosen Masse im oberen Schwellkörperbereich. Eine Inzisionsbiopsie mit nachfolgender breiter immunohistochemischer Untersuchung ergab ein hochgradig undifferenziertes pleomorphes Sarkom oder pleomorphes storiformes MFH. Neben dem Fallbericht stellen die Autoren einen Literaturreview zur Vertiefung der Diskussion über die schwierige Diagnosestellung vor.
Abstract
According to the most recent World Health Organization (WHO) guidelines a diagnosis of malignant fibrous histiocytoma (MFH) should be made only for lesions composed of tumor cells without evidence of a specific line of differentiation. This is therefore a diagnosis by exclusion which is why the name of undifferentiated high-grade pleomorphic sarcoma (UPS) should be preferred. Soft tissue sarcomas currently have an incidence in all body regions of approximately 20 cases per 1 million inhabitants per year. Soft tissue tumors of the penis represent approximately 5 % of all penile tumors and the incidence of penile sarcomas is estimated to be approximately 0.6-1 case per 100,000 patients. Only seven cases have so far been reported in the literature. This article describes the case of a 61-year-old Caucasian male who presented with a painless mass sited in the upper part of the corpus cavernosa. An incisional biopsy with a subsequent investigation using an extensive immunohistochemical panel were performed and a high-grade undifferentiated pleomorphic sarcoma or pleomorphic storiform MFH was diagnosed. In addition to the case report a literature review is presented to elaborate the discussion on the differential diagnoses of these kinds of lesions.
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G. Crisman, G. Margiotta, M. Calabresi, S. Discepoli und P. Leocata geben an, dass kein Interessenkonflikt besteht.
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Crisman, G., Margiotta, G., Calabresi, M. et al. Malignes fibröses Histiozytom des Penis. Pathologe 36, 385–388 (2015). https://doi.org/10.1007/s00292-015-0036-9
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DOI: https://doi.org/10.1007/s00292-015-0036-9