Zusammenfassung
Das epitheloide Sarkom ist ein seltenes Sarkom mit schlechter Langzeitprognose, das gehäuft bei jungen Patienten im Bereich der distalen Extremitäten auftritt und klinisch oft als eine indolente, nichtneoplastische Läsion fehlinterpretiert wird. Histologisch sind epitheloide Sarkome durch ein multinoduläres Wachstum mit zentralen Nekrosen charakterisiert. Sie bestehen aus zytologisch relativ uniformen epitheloiden Zellen, die eine Koexpression von Vimentin, Panzytokeratin und epithelialem Membranantigen sowie von CD34 in etwa der Hälfte der Fälle aufweisen. Interessanterweise ist bei der Mehrzahl der Tumoren ein Verlust der Expression von INI1 zu beobachten, wobei die Inaktivierung des Tumorsupressorgens SMARCB1/INI1 nur sehr selten durch Mutationen erfolgt. Das epitheloide Sarkom vom proximalen Typ besteht aus in konfluierenden Formationen angeordneten deutlich vergrößerten epitheloiden und rhabdoiden Tumorzellen und repräsentiert die morphologische Progressionsform dieser Tumorentität. Das fibromähnliche epitheloide Sarkom, das angiomatoide epitheloide Sarkom und das myxoide epitheloide Sarkom stellen seltene morphologische Formvarianten dar und müssen bei der Differenzialdiagnose zu anderen benignen und malignen Tumoren besonders beachtet werden.
Abstract
Epithelioid sarcoma represents a rare sarcoma with a poor long-term prognosis that arises predominantly on the distal extremities of young adult patients, often mimicking a benign, non-neoplastic condition. Histologically, epithelioid sarcoma is characterized by a multinodular growth with central necrosis, and the neoplasms are composed of relatively uniform epithelioid tumour cells showing a coexpression of vimentin, epithelial membrane antigen and pancytokeratin, and in about half of the cases of CD34. Interestingly, most cases of epithelioid sarcoma show a loss of INI1, whereas the inactivation of the tumour suppressor gene SMARCB1/INI1 is only rarely caused by mutation. The proximal variant of epithelioid sarcoma is composed of confluent sheets of enlarged epithelioid and rhabdoid tumour cells and represents the morphological progression of this entity. The fibroma-like variant of epithelioid sarcoma as well as the angiomatoid and myxoid variants of epithelioid sarcoma are rare morphological variants and need to be considered in the differential diagnosis of other benign and malignant neoplasms.
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Mentzel, T. Epitheloides Sarkom. Pathologe 31, 135–141 (2010). https://doi.org/10.1007/s00292-009-1250-0
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DOI: https://doi.org/10.1007/s00292-009-1250-0