Abstract
BackgroundWhereas we can use several human epithelioid sarcoma (ES) cell lines for basic and preclinical research, an angiomatoid ES cell line has not been reported to date. We have treated a case of an angiomatoid ES developing in the right upper extremity of a 67-year-old man.MethodsAn angiomatoid ES cell line, Asra-EPS was newly established and characterized for its morphology, growth rate and chromosomal analysis. Tumorigenicity of Asra-EPS cells was also analyzed in athymic nude mice.ResultsAsra-EPS cells were round, polygonal or spindle-shaped with an abundant cytoplasm and have been maintained continuously in vitro for over 150 passages during more than 15 months. These cells secreted cancer antigen 125 (CA 125), interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF) into the culture medium. Asra-EPS cells were tumorigenic when implanted in nude mice with tumors reaching a volume of 1000 mm3 at around 50 days. Histological features of tumors formed in mice were essentially the same as those of the original tumor, exhibiting a multinodular proliferation of eosinophilic epithelioid and spindle-shaped cells with prominent areas of hemorrhage and blood-filled cystic spaces strikingly corresponding to the potential of hemorrhagic cyst formation in the original tumor. They showed immunopositive staining for cytokeratins (AE1/AE3 and CAM5.2), epithelial membrane antigen (EMA), vimentin, CD31, CD34 and CA 125, but negative for integrase interactor 1 (INI-1) and factor VIII-related antigen.ConclusionsThe established cell line represents a biologically relevant new tool to investigate the molecular pathology of human angiomatoid ES and to evaluate the efficacy of novel therapeutics both in vitro and in vivo.
Highlights
Whereas we can use several human epithelioid sarcoma (ES) cell lines for basic and preclinical research, an angiomatoid ES cell line has not been reported to date
Blood analyses revealed the data to be within normal limits except for a marked elevation of C-reactive protein (CRP) and cancer antigen 125 (CA 125)
We showed that Asra-EPS could constitutively produce CA 125 and IL-6
Summary
Whereas we can use several human epithelioid sarcoma (ES) cell lines for basic and preclinical research, an angiomatoid ES cell line has not been reported to date. Epithelioid sarcoma (ES) was first described in 1970 by Enzinger as a distinct soft tissue tumor with mixed epithelial and mesenchymal phenotype, but the origin and true nature of ES remain controversial [1]. A small number of ES cell lines have successfully been established [10,11,12,13,14,15,16,17,18,19,20,21], but there is no cell line from angiomatoid ES reported (Table 1). We present a case of angiomatoid ES arising in a 67-year-old man and establish an angiomatoid ES cell line from this patient. We describe the characteristics of a newly established angiomatoid ES cell line, Asra-EPS, both in vitro and in vivo
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