Differential expression of leukocyte differentiation antigens in small round blue cell sarcomas

Abstract

Small round blue cell sarcomas (SRBCS) comprise a group of cytomorphologically poorly differentiated neoplasms that are characterized by different histogenesis and biologic behavior. Twenty-seven well-characterized SRBCS were examined immunohistochemically to detect the expression of a panel of leukocyte differentiation (CD) antigens and class I (HLA-A,B,C) and class II (HLA-DR) major histocompatibility complex antigen. Although various cell surface antigens were detectable in SRBCS, the pan-leukocytic-histiocytic CD53 antigen was absent in the neoplastic population of all tumors studied; this finding allowed the authors to discriminate these lesions from lymphomas and leukemias. Some antigens had a differential pattern of expression in the SRBCS group, in particular in the undifferentiated tumor cell populations. In most instances, neuroblastomas (NB) and ganglioneuroblastomas (GNB) were CD9+/CD24+/CD56+ but CD40-/HLA-A,B,C-. Rhabdomyosarcomas (RMS) were CD56+ in all specimens and CD9+ in many samples; generally, they showed CD24-/CD40-/HLA-A,B,C-. Ewing sarcomas (ES) and peripheral primitive neuroectodermal tumors (pPNET) were HLA-A,B,C+/CD40+ but CD9-/CD24-/CD56- in most instances. Thus, with few exceptions, the expression of CD9 and CD56 and the simultaneous absence of HLA-A,B,C and CD40 differentiated GNB, NB, and RMS from ES and pPNET. GNB, NB, and RMS differed in regard to their CD24 expression. These data show that various types of SRBCS have different patterns of cell surface antigens. Therefore, these antigens are considered to be helpful in the immunophenotypic subclassification of SRBCS: The immunophenotypic similarities between ES and pPNET, however, might be an additional argument for a close relationship between these two lesions.