Abstract

A group of high-grade small round-cell neuroectodermal tumors developing in soft tissues and bones has been recently identified in children and young adults on the basis of the data of classical morphology and the results obtained by new techniques (cell culturing, molecular genetics, immunohistochemistry). To denote these tumors, the term "primitive peripheral neuroectodermal tumor" (PNET) has been proposed. The problem is of great practical importance in the differential diagnosis of formally similar malignancies (lymphoma, embryonal rhabdomyosarcoma, synovial sarcoma, small-cell variant of osteosarcoma), this makes PNET and Ewing's sarcoma be histogenetically and clinicodiagnostically of special scientific and practical interest.

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