Peripheral neuroectodermal sarcoma of soft tissue (peripheral neuroepithelioma): a pathologic study of ten cases with differential diagnosis regarding other small, round-cell sarcomas

Abstract

Peripheral neuroepithelioma of soft tissue belongs to the group of peripheral neuroectodermal tumors (PNETs), but because of its clinical, biological, and morphological characteristics, it differs from other small, round-cell sarcomas that appear in children (neuroblastoma) or in the thoracopulmonary region (Askin's tumor) and bone (peripheral neuroectodermal sarcoma of bone). We report ten new cases of such PNET variety, based on their histologic, immunohistochemical, and electron microscopic findings. In all of these cases, the clinicopathologic correlations demonstrated high malignancy, with an ominous outcome in nine cases. The mean age of the patients was 32.6 years and there was a clear male predominance (eight men, two women). Histologically, the presence of Homer-Wright rosettes is mandatory for diagnosis, being complemented with positive immunohistochemistry for several neural immunomarkers using paraffin-embedded material. Neuron-specific enolase, E-36, HNK-1, and chromogranin neural markers proved to be positive in a high number of cases, but other markers (S-100 protein, synapto-physin, GFA protein, and neurofilaments [70 kilodalton]) were absent. Electron microscopy confirmed the presence of neural structures, both by scanning and transmission electron microscopy.