[Desmoplastic small cell tumor with multi-phenotype differentiation]

The desmoplastic small cell tumor with divergent differentiation is a malignant tumor with a complex phenotype. Its correct classification remains difficult. The histologic pattern shows tumor cell nests surrounded by a cellular desmoplastic stroma. The immunohistochemical response to epithelial and muscle markers is positive. Neural differentiation and association with cytogenetic abnormality are observed. This tumor probably belongs to the family of primitive small round cell tumors that predominantly occur during childhood.