Intra-abdominal desmoplastic small cell tumor in an adolescent suggesting a neurogenic origin

A case of a desmoplastic small cell tumor of the large omentum associated with gross ascites that occurred in a male adolescent is reported. Light microscopic studies revealed that the tumor cells were small and epithelioid in nature with eosinophilic hyaline material located in the perinuclear area. They were surrounded by rich desmoplastic and myxoidal stromal bands. Immunohistochemical staining revealed globoid perinuclear positivity for desmin. Vimentin, cytokeratin (AE3, CaM 5.2), epithelial membrane antigen, tissue polypeptide antigen, neuron-specific enolase, chromogranin A, endocrine granule constituent and synaptophysin were also positive in the cytoplasm. Electron microscopy revealed whorled intermediate filaments and some dense core granules in the cytoplasm. Bundles of microtubules in the cytoplasmic process and occasional cell junctions of zonulae adherentes in the tumor cells were also observed. DNA analysis of the tumor cells showed the three-fold amplification of the N-myc gene. Although desmoplastic small cell tumors showed a heterogeneous pattern with immunohistochemical studies, it is suggested that the tumor may originate from neurogenic cells.