title: Gastrointestinal autonomic nerve tumor presenting as high-grade sarcoma. Case report and review of the literature.
abstract: Gastrointestinal autonomic nerve (GAN) tumors, also known as plexosarcomas, are a rare distinct subtype of the gastrointestinal stromal tumors. These tumors are usually histologically low-grade, epithelioid or spindle-cell neoplasms that can be distinguished from the other gastrointestinal stromal tumors on the basis of their unique ultrastructural features. A 66-year-old female presented with a histologically high-grade sarcoma of the small bowel. Ultrastructural studies showed features of a GAN tumor. The light microscopic and ultrastructural features are described. The tumor cells gave strong, diffuse staining for vimentin and synaptophysin, and weak focal staining for neuron-specific enolase and S100. While usually presenting as low-grade neoplasms on histologic examination, this case demonstrates that GAN tumors should be considered in the differential diagnosis of a histologically high-grade sarcoma of the gastrointestinal tract, especially when evidence of smooth muscle, peripheral nerve sheath, or neuroblastic origin is not forthcoming.
authors.affiliation: Department of Pathology, University of Arkansas for Medical Sciences, Little Rock 72205.
authors.forename: J R
authors.initials: JR
authors.lastname: Thomas
authors.forename: R E
authors.initials: RE
authors.lastname: Mrak
authors.forename: N
authors.initials: N
authors.lastname: Libuit
mesh_terms: D000368:Aged; D001341:Autonomic Nervous System; D005260:Female; D006801:Humans; D007078:Ileal Neoplasms / Q000473:pathology* / Q000648:ultrastructure; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000556:secondary; D009380:Neoplasms, Nerve Tissue / Q000648:ultrastructure*; D012509:Sarcoma / Q000473:pathology*; D016708:Synaptophysin / Q000032:analysis; D014746:Vimentin / Q000032:analysis
chemical_list: D016708:Synaptophysin; D014746:Vimentin