title: [High-grade small bowel angiosarcoma associated with angiosarcomatosis: a case report].
abstract: Angiosarcoma is a rare soft-tissue neoplasm occurring most often in the skin and the subcutaneous tissues and very rarely in the gastrointestinal tract. We report a case of a 25-year-old woman who presented with a small intestinal angiosarcoma associated with angiosarcomatosis. The diagnosis was established on surgical intestinal resection, that showed a high-grade angiosarcoma with epithelioid component and foci of agressive form of hemangioendothelioma. Immunohistochemical study revealed tumour cell positivity with endothelial markers CD31 and factor VIII whereas CD34 and epithelial markers were negative. The tumour displayed KIT (CD117) immunoreactivity without KIT or PDGFRA mutation on molecular analysis. Clinical and pathological features as well as differential diagnosis of this rare entity in gastrointestinal tract are discussed.
authors.affiliation: Service d'anatomie et cytologie pathologiques, hôpital de la Croix-Rousse, 103, grande rue de la Croix-Rousse, 69317 Lyon cedex 04, France.
authors.forename: Sanae
authors.initials: S
authors.lastname: Chahbouni
authors.forename: Raphaëlle
authors.initials: R
authors.lastname: Barnoud
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Watkin
authors.forename: Mojgan
authors.initials: M
authors.lastname: Devouassoux-Shisheboran
mesh_terms: D000328:Adult; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D003131:Combined Modality Therapy; D003951:Diagnostic Errors; D004317:Doxorubicin / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000737:chemistry / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D006394:Hemangiosarcoma / Q000737:chemistry / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000556:secondary* / Q000601:surgery; D006801:Humans; D007078:Ileal Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D008643:Mesentery / Q000473:pathology*; D009378:Neoplasms, Multiple Primary / Q000737:chemistry / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D010534:Peritoneal Neoplasms / Q000737:chemistry / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000556:secondary* / Q000601:surgery; D014395:Peritonitis, Tuberculous / Q000175:diagnosis
chemical_list: D000903:Antibiotics, Antineoplastic; D014408:Biomarkers, Tumor; D004317:Doxorubicin