[High-grade small bowel angiosarcoma associated with angiosarcomatosis: a case report]

Ann Pathol. 2011 Aug;31(4):303-6. doi: 10.1016/j.annpat.2011.04.002. Epub 2011 Jul 8.
[Article in French]

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.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Antibiotics, Antineoplastic / therapeutic use
  • Biomarkers, Tumor / analysis
  • Combined Modality Therapy
  • Diagnostic Errors*
  • Doxorubicin / therapeutic use
  • Fatal Outcome
  • Female
  • Hemangioendothelioma, Epithelioid / chemistry
  • Hemangioendothelioma, Epithelioid / diagnosis*
  • Hemangioendothelioma, Epithelioid / drug therapy
  • Hemangioendothelioma, Epithelioid / pathology
  • Hemangioendothelioma, Epithelioid / surgery
  • Hemangiosarcoma / chemistry
  • Hemangiosarcoma / diagnosis
  • Hemangiosarcoma / drug therapy
  • Hemangiosarcoma / pathology
  • Hemangiosarcoma / secondary*
  • Hemangiosarcoma / surgery
  • Humans
  • Ileal Neoplasms / chemistry
  • Ileal Neoplasms / diagnosis*
  • Ileal Neoplasms / drug therapy
  • Ileal Neoplasms / pathology
  • Ileal Neoplasms / surgery
  • Mesentery / pathology*
  • Neoplasms, Multiple Primary / chemistry
  • Neoplasms, Multiple Primary / diagnosis*
  • Neoplasms, Multiple Primary / drug therapy
  • Neoplasms, Multiple Primary / pathology
  • Neoplasms, Multiple Primary / surgery
  • Peritoneal Neoplasms / chemistry
  • Peritoneal Neoplasms / diagnosis
  • Peritoneal Neoplasms / drug therapy
  • Peritoneal Neoplasms / pathology
  • Peritoneal Neoplasms / secondary*
  • Peritoneal Neoplasms / surgery
  • Peritonitis, Tuberculous / diagnosis

Substances

  • Antibiotics, Antineoplastic
  • Biomarkers, Tumor
  • Doxorubicin