title: Kasabach-Merritt syndrome associated to angiosarcoma of the breast. A case report and review of the literature.
abstract: BACKGROUND
  Kasabach-Merritt syndrome is characterized by the association of a consumptive thrombohemorrhagic disorder and angioma occurring usually in children. In the present study, a case of Kasabach-Merritt syndrome associated to an angiosarcoma of the breast is reported.
  
  
  METHODS
  The tumor together with the thrombohemorrhagic disorder manifested in a 28-year-old woman. The patient underwent mastectomy. The tumor recurred in the same site 2 years later. The patient died of severe anemia 8 years after the first appearance of the angiosarcoma.
  
  
  METHODS
  Tissues were formalin fixed and paraffin embedded; in addition, selected sections were immunohistochemically stained.
  
  
  CONCLUSIONS
  A well-differentiated angiosarcoma was visible throughout the removed organ. A review of the literature showed that only 6 cases of Kasabach-Merritt syndrome associated to malignant vascular tumors have been previously reported. All these 6 cases occurred in adult patients. On the contrary, Kasabach-Merritt syndrome associated to benign vascular tumors affects children. This is the first case occurring in the breast.
authors.affiliation: Department of Pathology, University of Bologna, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Mazzocchi
authors.forename: M P
authors.initials: MP
authors.lastname: Foschini
authors.forename: F
authors.initials: F
authors.lastname: Marconi
authors.forename: V
authors.initials: V
authors.lastname: Eusebi
mesh_terms: D000328:Adult; D000347:Afibrinogenemia / Q000150:complications; D001943:Breast Neoplasms / Q000209:etiology* / Q000473:pathology; D005260:Female; D006391:Hemangioma; D006394:Hemangiosarcoma / Q000209:etiology* / Q000473:pathology; D006801:Humans; D011696:Purpura, Thrombocytopenic; D013577:Syndrome