Kasabach-Merritt syndrome associated to angiosarcoma of the breast. A case report and review of the literature.
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.
Mazzocchi
A
A
Department of Pathology, University of Bologna, Italy.
Foschini
M P
MP
Marconi
F
F
Eusebi
V
V
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