Key result
Mastectomy for breast angiosarcoma in Kasabach-Merritt syndrome precedes fatal severe anemia at 8 years.
Why the study?
Kasabach-Merritt syndrome is usually associated with benign vascular tumors in children, but its association with malignant vascular tumors in adults, especially in the breast, is rare and not well documented.
Case Report (n=1)
This represents the first reported case of Kasabach-Merritt syndrome associated with an angiosarcoma occurring in the breast.
Alerts to possible Kasabach-Merritt syndrome in adult breast angiosarcoma; leaves open broader associations.
INTRODUCTION: 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. CLINICAL HISTORY: 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. MATERIAL AND METHODS: Tissues were formalin fixed and paraffin embedded; in addition, selected sections were immunohistochemically stained. RESULTS AND 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.
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Foschini et al. (1993) conducted a case report in Kasabach-Merritt syndrome associated to angiosarcoma of the breast (n=1). Mastectomy was evaluated. Mastectomy in a 28-year-old woman with Kasabach-Merritt syndrome and breast angiosarcoma was followed by tumor recurrence at 2 years and death from severe anemia at 8 years.
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