Mazzocchi A, Foschini M P, Marconi F, Eusebi V
Department of Pathology, University of Bologna, Italy.
Tumori. 1993 Apr 30;79(2):137-40. doi: 10.1177/030089169307900212.
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.
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.
Tissues were formalin fixed and paraffin embedded; in addition, selected sections were immunohistochemically stained.
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.
卡萨巴赫 - 梅里特综合征的特征是通常发生在儿童身上的消耗性血栓出血性疾病与血管瘤相关联。在本研究中，报告了一例与乳腺血管肉瘤相关的卡萨巴赫 - 梅里特综合征病例。
肿瘤连同血栓出血性疾病在一名28岁女性中出现。患者接受了乳房切除术。两年后肿瘤在同一部位复发。血管肉瘤首次出现8年后，患者死于严重贫血。
组织用福尔马林固定并石蜡包埋；此外，对选定切片进行免疫组织化学染色。
在切除的器官中可见一个高分化血管肉瘤。文献回顾显示，此前仅报道过6例与恶性血管肿瘤相关的卡萨巴赫 - 梅里特综合征病例。所有这6例均发生在成年患者中。相反，与良性血管肿瘤相关的卡萨巴赫 - 梅里特综合征影响儿童。这是首例发生在乳腺的病例。
