Larbcharoensub Noppadol, Singhsnaeh Arunee, Leelachaikul Paisan
Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Thailand.
J Med Assoc Thai. 2004 Sep;87(9):1116-22.
Angiosarcoma is a relatively uncommon primary malignant tumor of the heart derived from the endothelial cells. The authors report a case of primary cardiac angiosarcoma of the right atrium with pulmonary, hepatic, cerebellar and bony metastases in a 46-year-old man who clinically presented as pulmonary hemorrhage. The patient's occupational history had occasional exposure to polyvinyl chloride. The autopsy examination of the cardiac angiosarcoma characterized macroscopically by a right atrial tumor and histologically by anastomosing vascular channels and minute atypical pleomorphic endothelial cells. Immunohistological stainings were positive CD31, CD34 and focal positive factor VII-related antigen. Clinical and pathologic features with briefly reviewed relevant literatures are discussed This is the first reported description in the literature of a primary cardiac angiosarcoma with systemic metastases to multiple organs in Thailand.
血管肉瘤是一种相对罕见的起源于内皮细胞的原发性心脏恶性肿瘤。作者报告了一例46岁男性右心房原发性心脏血管肉瘤，伴有肺、肝、小脑和骨转移，临床表现为肺出血。该患者职业史中有偶尔接触聚氯乙烯的情况。心脏血管肉瘤的尸检检查在宏观上表现为右心房肿瘤，组织学上表现为相互吻合的血管通道和微小的非典型多形性内皮细胞。免疫组织化学染色显示CD31、CD34阳性，因子VII相关抗原局灶性阳性。本文讨论了临床和病理特征，并简要回顾了相关文献。这是泰国文献中首次报道的原发性心脏血管肉瘤伴有全身多器官转移的病例。
