Burke A P, Virmani R
Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000.
Am J Surg Pathol. 1991 Mar;15(3):289-95. doi: 10.1097/00000478-199103000-00009.
We reviewed nine primary cardiac sarcomas with osteosarcomatous differentiation. The patients' ages ranged from 24 to 67 (mean 38 years). All tumors were surgical specimens from the left atrium; many were clinically diagnosed as atypical myxomas. In eight cases complete excisions were attempted, one requiring reconstruction with grafting; one tumor was biopsied only. Two tumors extended into the pulmonary veins. Three patients died within 2 weeks after the initial surgery from postoperative complications; five patients had metastatic disease or died from disease; and one patient was lost to follow-up. Metastatic sites included lungs, thyroid, and skin. In addition to osteosarcoma, four tumors showed chondroid differentiation, three had osteoclastic cells, four had a prominent spindle cell component, and one had myxoid areas. All tumors showed immunohistochemical positivity for vimentin; stains for cytokeratin and desmin were negative. S-100 positivity was demonstrated in chondrosarcomatous areas of one tumor. We conclude that most cardiac osteosarcomas are clinically mistaken for myxomas because of location in the left atrium. They are larger, tend to infiltrate, and are very aggressive neoplasms. Histologically a variety of patterns may be encountered in addition to the osteosarcoma.
我们回顾了9例具有骨肉瘤分化的原发性心脏肉瘤。患者年龄在24至67岁之间（平均38岁）。所有肿瘤均为来自左心房的手术标本；许多在临床上被诊断为非典型黏液瘤。8例尝试进行了完整切除，1例需要植骨重建；1例仅进行了活检。2例肿瘤延伸至肺静脉。3例患者在初次手术后2周内因术后并发症死亡；5例患者有转移性疾病或因病死亡；1例患者失访。转移部位包括肺、甲状腺和皮肤。除骨肉瘤外，4例肿瘤显示软骨样分化，3例有破骨细胞，4例有显著的梭形细胞成分，1例有黏液样区域。所有肿瘤波形蛋白免疫组化均呈阳性；细胞角蛋白和结蛋白染色均为阴性。1例肿瘤的软骨肉瘤区域S-100呈阳性。我们得出结论，大多数心脏骨肉瘤由于位于左心房，临床上易被误诊为黏液瘤。它们体积较大，易于浸润，是极具侵袭性的肿瘤。组织学上，除骨肉瘤外还可见多种形态。
