原发性心横纹肌肉瘤临床病理观察

Clinicopathological features of cardiac primary rhabdomyosarcoma

目的探讨心横纹肌肉瘤的临床病理学特征及诊断、鉴别诊断要点。方法对1例心横纹肌肉瘤进行临床病理分析及免疫组化研究,并复习相关文献。结果镜下见瘤细胞弥漫生长,疏密不等,部分区域有黏液样基质,部分区域为红染基质。瘤细胞大部分为大圆形、短梭形,核深染,不均匀成块状。部分细胞分化较好,核分裂象易见,可见少量瘤巨细胞。肿瘤组织结节样突向心肌内。免疫组化：PAS和CD68（＋）、desmin、vimentin、CD68和CD117（＋＋＋）,MyoD1（＋＋）,CK、PCNA68%（＋）,myoglobin、S-100、p53和Ki-67（-）。结论原发性心横纹肌肉瘤罕见,恶性程度高、侵袭性强,组织形态学及临床表现有较大差异,免疫组化检测能增加诊断的正确性。

Objective To study the clinicopathological characteristics of cardiac primary rhabdomyosareoma and to elucidate its diagnosis and differential diagnosis. Methods Clinieopathological characteristics and immunohistochemical findings were analyzed in a case of cardiac primary rhabdomyosarcoma and related literatures were reviewed. Results The tumor was composed of big roundish and spindle cells with deep stained and pleemorphic nuclei. Tumor cells were heterogeneously diffuse. In some areas there was mucoid or pink stroma. Some cells were well differentiated and showed caryokinesis. Several egacaryocytes were visible. Nodular tumor tissue infiltrated into normal myocardium. Immunohistochemically, tumor cells were positive for PAS, S-100, desmin, vimentin, CD68, MyoD1, Ki-67 and myoglobin, and but negative for CK, CD117, and P53. 68% tumor cells were PCNA positive. Conclusions Cardiac primary rhabdomycsarcoma is rare, and high malignant and invasive. As its morphological and clinical features are heterogeneous, the staining can help in the diagnosis.