粒细胞肉瘤38例临床病理分析

Clinicopathologic features of granulocytic sarcoma： a study of 38 cases

目的探讨粒细胞肉瘤的临床病理特征及鉴别诊断。方法对38例粒细胞肉瘤患者的病理组织进行HE及免疫组织化学（EnVision法）染色观察，结合临床资料进行分析，并复习相关文献。结果患者发病年龄2～77岁，平均年龄43．3岁，男23例，女15例。临床主要表现为体表淋巴结肿大、局部软组织肿块及疼痛。其中随访18例，死亡14例，平均生存时间16．9个月。组织学表现为肿瘤细胞弥漫成片，小～中等大小，形态较一致，胞质少而淡染，核呈圆形或不规则形，可见核仁，核分裂象易见。部分肿瘤具有“列兵样”组织结构，肿瘤细胞间可见散在分布的幼稚嗜酸性粒细胞。免疫组织化学染色显示瘤细胞表达抗髓过氧化物酶、CD43，部分表达CD68、溶菌酶、CD99和末端脱氧核苷酸转移酶，而CD3、CD20、CD79a、AE1／AE3和胎盘碱性磷酸酶阴性。结论粒细胞肉瘤临床少见，形态学上易误诊为非霍奇金淋巴瘤、Ewing肉瘤／PNET、胚胎性横纹肌肉瘤等，免疫组织化学技术对于确诊本病具有重要价值。

Objective To study the clinicopathologic features of granulocytic sarcoma. Methods The clinical and pathologic findings of 38 cases of granulocytic sarcoma were retrospectively analyzed. Immunohistoehemical study was performed and the literature was reviewed. Results The age of patients ranged from 2 to 77 years （ mean = 43.3 years）. The male-to-female ratio was 1.5 ： 1. Major clinical presentations included superficial lymph node enlargement and painful soft tissue mass. Follow-up data were available in 18 patients; and 14 of them died of tumor-related diseases. The average duration of survival of the patients was 16. 9 months. Histologically, the tumor cells were relatively uniform in appearance and small to medium in size. The cytoplasm was scanty and pale in color. The nuclei were round or focally irregular, with fine chromatin and inconspicuous nucleoli. Mitosis figures were readily identified. Scattered immature eosinophilic myelocytes were seen. Immunohistochemical study showed that the tumor cells in all cases expressed MPO and CD43. Most cases were also positive for CD68, lysozyme, CD99 and TdT. The staining for CD3, CD20, CD79a, pan-cytokeratin and PLAP were negative. Conclusions Granulocytic sarcoma is a known histologic mimicker of non-Hodgkin lymphoma, Ewing sarcoma/PNET and embryonal rhabdomyosarcoma. Detailed morphologic examination, when coupled with immunohistochemical study, is useful in arriving at a correct diagnosis.