原发性纵隔粒细胞肉瘤的诊治

The diagnosis and treatment of primary mediastinal granulocytic sarcoma

目的提高对纵隔粒细胞肉瘤(granulocytic sarcomas,GS)的临床病理特征、诊断、治疗的认识。方法回顾性总结我院确诊的1例纵隔GS的诊治资料,并结合近30年的相关文献进行分析。结果 GS好发于骨、神经系统、淋巴结、皮肤、小肠、宫颈、卵巢等部位,发生在纵隔的非常罕见,在临床上与胸腺瘤、淋巴瘤难以鉴别,在光镜下以弥漫小细胞浸润为主,易误诊为淋巴瘤,免疫组化显示髓过氧化物酶(MPO)、抗溶菌酶抗体染色(Lysozyme)、CD43、CD68等阳性有利于本病的诊断。治疗上以化疗为主,化疗方案以应用急性非淋巴细胞性白血病化疗方案为佳。结论临床医师应了解纵隔GS的临床病理特征,对于不能明确诊断的纵隔肿瘤应重视穿刺活检的重要性,以明确诊断,进而制定正确的治疗方案,免疫组化对于明确诊断有重要意义。

Objective To improve the diagnosis and differential diagnosis of primary mediastinal granulocytic sarcoma（GS）.Methods The clinico-pathological and radiological features of a patient with primary mediastinal GS were presented.The diagnosis and treatment were analyzed in conjunction with reviewing related literature in recent 30 years.Results GS usually occurred in the bones,the nervous system,lymph nodes,skin,intestine,cervix,ovaries and other parts.The primary mediastinal GS was rare.It was difficult to be identified and was often misdiagnosed as malignant thymoma or lymphoma.It showed diffuse small cells under the microscope and was apt to be misdiagnosed as lymphoma.Myeloperoxidase（MPO）,anti-lysozyme staining,CD43 and CD68 showing positive in immunohistochemical examination would be helpful to diagnosis of GS.The main treatment was chemotherapy based on acute non-lymphocytic leukemia chemotherapy regimen.Conclusion Clinicians should pay attention to the clinical and pathological characteristics of primary mediastinal GS.If the diagnosis of mediastinal tumors can not be determined,biopsy can help to confirm the diagnosis and direct the right treatment.Immunohistochemistry is of great significance for the diagnosis of GS.