原发性粒细胞肉瘤1例并文献分析

Primary granulocytic sarcoma: A case report and review of 87 cases in the literature of the late 20 years

目的:分析原发性粒细胞肉瘤(GS)的临床特征包括疾病的发病率,诊断流程,预后和治疗策略等。方法:对我院近期内确诊的1例原发于卵巢的GS和近20年MEDLINE文献关于原发性GS的资料87例进行总结分析。结果:分析资料完整的88例患者,其中65例确诊为原发性GS后转化为急性非淋巴细胞白血病(AN-LL),其余23例未发生转化目前仍在随访中。淋巴结,皮肤,泌尿生殖系统和消化系统是GS最常见的髓外发生部位;经典的GS肿瘤细胞形态具有不同阶段髓细胞分化的特点,免疫组化示MPO,NCAE(氯化醋酸AS-D萘酚酯酶),CD43和CD68等阳性;37例患者初诊时被误诊,大多数误诊为恶性淋巴瘤。传统的ANLL化疗方案疗效明显优越于恶性淋巴瘤方案和单独放疗。结论:减少原发性GS向ANLL转化并延长生存期,关键在于早期确诊和早期采用ANLL方案全身化疗。

Objective：The purpose of this study was to reveal the clinical characteristics of the primary granulo cytic sarcoma （GS）, including the frequency, diagnosis procedure, prognostic significance, and the treatment strategies, etc. Method： A case report from An Hui Provincial Hospital and clinical records of 87cases gathered by using a literature search on Medline from other institutions in the late 20 years were analyzed. Result： Total of 88 cases, including 65 patients who preceded acute nonlymphoblastic leukemia （ANLL） and 23 patients who did not develop to ANLL Lymphonode, skin, reproductive organs and digestive organs were the most common extramed ullary sites of involvement by primary GS. The classic morphologic features of the tumors were all stages of myeloid differentiation, immunohistochemistry features of （;S were positive of MPO, NCAE, CD43, and CD68 etc. Thirty-seven patients initially were misdiagnosed, and the disease was most often misdiagnosed as malignant lymphoma. The aggressive chemotherapy given to ANLL led to a longer nonleukemic period and better survival than the chemotherapy used for malignant lymphoma or the radiotherapy alone. Conclusion.. To reduce the rise of subsequent ANLL in patients with primary GS, and prolong the survival time, it is important to make an accurate histo logic diagnosis. All isolated GS patients, even those appear to be cured by resection or irradiation of the tumor, should be treated with intensive chemotherapy similar to that used to treat ANLL during the nonleukemie period as soon as possible.