儿童粒细胞肉瘤诊治分析  

Diagnosis and treatment studies on childhood granulocytic sarcoma

作　　者：李军[1] 翟晓文[1] 陈莲[2] 王宏胜[1] 苗慧[1] 钱晓文[1] 

机构地区：[1]复旦大学附属儿科医院血液肿瘤科,上海201102 [2]复旦大学附属儿科医院病理科,上海201102

出　　处：《中国小儿血液与肿瘤杂志》2014年第5期241-245,共5页Journal of China Pediatric Blood and Cancer

摘　　要：目的探讨儿童粒细胞肉瘤(GS)的临床表现、病理组织学及免疫组化特点、治疗及预后。方法收集2004年1月-2014年1月,复旦大学附属儿科医院血液科收治的6例GS患儿资料,对其临床表现、诊断、治疗及预后进行分析。结果 6例GS患儿均累及软组织、淋巴结、椎体、腹腔脏器等多个部位。4例诊断为原发性GS;2例同时伴骨髓浸润,骨髓形态及免疫分型均提示急性髓细胞白血病(AML)。5例患儿接受了联合化疗,目前2例CR(分别持续44个月、3个月),2例CR后复发,1例诱导治疗无效,复发与无效者均已放弃治疗。结论 GS临床罕见,临床表现多样,细胞形态学检查及免疫组织化学检查对其准确诊断非常必要。针对AML的化疗方案仍是首选的治疗方案。预后相对较差,难治复发性GS的治疗选择仍是难点。Objective To study the clinical characteristics,pathologic features,diagnosis and treatment as well as prognosis of childhood granulocytic sarcoma（GS）. Methods From January 2004 through January 2014,six cases diagnosed as GS in our hospital were included in this study. Clinical manifestations,histopathological characteristics,diagnosis and therapy protocol were studied. Results Four patients were diagnosed as primary GS,two out of four cases occurred concurrently with AML. The most primary sites of GS are soft tissue, lymph node, vertebral body and abdominal viscera.Myeloperoxidase（6 /6,100%） was the most sensitive and specific immunohistochemical marker. Five patients were treated with systemic chemotherapy two of them received tumor resection before chemotherapy,one reached CR then went to next course,one patient got sustained remission after chemotherapy. Two patients relapsed after remission,One patient＇s induction therapy was unresponsive.Conclusions GS is a rare disease with diverse clinical manifestations,the prognosis is generally poor.Right diagnosis of GS is very much detailed morphological and immunohistochemical study dependent.Anti-AML chemotherapy is still the first line treatment. The treatment for refractory and relapsed GS is still difficult.

关 键 词：粒细胞肉瘤 儿童 免疫组化 诊断 治疗 

分 类 号：R733.71[医药卫生—肿瘤]