【作者】 毛伟芳； 劳伟峰； 陈琳琳； 朱洪波； 宋章法； 黄学锋； 王达； 林建江；

【Author】 Mao Wei-Fang M.D. Lao Wei-Feng M.D. Chen Lin-Lin M.D. Zhu Hong-Bo M.D. Song Zhang-Fa M.D. Huang Xue-Feng M.D. Wang Da M.D. Lin Jian-Jiang M.D. Department of Clorectal Surgery, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, 310016,China Department of Clorectal Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, 310003,China

【机构】 浙江大学附属邵逸夫医院肛肠外科； 浙江大学附属第一医院肛肠外科；

【摘要】 目的:孤立性髓外浆细胞瘤(Solitary Extramedul— fary Plasmacytoma SEP)是一种少见肿瘤,占浆细胞肿瘤的4％左右。80％发生于头颈部,出现在直肠肛门罕见报道。本研究主要探讨直肠肛门部髓外浆细胞瘤的临床特点及其诊断和治疗。方法与结果:4例直肠肛门部SEP均为女性,年龄在31岁-57岁。临床症状:反复便血1～4个月,颜色鲜红。肿瘤均位于距肛4cm以下,呈肿块型或结节状。其中肿块最大约3．0cmx4．0cm大小。入院诊断2例直肠癌、1例肛门结节、 1例直肠溃疡伴肛乳头肥大。3例术前活检病理,2例直肠浆细胞瘤,1例肛门炎性结节(浆细胞浸润)。1例免疫球蛋白偏高:lgA:507mg／dl,LAM:1010mg／dl,B2-Mg0．46mg／ dl,手术2个月后正常。另外三例免疫球蛋白均正常。骨骼X 摄片、磁共振、骨髓穿刺、血常规、血生化等检查均正常。尿本周蛋白均阴性。3例ECT检查未见异常。治疗:1例行腹会阴联合切除术,3例行经肛局部切除术。手术后病理报告:直肠浆细胞瘤。免疫组化:2例κ(+)、λ(-)。2例κ(-)、λ(+)。4例肿瘤细胞PC(+),CD138(+),CD79(+),CD20(-)。4 例病人目前均健在。无局部复发,未发现有多发性骨髓瘤。随访最长为11年,最短为6个月。结论:孤立性髓外浆细胞瘤是一种少见肿瘤。直肠肛门部SEP的临床主要表现,反复无痛性便血,肛门指检触及肿块或结节,质地中等,边界欠清。肿块表面糜烂或溃疡。症状和体症很难与直肠癌相区别。病理学检查是SEP的诊断关键。手术和放疗是治疗直肠肛门部浆细胞瘤的主要手段。SEP预后较好。更多还原

【Abstract】 Objective: To present 4 cases of seldom SEP located in anorectum region and discuss the diagnostic and treatment strategy. Results: From 31 to 57 years old female were referred to our division. Their clinical characteristics were recurrent painless hematochezia for 1 to 4 months, palpable rectal mass with unclear margin 4cm or below to anus. The biggest one was 3 by 4cm. The admittable diagnoses were two rectal tumors, one anal mass and one rectal ulcer. Three patients were performed biopsy before operation, two were plasmacytoma and one was inflammatory tissue with plasma cell infiltration. Bone scan, skeletal survey and bone marrow aspirate biopsy were normal. No monoclonal paraprotein was identified in serum or urine, and there was no significative abnormality in chemistries of serum and urine. Four cases were all accepted operative therapy, three localregional resection and one Mile’ s procedure. Postoperative pathological diagnosises were all plasmacytoma. Two light chains showed κ ( +) and two λ (+) by immunofixation. They were all PC (+), CD138 (+), CD79 (+) and CD20 (-) in immunohistochemistry. At clinical follow-up 6 months to 11 years, the patients were asymptomatic and digital examination revealed no local recurrence. Conclusion: SEP of anorectum is an exceeding rare tumor. It presents as medium hard mass or ulcer with unclear margin, and normally has recurrent painless hematochezia. Invasive proliferation of monoclonal plasma cell is the determinant of a SEP diagnosis after excluding systemic involvement. Surgery and radiation therapy are undoubtedly the most common therapeutic choices. Although having good prognosis, patients with SEP are advised to be long-term surveilled because of its potential of transforming to myeloma.更多还原