10例头颈部髓外浆细胞瘤的临床分析及文献复习

Extramedullary Plasmacytoma in the Head and Neck:a Report of 10 Cases and Literature Review

背景与目的:髓外浆细胞瘤(extramedullary plasmacytoma,EMP)为一种罕见肿瘤,主要发生在头颈部,约80%的EMP发生于上呼吸道及上消化道。在临床表现和病理学上,该肿瘤极易与头颈部常见的一些肿瘤相混淆。本研究通过回顾性分析,探讨头颈部髓外浆细胞瘤的临床特点及其诊断及治疗。方法:回顾分析我院1977年1月～2003年12月期间收治的10例头颈部髓外浆细胞瘤患者的资料,全组10例患者中,单纯手术治疗2例、手术加辅助放疗2例、根治性放疗5例(其中1例于放疗后行辅助化疗),1例未治疗。结果:临床表现主要表现为局部肿物及其引起的相应症状。本组患者所有病例均经病理学诊断为浆细胞瘤,3例行免疫组化检测IgG,1例为λ型,2例为κ型。6例检测过尿本周蛋白均为阴性;4例检测过M蛋白,均为阴性;4例骨髓穿刺均未见异常;4例全身骨X线检查均正常。末次随访2004年5月,随访率90%,6例无瘤生存的患者中有5例无瘤生存时间超过1年(其中3例超过15年);死亡3例;失访1例。结论:EMP的诊断主要依靠临床表现和病理;放疗和手术是治疗头颈部髓外浆细胞瘤的主要手段。

BACKGROUND & OBJECTIVE: Extramedullary plasmacytoma (EMP), a rare tumor, often appears in the head and neck region. About 80% of EMPs occur in the submucosa of the upper aerodigestive tract. On both clinical presentation and pathologic examination, these tumors may be confused with some common tumors in the head and neck. This study was to evaluate clinical feature, diagnosis, and treatment of EMP in the head and neck. METHODS: Clinical data of 10 consecutive patients with pathologically confirmed EMP in the head and neck, treated in our hospital from Jan. 1977 to Dec. 2003, were reviewed. Of the 10 patients, 2 underwent surgery alone, 2 underwent surgery plus adjuvant radiotherapy,4 received radical radiotherapy, 1 received radical radiotherapy plus adjuvant chemotherapy, and 1 gave up treatment. RESULTS: The patients mainly manifested as local masses and relevant symptoms. Of the 3 patients received immunohistochemical staining to detect IgG, 1 was λ type, and 2 were κ type. Six patients received Bence-Jones protein detection, and 4 patients received serum myeloma protein detection; all of them showed negative results. Bone marrow biopsy was made in 4 patients, skeletal survey was made in 4 patients; all results were normal. By the end of follow-up (Mar. 2003), 6 patients were still alive (5 patients survived for more than 1 year, 3 of the 5 patients survived disease-freely for more than 15 years); 1 died of local recurrence 2 years after diagnosis; 1 died of multiple myeloma; 1 died of heart disease before treatment; 1 lost follow-up. CONCLUSIONS: The diagnosis of EMP mainly depends on clinical manifestation and pathologic results. Radiotherapy and surgery are the main treatments for EMP in the head and neck.