Liu Tian-Run, Yang An-Kui, Chen Fu-Jin, Guo Gui-Fang, Wei Mao-Wen, Chen Wei-Chao, Zeng Zong-Yuan
Department of Head and Neck Oncology, Cancer Center, Sun Yat-sen University, Guangzhou, Guangdong 510060, P. R. China.
Ai Zheng. 2005 Jun;24(6):714-7.
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.
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.
The patients mainly manifested as local masses and relevant symptoms. Of the 3 patients received immunohistochemical staining to detect IgG, 1 was lambda type, and 2 were kappa 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.
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.
髓外浆细胞瘤(EMP)是一种罕见肿瘤,常出现在头颈部区域。约80%的EMP发生于上呼吸消化道黏膜下层。在临床表现和病理检查方面,这些肿瘤可能会与头颈部一些常见肿瘤相混淆。本研究旨在评估头颈部EMP的临床特征、诊断及治疗。
回顾性分析1977年1月至2003年12月在我院接受治疗的10例经病理确诊的头颈部EMP患者的临床资料。10例患者中,2例仅接受手术治疗,2例接受手术加辅助放疗,4例接受根治性放疗,1例接受根治性放疗加辅助化疗,1例放弃治疗。
患者主要表现为局部肿块及相关症状。3例行免疫组化染色检测IgG的患者中,1例为λ型,2例为κ型。6例患者进行了本周氏蛋白检测,4例患者进行了血清骨髓瘤蛋白检测;所有结果均为阴性。4例患者进行了骨髓活检,4例患者进行了骨骼检查;所有结果均正常。至随访结束(2003年3月),6例患者仍存活(5例存活超过1年,其中3例无病存活超过15年);1例诊断后2年死于局部复发;1例死于多发性骨髓瘤;1例在治疗前死于心脏病;1例失访。
EMP的诊断主要依靠临床表现和病理结果。放疗和手术是头颈部EMP的主要治疗方法。