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Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue of the Salivary Glands-Clinical Experience in a Single Institution

唾液腺粘膜相關淋巴組織淋巴瘤

摘要


背景:唾液腺粘膜相關淋巴組織淋巴瘤是淋巴瘤中一個獨特的分類,常被描述為一個低度惡性且局限於局部的腫瘤。此疾病相當罕見,過去的研究多半是小型或零星的病例報告,且多半來自西方國家。目的及目標:藉由唾液腺粘膜相關淋巴組織淋巴瘤的系列病例,來分析此疾病的臨床特徵及預後表現。材料及方法:自台北榮總病理資料庫中,搜尋1990年至2013年間診斷為唾液腺粘膜相關淋巴組織淋巴瘤的病患。記錄其基本資料、臨床表現及治療方式。完整手術定義為依解剖位置的唾液腺切除且無殘留腫瘤。復發及存活由病歷紀錄或電話追蹤擷取,並統計分析。結果:研究期間,共十位病患診斷為唾液腺粘膜相關淋巴組織淋巴瘤。平均年紀50.9歲(29-69歲)。三位病患患有修格蘭氏症候群。依原發部位而分:六位腮腺,三位頷下腺,一位小唾液腺。追蹤期間,共有五位病患復發,中位復發時間為39.9個月(11-63月)。五年無復發存活率為53.3%.腫瘤惡性度、放射治療及化學治療對無復發存活期無影響。但完整手術似乎有較高的無復發存活期,雖然統計無顯著差異。(p=0.089)結論:唾液腺粘膜相關淋巴組織淋巴瘤擁有緩慢的進程但較高的復發率。完整的切除腫瘤也許跟低腫瘤復發率有所相關。

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並列摘要


Background: Extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) is a distinct type of lymphoma with unique clinicopathologic features. Salivary gland MALT lymphoma is rare and generally described as a low-grade neoplasm, as well as a tendency to remain localized. Available data in the literature are limited, confined to small series and isolated case reports, and most of them were from western countries. Aim and Objectives: The purpose of the study is to assess the clinical features and biological behaviors of patients with MALT lymphomas involving the salivary glands. Materials and Methods: The surgical pathology database of Taipei Veterans General Hospital for the years 1990 through 2013 were reviewed. All cases with a diagnosis of salivary gland MALT lymphoma were selected. Patient demographics, clinical presentation, treatment modality were recorded. Definitive surgery was defined as anatomical resection and free of residual tumor. Recurrence and survival were extracted from medical records and follow-up phone calls. The data were statistically analyzed. Results: Ten cases of primary salivary gland MALT lymphoma were collected. Their mean age was 50.9 years (range, 29-69 years). Three patients had documented Sjogren’s syndrome. Primary localizations included parotid gland in 6 patients, submandibular gland in 3 and minor salivary gland in 1. Throughout the follow-up, 5 patients developed recurrence. The median time of recurrence was 39.9 months (range, 11-63). 5-year recurrence free survival (RFS) was 53.3%. Tumor grading, radiotherapy and chemotherapy did not have a significant impact on RFS. For patients with localized and locally disseminated disease, definitive surgery seemed to lead to higher RFS, although the difference is not statistically significant (p = 0.089). Conclusion: MALT lymphomas of the salivary glands has indolent course but higher recurrence rate. Total excision of the tumor might be associated with lower recurrence rate.

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