Inayat Faisal, Munir Ahmed, Wahab Ahsan, Younus Fariha, Zafar Fahad, Ullah Waqas
1 Allama Iqbal Medical College, Lahore, Pakistan.
2 Services Institute of Medical Sciences, Lahore, Pakistan.
J Investig Med High Impact Case Rep. 2018 Jan-Dec;6:2324709618820887. doi: 10.1177/2324709618820887.
Primary esophageal diffuse large B-cell lymphoma (DLBCL) is an extremely rare clinicopathologic entity. We report one case from our clinical experience and undertake a review of the previously published cases. A systematic literature search of the medical databases PubMed and Google Scholar was conducted. A total of 15 cases fulfilled the inclusion criteria. The data on patients' characteristics, epidemiology, clinical features, HIV status, gross appearance of the lesion, esophageal location, treatment, and outcome were collected and analyzed. Primary esophageal DLBCL was more common among males, primarily in the fifth and sixth decades of life. Dysphagia was the most common initial clinical presentation. Tissue biopsy with immunohistochemistry was an indispensable diagnostic modality. The mainstay of treatment was chemotherapy with cyclophosphamide, vincristine, doxorubicin, and prednisone regimen, in addition to anti-CD20 antibody rituximab, with or without radiotherapy. This review serves to outline our current understanding of the epidemiology of and risk factors for primary esophageal DLBCL, the pathophysiology of this disorder, and currently available approaches to diagnosis and management.
原发性食管弥漫性大B细胞淋巴瘤（DLBCL）是一种极其罕见的临床病理实体。我们报告一例来自我们临床经验的病例，并对先前发表的病例进行综述。我们对医学数据库PubMed和谷歌学术进行了系统的文献检索。共有15例符合纳入标准。收集并分析了患者特征、流行病学、临床特征、HIV状态、病变大体外观、食管位置、治疗及预后等数据。原发性食管DLBCL在男性中更为常见，主要发生在50和60岁年龄段。吞咽困难是最常见的初始临床表现。组织活检及免疫组化是不可或缺的诊断方法。除抗CD20抗体利妥昔单抗外，治疗的主要手段是采用环磷酰胺、长春新碱、多柔比星和泼尼松方案进行化疗，可联合或不联合放疗。本综述旨在概述我们目前对原发性食管DLBCL的流行病学和危险因素、该疾病的病理生理学以及目前可用的诊断和管理方法的理解。
