Abstract
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.
Highlights
Primary esophageal non-Hodgkin’s lymphoma is an uncommon gastrointestinal pathology that was first reported by Berman and colleagues in 1979.1 The incidence of this tumor is so infinitesimal that less than 30 cases have been described far.[2]
Different MeSH (Medical Subject Headings) terminologies, including “primary,” “diffuse large B-cell lymphoma,” and “esophagus,” were combined using the Boolean operators “AND” and “OR” with the terms “DLBCL,” “primary esophageal lymphoma,” and “treatment.” a few articles were retrieved through a manual search using the reference list of all accessible publications
The study inclusion criteria for the final comparative analysis comprised of the characteristics, 1) articles describing primary esophageal DLBCL, 2) availability of content in the full-text form, and 3) documentation of study findings in the English language
Summary
Primary esophageal non-Hodgkin’s lymphoma is an uncommon gastrointestinal pathology that was first reported by Berman and colleagues in 1979.1 The incidence of this tumor is so infinitesimal that less than 30 cases have been described far.[2] In one study, the incidence was only 0.2%, which was accounted by 3 cases of the total 1467 cases of primary gastrointestinal lymphoma.[3] Esophageal involvement in lymphoma is predominantly due to the local invasion from gastric or mediastinal lesions and represents
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