Primary Esophageal Diffuse Large B-Cell Lymphoma With Barrettʼs Esophagus: A Case Report and Literature Review

Abstract

Diffuse large B-cell lymphoma (DLBCL) is the most common type of NHL in United States and accounts for 40% of NHL worldwide. GI lymphomas are rare and primarily located in the stomach, small intestine, and colon with esophageal being the rarest location. We present a rare case of rapidly growing esophageal lymphoma arising in the background of long segment Barrett's esophagus. A 76-year-old Caucasian male presented to our facility for poor oral intake and weight loss of 40 pounds in four months. Abdominal CT demonstrated circumferential distal esophageal wall thickening (Figure 1). EGD demonstrated erythematous and circumferentially nodular mucosa in the distal esophagus.1739_A Figure 1. CT scan showing distal esophageal thickening.Biopsies revealed Barrett's esophagus with erosive esophagitis but was negative for malignancy. Esophageal brushings showed candida esophagus. A 3-week course of fluconazole was prescribed. Repeat EGD in 4 weeks demonstrated salmon colored mucosa without nodules. Distal esophagus had ulcerated, granular and friable mucosa covered with exudates. (Figure 2). Biopsies from this region returned positive for high grade B-Cell lymphoma with C-MYC and BCL-2 rearrangements. Immunoperoxidase studies showed neoplastic B cells positive for CD20, PAX-5, BCL-6 and CD10. C-MYC was positive in 60% cells and KI-67 positive in 80% cells. PET scan demonstrated marked uptake in the distal esophagus without any evidence of regional or distal metastatic disease. Bendamustine and rituximab was initiated for treatment of diffuse large B cell lymphoma. The patient had an uneventful hospital course and was discharge home to continue chemotherapy. He continues to do well on follow up clinic visit.1739_B Figure 2. Distal Esophagus: Ulcerated, granular and friable mucosa with exudatesGastrointestinal lymphoma accounts less than 20% of extra nodal lymphomas with primary esophageal lymphoma accounting for less than 1% of GI lymphomas. Our patient satisfies Dawson's criterion of primary GI lymphoma which includes an absence of peripheral lymphadenopathy, absence of mediastinal adenopathy, lack of liver or spleen involvement, and normal peripheral blood count. Primary esophageal lymphomas are most commonly found in the distal esophagus, may present with dysphagia, weight loss, odynophagia or symptoms of upper gastrointestinal bleeding. To our knowledge this is the first reported case of esophageal lymphoma arising in background of long segment Barrett's esophagus. We hope that our description of this patient's esophageal DLBCL contributes to the limited data available.