S2051 Primary Esophageal Lymphoma or Lymphoma Presenting as an Esophageal Mass: Rare Disease or Rare Presentation of a Disease?

Abstract

Introduction: GI tract is the most common site of extra nodal non Hodgkin lymphoma (NHL) with the usual sites being stomach, small intestine and colon. Esophageal involvement has been reported in < 1% of cases and mostly occurs as an extension of mediastinal or gastric lymphoma. Here, we present a case of diffuse large B cell lymphoma (DLBCL) presenting with obstructive esophageal symptoms and diagnosed on esophageal ulcer biopsy. Case Description/Methods: A 72-year-old White male with history of 84 pack years smoking, GERD, presented with a month of shortness of breath, dysphagia to solids, odynophagia, loss of appetite, and 20 lbs. weight loss. Labs revealed WBC count 7000/µL. CT chest without contrast showed a large mediastinal mass with hilar extension, causing esophageal obstruction and two masses within the right lobe of liver. Endoscopy showed a mid-esophageal ulcer without bleeding, and atypical lymphoid cells on biopsy. Immunohistochemistry was positive for CD20, PAX5, BCL2 and BCL6, MUM1 and negative for CD10, cyclinD1 with MIB1 90%, thus consistent with DLBCL. Biopsy of liver mass showed similar histology and immunohistochemistry. HIV testing was negative. He was started on EPOCH based chemotherapy for the DLBCL. On chart review, CT Lung screening a year ago showed several filling defects in mid esophagus, however the filling defects were not evaluated as he was lost to follow up. Discussion: Primary esophageal DLBCL is a rare NHL with only handful of cases reported in literature to date. Our patient did not satisfy Dawson’s criteria for diagnosis of primary GI lymphoma which requires absence of peripheral and mediastinal lymphadenopathy, lack of liver and spleen involvement, and normal peripheral blood count. However, the utility of Dawson’s criteria for diagnosis of primary esophageal lymphoma has been questioned. Esophagus is a mediastinal structure and has rich lymphatic network, thus, predisposing to early mediastinal lymph nodes involvement. Also, esophageal cancers have been known to spread to liver via venous channels. Our patient had several mid esophageal filling defects a year ago and now presented with a large mediastinal mass encasing esophagus, thus, supporting the diagnosis of primary esophageal lymphoma. This case highlights the importance of endoscopic evaluation of esophageal filling defects seen on imaging.Figure 1.: Low dose CT lung screening performed a year prior shows filling defects in mid esophagus (red arrow) as seen in (A) axial section and (B) sagittal section. (C) CT chest without contrast on presentation showing large mediastinal mass, at the same level as image A causing esophageal obliteration. (D) Endoscopic biopsy of esophageal ulcer showing atypical lymphoid cells.