S4278 A Unique Case of Epstein-Barr Virus-Positive Mucocutaneous Ulcer Presenting as Progressive Dysphagia and an Esophageal Mass

Introduction: Epstein-Barr virus-positive mucocutaneous ulcer (EBVMCU) is a rare form of EBV-associated lymphoproliferative disease that can occur within the gastrointestinal tract. Here, we present a unique case of EBVMCU presenting as rapidly progressive dysphagia and an esophageal mass. Case Description/Methods: A 60-year-old woman with a past medical history of bipolar disorder, Hashimoto’s thyroiditis presented for a 10-month history of progressive dysphagia. Symptoms included dysphagia to solids progressing to liquids, daily induced emesis of retained food, and a 15-pound weight loss. Computed tomography showed long-segment esophageal thickening. Esophagogastroduodenoscopy showed a large, fungating and ulcerating mass that encompassed 2/3 of the lumen circumference in the mid to distal esophagus and the gastroesophageal junction with >50% obstruction of the esophageal lumen. Pathology of the mass revealed fragments of esophageal squamous mucosa with sub-epithelial infiltration by large, irregular, hyperchromatic lymphoid cells, positive for CD20, CD30, PAX5, and MUM1 on immunohistochemistry, felt to be consistent with EBVMCU. Positron emission tomography scan showed disease limited to esophagus. Given the degree of her symptoms, the patient was treated with rituximab followed by radiation with only partial response. She had persistent symptoms and ongoing disease endoscopically and on positron emission tomography prompting treatment with the combination chemotherapy RCHOP. Subsequent esophagogastroduodenoscopy after completion of RCHOP showed esophageal stenoses with pathology negative for EBVMCU. Patient’s esophageal stenoses were managed with placement of fully covered metal stents and she remains under surveillance with hematology. Discussion: EBVMCU is a rare lymphoproliferative disorder that was first described in 2010. It is generally seen in immunocompromised patients, but it is also seen in older patients without overt immunodeficiencies due to the immunosenescence of aging. It is most commonly seen in the oropharyngeal mucosa, skin, or the gastrointestinal tract. Careful review of pathology and imaging is required to distinguish EBVMCU from diffuse large B-cell lymphoma and Hodgkin lymphoma. EBVMCU is generally indolent and can spontaneously regress. However, for patients with significant symptoms treatment options include rituximab, radiation, surgical excision, and RCHOP. Gastroenterologists should consider EBVMCU when evaluating patients with ulcerated esophageal tumors.