Primary Esophageal Extranodal Marginal Zone Lymphoma: A Rare Tumor

Abstract Primary esophageal extranodal marginal zone lymphoma (ENMZL) is a rare and challenging malignancy to diagnose. A case involving a 66-year-old woman with a 6-month history of worsening dysphagia, odynophagia, and weight loss is presented. Imaging studies revealed a large mediastinal mass and a distal esophageal mass causing partial obstruction, with no evidence of enlarged lymph nodes. Despite biopsies from esophagoscopy and endoscopic ultrasound not providing a definitive diagnosis, the patient underwent surgical excision of the middle esophageal mass. Histopathological analysis confirmed primary ENMZL. The patient was subsequently referred for chemotherapy as stage I ENMZL. Treatment strategies may vary, typically involving a combination of chemotherapy and radiotherapy, with surgical options considered as well. Raising awareness of this rare tumor among medical professionals is vital for improving patient outcomes.