Diffuse large B cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma, comprising about 30–40% of all cases world-wide. It is Characterised with a rapidly growing tumour mass in single or multiple, nodal or extra-nodal sites, with not otherwise specified as the most common subtype, which represents 80–85% of all cases [5]. 5th edition of the World Health Organization Classification of Haematolymphoid Tumours in 2022 focused on lymphoid neoplasms had modified the name from “diffuse large B-cell lymphoma “to “large B-cell lymphoma“, acknowledging the fact that a diffuse growth pattern is either not present or cannot be assessed in some entities (e.g., fibrin-associated large B-cell lymphoma or fluid-overload associated large B-cell lymphoma) [6]. DLBCL is a disease of elderly population with a median age in the 7th decade and with rare occurrence among young adults and children. A slight male predominance is also seen in the condition. DLBCL present in localized manner in approximately 20% of patients. Disseminated extra-nodal disease is uncommon, and one third of patients presents with systemic symptoms. Even though DLBCL are aggressive, they are potentially curable malignancies [7]. Primary Gastrointestinal (GI) lymphoma is a rare clinically nonspecific entity which are difficult to distinguish from other benign and malignant GI lesions. Primary GI Lymphoma can involve any part of the GI tract, frequent sites in order of its occurrence are the stomach followed by small intestine and ileocecal region [3]. Oesophageal involvement has been reported in less than 1% of cases and mostly occurs as an extension of mediastinal or gastric lymphoma. Primary oesophageal B Cell lymphoma is extremely rare with very few case reports in the literature [8]. Even though our case satisfies the Dawson’s criteria, strict application of Dawson’s criteria is controversial in different clinical settings.

Clinical diagnosis of primary oesophageal lymphoma is extremely difficult with variable clinical symptoms, imaging, and upper GI endoscopy findings. It is hard to prove the disease with a biopsy due to its submucosal location of the tumor. Clinical manifestation can vary from a range variable presentation like protruding a mass, submucosal nodule, ulcer, thickening etc [9]. Introduction of EUS guided biopsy has made great progress in the diagnostic accuracy of oesophageal lymphomas [10] Contrast enhanced CT of thorax and abdomen may help in differentiating primary oesophageal lymphoma from lymph node involvements in the cervical or mediastinal regions, in staging of the disease, ruling out complications and in evaluating treatment therapy [11].

The imaging modality of choice for staging and follow-up in Hodgkin disease and most non-Hodgkin lymphomas is 2-[fluorine-18] Fluoro-2-Deoxy-d-Glucose (FDG) Positron Emission Tomography (PET) scan. Considering GI lymphoma, FDG uptake is different according to the different location with  oesophageal lymphoma manifests as circumferential thickening of the oesophageal wall with increased FDG uptake [12].

To confirm the diagnosis of DLBCL, one must confirm the B-cell lineage of the lymphoma cells, with expression of CD20. In untreated lymphomas, CD20 is expressed in the most mature B-cell neoplasms, with some notable exceptions. Monoclonal antibody therapy (rituximab) targets CD-20 which is present in most B-cell lymphomas. Rituximab-treated B-cell lymphomas may not express CD20, which requires additional immune-stains, like PAX5, CD79a, CD19, or CD22, to identify B cells [13].

Surgical intervention may be warranted in case of an oesophageal lymphoma in the background of obstruction, bleeding, perforation, or diagnostic dilemma. Minor stenosis can be managed with endoscopic dilation [14]. Chemotherapy and radiotherapy are the mainstay of treatment with very favourable outcome depending on the type of the lymphoma especially the CHOP regimen. Addition of rituximab with CHOP regimen have shown a higher treatment response rate and better outcome for DLBCL patients [15]. External beam radiation at the dose of 40 Gy can also be used in the management of oesophageal lymphoma [16].  Post operative chemotherapy, radiotherapy or both can increase the survival and reduce recurrence. Early stage tumors, without lymphadenopathy, low and intermediate grade, size <10 cm with complete excision can be observed without adjuvant therapy [17]. Extra nodal DLBCL tends to be localised to the anatomical site, hence prognostically more favorable than nodal equivalents.