Primary Oesophageal Lymphoma: A Diagnostic Dilemma

Primary oesophageal lymphoma is an exceptionally rare cancer originating in the esophagus, distinct from more common oesophageal malignancies. Dawson's criteria has been the mainstay for diagnosis for such entities. However, recognizing primary oesophageal lymphoma is particularly challenging due to its rarity, diverse clinical presentations, and non-specific radiological and endoscopic features. These factors often lead to diagnostic delays, complicating timely treatment and resulting in poor patient outcomes. We hereby present a challenging case and literature review about this rare malignancy. A 78-year-old male was referred to Gastroenterology for evaluation of dysphagia. Initial esophagogastroduodenoscopy (OGD) revealed an oesophageal ulcer with features consistent with oesophageal candidiasis. A follow-up OGD identified an oesophageal stricture. Histopathology and immunohistochemistry of biopsy samples confirmed a diagnosis of diffuse large B-cell lymphoma (DLBCL). A staging CT was completed, and an oesophageal mass was shown extending into the mediastinum. The patient re-presented with worsening dysphagia, renal dysfunction, and hypercalcemia. Although lymphoma was confirmed, to ensure an appropriate regimen is commenced, a repeat OGD was recommended by the Hematology team. The patient's rapid deterioration necessitated pre-phase steroid treatment and management of tumor lysis syndrome. Before R-CHOP chemotherapy could begin, the patient deteriorated further. Following a multidisciplinary team (MDT) meeting and discussions with the patient and family, it was decided to manage the symptoms with palliative intent. This case highlights the diagnostic challenges posed by primary oesophageal lymphoma, emphasizing the importance of considering this rare malignancy in the differential diagnosis of oesophageal strictures, particularly in elderly patients. Aggressive disease progression complicates managing DLBCL in older patients. Moreover, this patient population has other co-morbidities that would preclude treatment options at the outset. Further studies are needed to establish optimal diagnostic and therapeutic strategies for this rare condition.