Primary esophageal lymphoma: An individual participant data meta-analysis

Primary esophageal lymphoma represents a rare form of gastrointestinal lymphoma, comprising less than 1% of all lymphomas. This study aims to review the literature on primary esophageal lymphoma, examining its clinical features, diagnosis, treatment, prognosis, and biological behavior.

A systematic search of the literature in the PubMed/Medline and Scopus databases was conducted by 2 independent reviewers focusing on adult cases of primary esophageal lymphoma.

Of 56 studies meeting inclusion criteria, 69 cases of primary esophageal lymphoma (male-to-female ratio of 2.8:1, mean age of 55.49 ± 18.79 years) were analyzed. Dysphagia (68.1%) was the most common symptom, often with weight loss (26.1%), whereas 5 patients (7.2%) remained asymptomatic. Primary esophageal lymphoma predominantly affected the lower (37.7%) and middle (21.7%) esophagus. Chemotherapy was the primary treatment (52.1%), followed by surgical resection (23.1%). Median follow-up was 16.93 months with 1-, 3-, and 5-year survival rates of 84.5%, 72.3%, and 28.9%, respectively. Survival was not significantly influenced by sex, age, treatment, immunosuppression or lymphoma type. Local lymphadenopathy significantly correlated with survival.

Primary esophageal lymphoma is a rare and diagnostically challenging malignancy with limited evidence to guide clinical decision-making. Although chemotherapy remains the cornerstone of treatment, surgery plays an important role in the management of primary esophageal lymphoma in selected scenarios such as obstruction, perforation, or inconclusive biopsies. On the basis of limited and heterogeneous data, our findings suggest a notably lower survival rate compared with classical lymphomas, underscoring the importance of multidisciplinary collaboration to ensure appropriate surgical, oncologic, and supportive care in this rare disease.