Background: Anaplastic kinase-1 positive lymphoma (ALK-1) is a very rare but distinct pathologic entity. ALK-1 lymphoma tends to affect the bone marrow, skin, lungs, soft tissue, but very rarely the gastrointestinal tract.
Methods: We report a case of ALK-1 positive lymphoma presenting as a tracheoesophageal fistula. The clinical presentation, histopathology, and management outcome are all discussed.
Conclusion: ALK-1 positive lymphoma of the upper aerodigestive involvement is extremely rare. An unusual presentation such as pharyngeal pouch with vocal cord palsy should suggest pathology. Histopathologic analysis can be difficult and immunohistochemical studies will aid are of paramount importance in the diagnosis and help in subsequent management and also act as prognostic indicators.