Esophageal squamous papillomatosis is rare and has been associated with gastroesophageal reflux and recurrent respiratory papillomatosis. We report a case of extensive esophageal papillomatosis, no airway involvement and a slowly progressive clinical course with progressive strictures and ultimately fatal squamous cell carcinoma. In-situ hybridization performed on biopsy specimens was negative for high-risk human papilloma virus types. Due to the paucity of reported cases, little is conclusively known about the etiology, natural course and best clinical management of this disease. Human papilloma virus has been linked to some, but not all, cases, and the clinical course has been reported to vary from spontaneous regression to malignant transformation. Surveillance for malignancy by conventional endoscopic biopsies or computed tomography scan appears to have low sensitivity. This case illustrates the difficulties in clinical management and establishing a definite etiology in esophageal squamous papillomatosis.
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