Posttransplant lymphoproliferative disorder presenting in the head and neck

Objective: To determine the incidence of focal head and neck posttransplant lymphoproliferative disorder (PTLD) in children, its clinical presentation, associated risk factors, and outcome following treatment. Study Design: Retrospective. Methods: The authors conducted a 5-year retrospective study of 61 children with liver transplants at the University of California, San Francisco. Suspected head and neck lesions were evaluated and biopsies were performed by an otolaryngologist. Diagnosis was made via histologic and immunohistochemical features and in situ Epstein-Barr virus (EBV) localization. Results: Eight patients (13.1%) developed PTLD, five (8.2%) in the head and neck. Four patients had large tonsils, and one presented with airway obstruction from a supraglottic mass. Negative pretransplant EBV serology was a risk factor for PTLD. Treatment consisted of antiviral therapy and decreased immunosuppression. All patients with head and neck PTLD are disease free with excellent liver function. Conclusions: A high incidence of PTLD was found, with 63% presenting in the head and neck. While Waldeyer's ring is most commonly involved, PTLD may also present in the supraglottis. Adjunctive antiviral therapy and decreased immunosuppression are effective forms of treatment. Given the increasing number of pediatric liver transplants being performed, otolaryngologists should be familiar with PTLD and have a high index of suspicion in this at-risk population.