Post-Transplant Lymphoproliferative Disorder of the Temporal Bone: Report of a Unique Case and Discussion

Post-transplant lymphoproliferative disorder (PTLD) is a well-recognized but relatively rare complication of solid organ and allogeneic bone marrow transplantation. Tumors, whether localized or disseminated, are aggressive, rapidly progressive, and often fatal. The vast majority of these cases are associated with Epstein-Barr virus (EBV) infection of B-cells. Most cases occur within the first year after transplantation, and incidence is directly related to the intensity of the immunosuppressive regimen. Accordingly, the mainstay of treatment is reduction or withdrawal of immunosuppression in spite of the risk of allograft dysfunction or loss. A variety of additional treatment approaches, including surgical excision, radiation therapy, combination chemotherapy, monoclonal antibodies, interferon therapy, and the use of immunoglobulin and cytotoxic T lymphocytes, have also been described.

The majority of cases present with lymphadenopathy, and in the head and neck cervical lymphadenopathy and adenotonsillar hypertrophy are the most common presenting signs. Here we report a unique case of facial nerve paralysis of unknown etiology ultimately found to be secondary to post-transplant lymphoproliferative disorder of the temporal bone. To our knowledge, this is the first reported case of PTLD presenting with facial palsy, and just the second reported case of PTLD of the temporal bone. We then discuss management of this complicated lesion and review the literature regarding treatment outcomes.