Orbital presentation of posttransplantation lymphoproliferative disorder: A small case series

Abstract Objective To describe a small series of patients with orbital presentation of posttransplantation lymphoproliferative disorder (PTLD). Design Retrospective, interventional case series. Participants Three patients with orbital presentation of histologically diagnosed PTLD. Methods Review of medical records. Main outcome measures Measured parameters included vision, proptosis, and tumor extent. Results Three cases of orbital PTLD are described. In two of the cases, the tumor initially demonstrated orbital signs and symptoms, whereas in the third case, orbital and systemic signs were synchronous. Two of three patients had disseminated disease discovered at the time of presentation. One adult patient had synchronous presentation of PTLD in the orbit and prostate. One pediatric patient had tumor dissemination to the liver at the time of presentation. The PTLD tumors were classified histologically as diffuse large cell lymphoma of monomorphic or immunoblastic type in all three cases. Treatment included local irradiation, decreased immunosuppression, and antilymphocyte monoclonal antibodies. Conclusions Orbital presentation is a rare manifestation of PTLD. However, ophthalmologists must consider this diagnosis carefully in organ transplant recipients with subtle orbital signs and symptoms at presentation. Early detection may alter prognosis. In each case presented, the diagnosis was established via lesion biopsy and subsequent histologic or flow cytometric evaluation, or both.