Orbital tumor in a child with post-transplant lymphoproliferative disorder

Purpose: Post-transplant lymphoproliferative disorder (PTLD) is a condition characterized by B cell tumors that arise in organ transplant recipients. PTLD represents a major cause of morbidity and mortality. We report an orbital tumor in a four year old liver transplant recipient with PTLD. Method: The patient's medical records were reviewed, including ophlhalmologic and systemic examinations as well as results of radiologic and pathologic studies. Results: The patient presented with a rapidly enlarging left superior orbital tumor. Computed tomography revealed that the lesion was eroding the frontal bone. Magnetic resonance imaging revealed extension of the mass into the subarachnoid space. The tumor produced a superotemporal indentation of the globe and posterior choroidal folds. An anterior orbitotomy and incisional biopsy were performed. Pathological analysis revealed large atypical lymphoid cells consistent with PTLD. The patient was treated with chemotherapy and reduction of immunosuppression. The orbital lesion and retinal abnormalities resolved over the following month. Conclusion: To our knowledge, this is the first reported case of an orbital tumor in PTLD. The patient was immunosuppressed when she presented. Her tumor responded rapidly to chemotherapy and reduction of immunosuppression. Ophthalmologists should be aware of PTLD and its potential for ocular involvement.