Primary T-cell immunoblastic lymphoma of the orbit in a pediatric patient

The authors report a case of an 8-year-old pediatric patient with a 2-week history of painless periorbital swelling unresponsive to antibiotic treatment.

Computed tomography (CT) showed a large, lateral, anterior left orbital soft tissue mass with bony erosion into the anterior cranial fossa through the roof of the orbit laterally. Surgical exploration showed a hard white mass that had eroded through the roof of the left orbit and into the anterior cranial fossa, with herniation of the brain and associated dura through the defect. Results of a complete evaluation of the child for systemic lymphoma, including a lumbar puncture, chest x-ray, bone scan, bone marrow aspirate, and chest/abdomen CT, were negative.

Results of histopathologic and immuno-histochemical evaluation showed a primary orbital T-cell immunoblastic lymphoma. The patient was treated with intrathecal ara-C (Cytosar-U) and methotrexate, 16.2 Gy of whole brain irradiation, and a chemotherapeutic protocol consisting of cyclophosphamide (Cytoxin), vincristine (Oncovin), methotrexate, daunomycin, and prednisone. The patient remains free of lymphoma 33 months after diagnosis, with 20/20 visual acuity in both eyes.

The authors believe that this is the youngest documented case of a primary T-cell immunoblastic lymphoma of the orbit.