Burkitt lymphoma presenting as a conjunctival mass.

Abstract

A sporadic Burkitt lymphoma, which presented as a primary conjunctival mass, demonstrates the approach to clinical diagnosis, staging, and current management of this rapidly growing malignant tumor, as well as newer concepts of its pathogenesis. A 16-year-old girl had rapid development of a conjunctival mass 6 weeks after an infectious mononucleosis-like illness, associated with a positive monospot test. A biopsy of the tumor was immunostained for lymphocyte markers. Additional clinical workup included computed tomographic scanning, nasopharyngeal biopsy, and studies of blood, bone marrow, and spinal fluid. In situ hybridization for Epstein-Barr virus (EBV)-encoded nuclear RNA was performed on the tumor and adenoidal tissue. The conjunctival biopsy showed a typical Burkitt lymphoma with markers positive for predominantly B lymphocytes. Computed tomographic scans indicated residual tumor in the anterior orbit. Biopsy of thickened adenoidal tissue showed only benign lymphoid hyperplasia. Evidence of EBV infection was found in the adenoidal tissue but not in the tumor cells. The patient was treated with combined chemotherapy on a Pediatric Oncology Group Study protocol for localized non-Hodgkin lymphoma, and is disease-free 4 1/2 years after diagnosis. A sporadic Burkitt lymphoma arising in the conjunctiva appears to be the first reported in this site. The lymphoma responded well to chemotherapy, which is now the usual course in stage 1 disease. A direct role for EBV in the oncogenesis of this sporadic tumor, similar to that in the endemic form, could not be supported because EBV-encoded nuclear RNA was absent in the tumor cells.