BURKITT-LYMPHOMA PRESENTING AS A CONJUNCTIVAL MASS

Purpose: A sporadic Burkitt lymphoma, which presented as a primary con junctival mass, demonstrates the approach to clinical diagnosis, stagi ng, and current management of this rapidly growing malignant tumor, as well as newer concepts of its pathogenesis. Patient and Methods: A 16 -year-old girl had rapid development of a conjunctival mass 6 weeks af ter an infectious mononucleosis-like illness, associated with a positi ve monospot test. A biopsy of the tumor was immunostained for lymphocy te markers. Additional clinical workup included computed tomographic s canning, nasopharyngeal biopsy, and studies of blood, bone marrow, and spinal fluid. In situ hybridization for Epstein-Barr virus (EBV)-enco ded nuclear RNA was performed on the tumor and adenoidal tissue. Resul ts: The conjunctival biopsy showed a typical Burkitt lymphoma with mar kers positive for predominantly B lymphocytes. Computed tomographic sc ans indicated residual tumor in the anterior orbit. Biopsy of thickene d adenoidal tissue showed only benign lymphoid hyperplasia. Evidence o f EBV infection was found in the adenoidal tissue but not in the tumor cells. The patient was treated with combined chemotherapy on a Pediat ric Oncology Group Study protocol for localized non-Hodgkin lymphoma, and is disease-free 4 1/2 years after diagnosis. Conclusions: A sporad ic Burkitt lymphoma arising in the conjunctiva appears to be the first reported in this site. The lymphoma responded weft to chemotherapy, w hich 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 end emic form, could not be supported because EBV-encoded nuclear RNA was absent in the tumor cells.