Bilateral orbital tumor as initial presenting sign in human T-cell leukemia virus-1 associated adult T-cell leukemia/lymphoma.

PURPOSE: To report a case of human T-cell leukemia virus type1 (HTLV-1)-associated adult T-cell leukemia/lymphoma (ATLL) whose initial sign was exophthalmos.

DESIGN

Case report.

METHODS

A 64-year-old Japanese man presented with exophthalmos and choroidal folds of the right eye without general symptoms and received ophthalmologic and laboratory examination, magnetic resonance imaging (MRI), and orbital biopsy.

RESULTS

Antibodies against HTLV-1 were extremely high but atypical lymphocytes were not present in the peripheral blood. MRI showed multiple tumorous lesions in both orbits. Orbital biopsy revealed an orbital mass that consisted of atypical lymphocytes originally from T cells and established the diagnosis of lymphoadenopathy type of ATLL.

CONCLUSION

Although ocular involvement by ATLL is extremely rare, ATLL can first present in the orbit, and only the results of a biopsy can provide definitive information for its diagnosis.