Testicular lymphoma, intraocular (vitreoretinal) lymphoma, and brain lymphoma: Involvement of three immunoprivileged sites in one patient

The brain, the testicles, and parts of the eye, including, inter alia, the vitreous, and the retina, are immune-privileged organs with relatively sealed blood-tissue barriers. These three organs may, rarely, develop aggressive primary B-cell lymphoma. Vitreoretinal lymphoma is commonly associated with primary central nervous system lymphoma (PCNSL) and with testicular lymphoma, which has a high incidence of relapse, one of the common sites of relapse is the brain. The association of testicular lymphoma with vitreoretinal lymphoma is extremely rare. In these three organs, in treating the lymphoma, the blood-tissue barrier must be overcome. We present a unique case of a patient with testicular lymphoma who 3 years later was diagnosed with monocular vitreoretinal lymphoma, and while being treated for the ocular lymphoma developed PCNSL. The lymphomas in the three organs were treated successfully: the testicular lymphoma by chemotherapy, rituximab, and radiation therapy, with intrathecal methotrexate (MTX) as a preventative measure; the vitreoretinal lymphoma by intravitreal injections of MTX alone; and the PCNSL by intravenous high-dose MTX, rituximab, dexamethasone, procarbazine, and intrathecal MTX. Ten months after completion of the last treatment, there were no signs of systemic, CNS or ocular recurrence.