Cephalic and ocular manifestations of EBV-associated plasmablastic lymphoma: Clinical and radiological response to bortezomib, EPOCH, and intrathecal methotrexate.

Plasmablastic lymphoma (PBL) is a rare and aggressive type of lymphoma, particularly affecting HIV-positive and immunocompromised individuals, with a median diagnosis age of 49 years. Cases of this malignancy in HIV-negative individuals are less common and rarely involve the bone marrow. While traditional chemotherapy regimens such as cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) were previously utilized in the management of such malignancy, the National Comprehensive Cancer Network currently recommends more intensive approaches. We present a rare stage IV Epstein-Barr virus (EBV)-positive PBL with a nasal cavity tumor extending into the left orbital sinus and encapsulating segments of the optic nerve in a 38-year-old young immunocompetent adult, without a significant past medical history. Treatment consisted of 6 cycles of Bortezomib in combination with dose-adjusted EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) and intrathecal methotrexate which exhibited significant clinical and radiological improvement suggesting the potential efficacy of this regimen. Vision returned to baseline, the mass size reduced significantly, and headaches improved. Given this outcome, it is highly encouraged to emphasize the need for further exploration of this treatment in larger clinical trials.