Rašić, Dejan M. (24400176900)Dejan M. (24400176900)RašićKnežević, Miroslav (36192212000)Miroslav (36192212000)KneževićTerzić, Tatjana (55916182400)Tatjana (55916182400)TerzićVlajković, Gordana (56619947100)Gordana (56619947100)Vlajković2025-06-122025-06-122017https://doi.org/10.1016/j.survophthal.2016.05.005https://www.scopus.com/inward/record.uri?eid=2-s2.0-84977618049&doi=10.1016%2fj.survophthal.2016.05.005&partnerID=40&md5=8f942a3a7285f2e7927e94f3a26ea428https://remedy.med.bg.ac.rs/handle/123456789/7422A 66-year-old man developed a slowly enlarging, bilateral, painless, periorbital, and orbital swelling with ptosis, nonaxial proptosis, chemosis, exposure keratopathy, and decreased vision in both eyes. He had fever, night sweats, and weight loss (B-symptoms), along with lymphadenopathy and elevated serum lactate dehydrogenase, with no prior history of lymphoma. A transpalpebral incisional biopsy revealed a rare case of mantle-cell lymphoma of blastoid variant, stage IVB. The main immunophenotype characteristics were cyclin D1+, CD5+, CD10−, CD23−, Bcl-6−/+, and a high (up to 80%) Ki-67 proliferation index. Following an excellent response to the immune-chemotherapy treatment plan, all ocular adnexal lymphoma manifestations disappeared completely; however, 13 months after the initial presentation, there was a recurrence of the disease with rapid worsening and death. The blastoid variant of mantle cell lymphoma, a rare subtype of mantle-cell lymphoma, is a highly aggressive neoplasm, ultimately having a fatal outcome. As the initial manifestation of the disease, ocular adnexal region blastoid variant of mantle-cell lymphoma is an exceptional event, with only one previous case reported. © 2016 Elsevier Inc.blasticblastoidconjunctivaeyelidlacrimal glandlacrimal saclymphomamantle cellocular adnexal regionorbit