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Surv Ophthalmol. 2017 Jan - Feb;62(1):83-88. doi: 10.1016/j.survophthal.2016.05.005. Epub 2016 May 30.

Bilateral ocular panadnexal mass as initial presentation of systemic blastoid variant of mantle-cell lymphoma.

Author information

1
Department of Ophthalmology, Faculty of Medicine, University of Belgrade, Serbia; Department of Ocular and Ocular Adnexal tumors, Orbital and Lacrimal System Pathology, and Ocular Oncology, Clinic of Eye Diseases, Clinical Center of Serbia, Belgrade, Serbia; Ophthalmic Pathology Laboratory, Clinic of Eye Diseases, Clinical Center of Serbia, Serbia. Electronic address: drnajed@gmail.com.
2
Department of Ophthalmology, Faculty of Medicine, University of Belgrade, Serbia; Department of Ocular and Ocular Adnexal tumors, Orbital and Lacrimal System Pathology, and Ocular Oncology, Clinic of Eye Diseases, Clinical Center of Serbia, Belgrade, Serbia.
3
Department of Ophthalmology, Faculty of Medicine, University of Belgrade, Serbia; Department of Hematopathology, Institute of Pathology, Faculty of Medicine, University of Belgrade, Serbia.

Abstract

A 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.

KEYWORDS:

blastic; blastoid; conjunctiva; eyelid; lacrimal gland; lacrimal sac; lymphoma; mantle cell; ocular adnexal region; orbit

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