Mucosa-associated lymphoid tissue lymphoma with intraocular and orbital involvement: case presentation and review of the literature

Abstract

Primary ocular lymphomas are typically confined to either the eye or the orbit. Rarely, in immune-competent patients, lymphomas affect both the eye and the orbit simultaneously. Mucosa-associated lymphoid tissue (MALT) lymphomas are the most common ocular lymphomas. They usually present primarily in the orbit but sometimes can present primarily in intraocular tissue. MALT lymphomas that occur initially in the uvea can sometimes spread to the adjacent orbit. We report a case of progressively enlarging MALT lymphoma in a 62-year-old immune-competent patient causing a severe mass effect in the orbit and simultaneously presenting with intraocular involvement. There was radiographic evidence of lymphoma confined to the orbit with intraocular involvement. The simultaneous presentation makes it difficult to determine if the lymphoma initially presented in the orbit or intraocular tissue, although the orbital component was more impressive. The case also includes a literature review of simultaneous orbital and intraocular MALT lymphomas. The patient responded to systemic chemotherapy with regression in size of the lymphoma, relief of the mass effect seen in the orbit, and the regression of the intraocular involvement.

Clinical Features of Primary and Systemic Metastatic Intraocular Lymphomas in Spanish Patients

“…Among IOLs, PVRL is the most described type of IOL by far, accounting for 72% as recently described [7] and 52% in our study. PUL are described rarely and usually excluded in IOL series, or reported separately [4, 8]. Nevertheless, PUL were found in 19% of our patients.…”

Imaging of head and neck mucosa-associated lymphoid tissue lymphoma (MALToma)

“…Other sites include other extraocular muscles (especially lateral rectus muscle), conjunctiva, eyelids and extraconal and intraconal soft tissues (Fig. 6) [48]. MALToma may be localised to one site or may spread to involve multiple adjacent sites, with bilateral disease (Fig.…”

“…Bone erosion and hyperostosis are uncommon. Intraocular extension is also uncommon and suggests the rare possibility of intraocular uveal MALToma, which spreads outside the globe via the optic nerve [48]. MRI is generally preferred over CT for orbital imaging due to better soft tissue contrast and avoidance of ionising radiation.…”

The outcome of radiation therapy as a primary treatment in orbital lymphoma: a systematic review

“…The conjunctiva, eyelid, lacrimal gland, and retrobulbar region are commonly affected by orbital MALT lymphomas (OAML). It is characterized by an indolent course and a confined tumor that is predominantly radiation sensitive [1][2][3].…”

“…However, the ideal radiation dose for the treatment of orbital adnexal lymphoma that achieves a high local control rate with a low risk of visually noticeable sequelae is unknown. So, a variety of approaches, involving radiation dose, volume, and lens shielding, have been used [1][2][3][4].…”