Bilateral mantle cell lymphoma of the ciliary body that responded to a combined local radiotherapy and chemotherapy regimen: a case report

Abstract

Background Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) that often affects men over the age of 60. Systemic metastasis of MCL to eyes is rare and intraocular involvement is even rarer, which usually affects the choroid and iris. To the best of our knowledge, ciliary body metastasis of systemic MCL has not been reported. Case presentation A 59-year-old Han Chinese male with past-history of systemic MCL complained of redness, pain and blurred vision in the left eye. Ocular examination revealed a normal appearance in the right eye, and conjunctival injection, pseudohypopyon and anterior protrusion of peripheral iris in the left eye, all of which were unresponsive to corticosteroid treatments. Ultrasound biomicroscopy (UBM) and B-scan were then performed which detected ciliary body masses in both eyes with no vitreous and retino-choroidal anomalies. Combined liquid-based cytology tests and gene rearrangement assays of the aqueous humor specimen confirmed this to be a B-cell malignancy. Then both eyes were treated with external beam irradiation (40 Gy, delivered evenly in twenty fractions) over a course of one month. Additionally, the left eye received intravitreal methotrexate (MTX) (weekly for the first month, every two weeks for the second month, and monthly thereafter) over a course of twelve months. This therapy eventually led to complete remission of all symptoms in one month and disappearance of the ciliary body masses in twelve months. Conclusion Here we first reported a case of bilateral ciliary body MCL infiltration which was diagnosed by combined liguid-based cytology and gene rearrangement of aqueous humor cells. UBM may serve as a valuable tool in the diagnosis and serial assessments of anterior segment tumors.

Case Report: Ocular Manifestations and Treatments of Ciliary Body Involvement by Lymphoma

“…Pei et al. ( 6 ) proposed that the presence of tumor cells in the aqueous humor makes it possible to diagnose ciliary body involvement by lymphoma. However, aqueous samples are frequently nonconfirmatory due to the scant cellularity of the sample ( 13 , 14 ).…”

“…The identification of lymphoma cells is considered the gold standard for the diagnosis of lymphomatous involvement of ciliary (5). Pei et al (6) proposed that the presence of tumor cells in the aqueous humor makes it possible to diagnose ciliary body involvement by lymphoma. However, aqueous samples are frequently nonconfirmatory due to the scant cellularity of the sample (13,14).…”

“…The majority of uveal lymphomas are confined to the choroid, while reports of ciliary body involvement have rarely been documented. This rare condition is further classified as primary or secondary lymphoma, depending on whether the disease is confined to the ciliary body or whether there is concomitant extensive systemic lymphoma (2)(3)(4)(5)(6)(7)(8). We herein report the clinical features, histopathological findings, systemic associations and treatment outcomes of three cases of ciliary body involvement by lymphoma with different primary lesions.…”

Iris metastasis of diffuse large B-cell lymphoma misdiagnosed as primary angle-closure glaucoma: A case report and review of the literature

“…We found 7 reports addressing the method for diagnosing intraocular lymphoma (13.4%), 1 report of primary intraocular lymphoma with concurrent involvement of both B-cells and T-cells (1.9%), 3 reports of unspecified pathological patterns (5.8%), and 11 reports of other pathological patterns (21.2%), such as concurrent mucosa-associated lymphoid tissue lymphoma, melanoma, and plasmacytoma involving the uvea. It was evident that most of these reported cases involved primary intraocular lymphoma; only six cases found via the PubMed search involved patients diagnosed with lymphoma with intraocular metastasis [ 9 , 10 , 11 , 12 , 13 , 14 ]. Thus, it is important to discuss this patient, analyze her clinical manifestations, study treatment procedures, and learn from our experience.…”

“…She had a history of diabetes and was initially misdiagnosed with neovascular glaucoma. Pei et al [ 14 ] reported a 59-year-old Han Chinese male with past history of systemic MCL with complaints of redness, pain, and blurred vision in the left eye. UBM and B-scan were then performed and ciliary body masses in both eyes were detected.…”