Mantle Cell Lymphoma in the Orbital and Adnexal Region

Department of Neuroscience and Pharmacology, Section of Eye Pathology, University of Copenhagen, Denmark.
The British journal of ophthalmology (Impact Factor: 2.98). 06/2009; 93(8):1047-51. DOI: 10.1136/bjo.2008.146910
Source: PubMed


To characterise clinicopathological features of mantle cell lymphoma (MCL) in the orbital and adnexal region.
Data on lymphoid lesions were retrieved searching the Danish Ocular Lymphoma Database 1980-2005. Specimens were collected from Danish pathological departments and re-evaluated with a panel of monoclonal antibodies. For all patients with confirmed MCL the complete clinical files were collected and reviewed.
Twenty-one patients with MCL in the orbital and adnexal region were identified comprising 9% (21/230) of all lymphoma in the ocular region. There were 18 male patients and three female patients with an age range from 60 to 90 years (median 75 years). Orbital and adnexal region MCL as first presenting symptom comprised 67% of the patients. Of these, 71% had bilateral involvement. The orbit (71%) and eyelids (64%) were the most commonly affected sites. All but two presented in stage III/IV. Secondary MCL comprised 33% of the patients. Bilateral affection (29%) was less common in this patient group. The median survival was not different between the two presentation groups. Patients receiving anti-CD20 (rituximab)-containing chemotherapy had a significantly better 5-year overall survival (OS) rate (83%) than patients in treatment regimes without rituximab (5-year OS rate, 8%).
Orbital and adnexal region MCL presents in elderly males. The orbit and eyelid are frequently involved. There is a very high proportion of systemic involvement in general with MCL of the orbital and adnexal region. Most patients presented with stage IV disease and had multiple relapses and short survival time. Treatment with rituximab-containing chemotherapy improved survival significantly compared with combination chemotherapy without rituximab.

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Available from: Jan Ulrik Prause
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    • "The overall survival was 43 months for those first presenting with ocular adnexal MCL while it was 51 months for those with secondary ocular adnexal MCL. Furthermore, bone marrow involvement was seen in 79% of those first presenting with ocular adnexal MCL as opposed to 57% in those with secondary ocular adnexal MCL [7]. This case report of a 78-year-old man presenting with bilateral eyelid masses and no prior history of lymphoma is a nice clinical example of how diagnostic zebras do occur and can greatly impact the clinical management of a patient. "
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    ABSTRACT: Orbital and ocular adnexal lymphomas are rare and represent around 1-2% of lymphomas and about 8% of the extranodal lymphomas. However, these entities represent the majority of orbital malignancies. Lymphomas of the ocular adnexal region are primary or secondary lymphomas, and the majority of them are composed of small, mature lymphocytes, which provide a large differential diagnosis. Thus, these entities are not easily distinguished from indolent lymphoid processes such as reactive lymphoid hyperplasia. Extranodal marginal zone lymphoma is the most common lymphoma in the ocular adnexal region. However, this entity cannot be distinguished from benign lymphoid proliferations or other lymphomas composed of small, mature lymphocytes by routine histopathology. We describe a 78-year-old man who presents with bilateral upper eyelid masses, which had been present and grew in size over the past twelve months prior to his presentation. A biopsy of the mass shows a monotonous population of small, mature lymphocytes. The immunohistochemical studies performed on the eyelid mass confirmed a monoclonal proliferation of B cells expressing cyclin-D1; therefore, a final diagnosis of mantle cell lymphoma was rendered. A literature review of mantle cell lymphoma with orbital and ocular adnexal involvement and the diagnostic pitfalls in this area of hematopathology are discussed.
    Full-text · Article · Apr 2013
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    • "Mantle cell lymphoma (MCL) is a relatively rare lymphoma, accounting for less than 10% of all lymphomas. In the ocular adnexal region, including rare cases of conjunctiva, MCL accounts for between 1-5% [4] and 9% of the lymphomas [5]. The orbit is the most commonly involved site of MCL involvement, followed by the lacrimal gland and the eyelid, similar to extranodal marginal zone lymphoma in this region [6]. "
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    ABSTRACT: Background To describe a rare manifestation of mantle cell lymphoma (MCL) in conjunctiva, with clinical, hisologic, immunohistologic and genetic findings together with review of the Literature. Case presentation Most ocular adnexal lymphomas are extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT). A few cases of ocular adnexal mantle cell lymphomas have been reported in the literature. We present a case of mantle cell lymphoma presenting as right conjunctival mass of at least three months duration in a 64-year-old man. Histopathologic examination showed a proliferation of monomorphous small-to-medium-sized lymphoid cells with cleaved nuclei in the subconjunctiva. By immunohistochemistry, the infiltrate was positive for CD20, CD5, BCL-2, cyclin D1, and the transcription factor SOX11. Fluorescent in situ hybridization demonstrated the presence of IGH-CCND1 fusion indicating t(11;14). Conclusion A rigorous approach to initial diagnosis and staging of small cell lymphomas of the ocular adnexa is needed. The recognition of ocular MCL requires appropriate immunohistochemical staining and/or genetic confirmation to differentiate this rare form of presentation of MCL from other more frequent small cell lymphomas.
    Full-text · Article · Dec 2012 · BMC Research Notes
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    • "The condition is quite indolent with a relatively good prognosis.[45] Radiation therapy (RT) has an important role in the management.[5–11] Clinical course of localized MALT lymphomas is variable and firm evidence is lacking due to the rare incidence of these lymphomas. "
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    ABSTRACT: To report the clinical and treatment outcome of patients with lacrimal gland lymphoma (LGL) treated with radiation therapy (RT) at Fox Chase Cancer Center, Philadelphia, PA, USA. Institutional review board approved retrospective chart review of eight patients and literature review. The study patients included six males and two females with a mean age of 70 years (range 58-88 years). The mean follow-up period was 23 months (range 3-74 months). Four patients had mucosa-associated lymphoid tissue (50%) lymphoma and four patients had other non-Hodgkin's lymphoma variants. Four patients had bilateral disease (50%). Four patients had primary LGL (stages I-IIAE, 50%) and four had LGL as part of systemic lymphoma (stage IVAE, 50%). The median RT dose was 2987 cGy (range 2880-3015 cGy). All patients had complete response to RT with symptomatic relief. Minimal dry eye was seen in all patients. There were no late effects such as corneal ulcer, radiation retinopathy, maculopathy, papillopathy, or secondary neovascular glaucoma. RT alone is an extremely effective treatment in the curative management of localized LGL and provides durable, local control of secondary LGL.
    Full-text · Article · Mar 2012 · Oman Journal of Ophthalmology
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