Mantle Cell Lymphoma of the Ocular Adnexa

Abstract

Purpose: Mantle cell lymphoma (MCL) is a rare type of lymphoma. Reports of MCL in the ocular adnexa have been published in only some studies with a limited number of cases. We reviewed the frequency of MCL of the ocular adnexal region in a large Mexican referral center.

Methods: Retrospective review of clinical records, histopathology and immunohistochemistry of medical cases (all genders and ages) diagnose with MCL of ocular adnexal from 1958-2013. a. Files from the Ophthalmic Pathology Service from the “Asociación para Evitar la Ceguera en México I.A.P. Dr. Luis Sánchez Bulnes”. b. Cases with diagnosis of Mantle cell lymphoma. c. Clinical data recorded for each patient (included year of diagnosis, gender, age, symptoms and clinical findings). d. Evaluation of clinical pictures, ocular ultrasound, computed tomography and magnetic resonance imaging. e. Follow-up.

Results: Twelve patients with MCL of the ocular adnexa were identified comprising 8% (12/155) of all lymphomas in the ocular region. There were 6 male patients and 6 female patients with an age range from 32 to 79 years old (median 66 years). Forty two percent had bilateral involvement. The orbit (83%) and the lacrimal gland (42%) were the most commonly affected sites. MCL of the adnexal region was the first manifestation of systemic disease. Fifty percent presented in stage II, one stage IV. Microscopically all the cases had a diffuse architectural pattern and expressed CD20 and cycline D1.

Conclusions: Twelve of one hundred and fifty five patients were diagnosed with MCL. Commonly ocular adnexa MCL presents in elderly males, however our patients did no present predominance of gender. The orbit and lacrimal gland were frequently involved. Less than 50% of our patients had bilateral orbital involvement. In the current series the end clinical stage was not as common as other series in the literature. Inmmunohistochemistry is mandatory to establish the definitive diagnosis.