ABSTRACT

Purpose:

To present the clinical and histopathological features, systemic involvement and treatment outcomes of the malignant lymphoma of the orbit and ocular adnexa.

Methods:

Twenty-one patients that had histologically proven orbital or ocular adnexial lymphoma between 1997 and 2007 were evaluated retrospectively. The age, gender, tumor localization, clinical and histopathological features, systemic state, treatment modalities and outcomes were recorded and analyzed.

Results:

The mean age was 54.67. Of 21 patients, 3 cases had eyelid involvement, 3 cases had conjunctival involvement and 6 cases had lacrimal gland involvement, whereas 4 cases had paranasal sinus lymphoma extending into the orbit. Clinical presentation differed regarding to tumor localization and the most frequent findings were, painless palpable mass, globe displacement, motility disturbance, proptosis and eyelid edema. All patients were diagnosed as non-Hodgkin lymphoma and the most frequent histological subtypes were Malt lymphoma and diffuse large cell B lymphoma. While systemic involvement was present in 47.6% of cases at presentation, this rate increased to 62% after a mean follow up time of 3.6 years. Radiotherapy and/or surgery was performed to patients with localized disease whereas additional chemotherapy was applied to patients with systemic involvement.

Comment:

Lymphoma was one of the most common tumors of the orbit and ocular adnexa.The clinical characteristics may differ regarding to the site of localization. The patients must be treated with a multidisciplinary management and the systemic evaluation must be performed to all patients to exclude the extraorbital involvement.