Treatment of primary orbital lymphomas

We present an overview of the treatment and clinical outcome of 15 orbital lymphomas, carried out in our center from 1993 to 2002. The surgical approach was determined by the location, and type of the lesion. Lateral orbitotomy was performed in five laterally located lesions, a transconjunctival approach in five medial, basal, extra-, intraconal lesions. Pterional intradural and extradural and intradural approaches (three) were used in case of intracranial involvement or location in the apex and optic canal. Two lesions of the lid or extraconal space were operated via eyebrow incision. Ten patients were diagnosed as extranodal marginal zone lymphoma. Diffuse large B-cell lymphoma (DLCL) was encountered in two patients, follicular lymphoma (FCL) in two patients, and diffuse lymphoplasmacytic/lymphoplasmacytoid lymphoma in one patient. All patients except one failed to reveal systemic disease and had a localized orbital lymphoma. Twelve of the patients responded to radiation therapy with usually 40 Gy. Cyclophosphamide, doxorubicin, vincristine, prednisone chemotherapy was applied in two patients, once in combination with radiotherapy. Local recurrence was seen in one patient with FCL. Systemic manifestation occurred in one patient with DLCL, undergoing ifosfamide, carboplatin, etoposide chemotherapy with complete remission. The course of orbital non-Hodgkin's lymphoma is variable and requires a multidisciplinary treatment. Therapeutic options include surgical biopsy, radiation therapy, and chemotherapy.