Article
Management of primary orbital lymphomas
Behandlungsstrategien bei primären orbitalen Lymphomen
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Published: | April 23, 2004 |
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Outline
Text
Objective
Extra-nodal presentation occurs in about 40% of patients with non-Hodgkin´s lymphoma (NHL), and the orbit accounts for 5-14% of all cases. The most common age of presentation is 50 to 70 years. We present an overview of the treatment and clinical outcome of 15 orbital lymphomas, carried out in our center from 1993 to 2002.
Methods
Lateral orbitotomy was performed in 5 laterally located lesions, a transconjunctival approach in 5 medial, basal, extra-, intraconal lesions. Pterional intradural and extradural and intradural approaches (3) 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.
Results
Ten patients were diagnosed as EMZL (extranodal marginal zone lymphoma). DLCL (diffuse large B-cell lymphoma) was encountered in 2 patients, FCL (follicular lymphoma) in 2 patients, and LPL (diffuse lymphoplasmatic/lymphocytoid lymphoma) in 1 patient. All patients except 1 failed to reveal systemic disease and had a localized orbital lymphoma. 12 of the patients responded to radiation therapy with usually 40 Gy. CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy was applied in 2 patients, once in combination with radiotherapy. Local recurrence was seen in 1 patient with FCL. Systemic manifestation occurred in 1 patient with DLCL, undergoing ICE (ifosfamide, carboplatin, etoposide) chemotherapy with complete remission.
Conclusions
The course of orbital NHL is variable and requires a multidisciplinary treatment. Radiotherapy is effective in indolent forms, chemotherapy is applied in aggressive lymphomas. Surgical biopsy should be performed via the adequate approach according to the location and extension of the lesion without additional morbidity.