Diagnostic difficulties and treatment of non-Hodgkin lymphoma of the orbit

There are problems connected with the diagnosis of non-Hodgkin lymphomas of the orbit, before deciding on the correct treatment.

Six out of 22 patients with a tumour of the orbit were treated between 1995 and 2004 for orbital non-Hodgkin lymphomas (NHL). Morphological-functional and aesthetic complications regarding the eyes, the delay between onset and diagnosing the disease and concomitant conditions were evaluated. Management was always tailored individually for each patient, depending on tumour location in the orbit. Surgical procedures comprised total or subtotal removal of the tumour via a semi-coronal access and lateral orbitotomy. Histological diagnosis of the tumours was made from the surgical specimens.

According to the REAL classification, 3 patients had a B-cell lymphoma from the MALT system with low malignancy, 2 other patients a diffuse B cell NHL and 1 patient a lymphoma from the germinal centre with intermediate malignancy. All patients had received chemotherapy. A 9-year remission was achieved in one, an 8-year in another and a 7-year remission in two more out of the total of 6 patients; one is living without relapse for 6 years. The last patient, in whom the lymphoma was diagnosed at the age of 70 years, died 4 years after the procedure without relapse due to cardiovascular and respiratory insufficiency.

Although the treatment of choice of NHL is chemotherapy, it is felt necessary to remove the whole or possibly the major part of the tumour in case of retrobulbar location when the histological subtype is unknown prior to surgery. However, the optic nerve should always been left intact, even when such tumour encircles this cranial nerve. Only sufficient biopsy material allows determination of the lymphoma subtype and selection of appropriate chemotherapy.