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Lymphome orbitaire primaire : Rapport de cas et revue de la littérature

Localized orbital non-Hodgkin's lymphoma (NHL) is a rare form of extranodal lymphoma. It represents 1% of all Non-Hodgkin’s lymphoma. This tumor is seen more commonly between 50 and 70 years old with a slight female predominance. The orbital infiltration by lymphoma is characterised by a palpable, firm or rubbery mass. The diagnostic of this tumor is difficult because the clinical signs and symptoms are nonspecific. We report the case of a men patient with primary orbital non-Hodgkin lymphoma, and describe the clinical and pathological aspects of the disease and its treatment modalities.

A 68-years-old man, without antecedents, presented with right orbital swelling increasing volume for six months and progressive loss of right eye vision. Ophthalmological examination showed a big orbital mass of the right eye with ptosis . Orbital Computed Tomography : process with extension to the lacrimal glands and the upper eyelid Biopsy with pathological examination and immunohistochemical study was for a large-Non-Hodgkin’s lymphoma B cell CD20+. The patient was staged IE according to the Ann Arbor Staging classification. He was treated with chemotherapy according to the R-CHOP protocol. 

The clinical response to the treatment was excellent .The patient receives a dose of 30 Gy of radiotherapy. After a follow up of 2 years, there was no reccurence of the tumor and no metastasis. 

Primary non-Hodgkin's lymphoma (NHL) of the orbit is a rare presentation, representing 8–10% of extranodal NHL. The diagnostic is confirmed by complete examination of the orbit, Computed Tomography Orbital image and histological examination of tumor biopsy as in our case.

Clinical signs often presented by a swelling, exophthalmos and orbital painless, with or without inflammation.

The treatment depends on the type and the extension of the tumor, however, the treatment for localized and asymptomatic forms is consisted only on observation but for the symptomatic forms or high grade, they are treated by surgical excision alone or with radiotherapy. Disseminated or metastatic forms are treated with chemotherapy according to the protocol CHOP or R-CHOP if CD20 positive with radiotherapy in some cases for a better local control [11] . Our patient receives the R-CHOP every 3 weeks. The patient was in good clinical condition throughout the period of chemotherapy and the lesion disappeared after the second cycle of chemotherapy. He received 4 cycles of this protocol then 30 Gy of radiotherapy. No sign of tumor recurrence showed after 8 months of the last course of radiotherapy.

Primary NHL of the orbit is rare. The physician should be taken in consideration when he find an orbital mass. The treatment with R-CHOP protocol proved excellent results.