Chronic Inflammatory Orbitopathy Hiding Orbital Lymphoma

Abstract

The incidence of lymphoma is constantly increasing worldwide. The reasons for this increase are unclear and likely multiple. B cell lymphomas represent the majority of non-Hodgkin lymphomas. Primary orbital localization remains a rare entity to think about in order to avoid missing a therapeutic emergency. In this article, we report the case of a 52-year-old man who has been treated for five years for an inflammatory orbitopathy with steroids, but the worsening of the clinical condition and the installation of exophthalmia led to push investigations and the outcome was a primary orbital lymphoma marginal zone type. The patient was treated by systemic chemotherapy with immunotherapy (RCHOP protocol) with a very good evolution and complete disappearance of the lesion after chemotherapy.

Orbital Lymphoma Masquerading as Euthyroid Orbitopathy

“…According to a report, the median latency between the diagnosis of thyroid orbitopathy and the development of periocular lymphoma in patients was 17.5 years [13]. According to another case report, the time to diagnosis was five years [14]. Our patient was diagnosed with lymphoma within two years of the diagnosis of euthyroid ophthalmopathy.…”

Predicting the prognosis of primary orbital lymphoma by clinical characteristics and imaging features

“…The incidence of primary orbital lymphoma has increased in recent years; however, the causes of this disease remain unclear. Several investigators suggested that the development of lymphoma, especially EMZL, may be associated with longterm chronic infections of the patients, such as chlamydia infection, helicobacter pylori infection, and chronic hepatitis [25][26][27][28] . The immune status was also considered to be associated with the development of orbital lymphoma [29][30] .…”