Orbital and palpebral lymphomas

Abstract

SummaryExtra nodal lymphomas may involve the ocular adnexae in 8% of cases. The mean age of onset is basically in the sixth decade. Lymphomas may present as a palpebral mass (39%), conjonctival infiltrate (39%) or proptosis (31%). It may involve orbital fat (47%), orbital muscles (26%), lacrymal gland (19%), preseptal tissues of eyelids (10%), or conjonctiva (60%). However, no specific location has been statistically associated to a better prognosis.Although MALT lymphoma (low grade with good prognosis) is the most common type (46%), other types such as follicular (20%), DLCB (11%), Mantel (5%), or other (17%) may occur. Lymphoma of ocular adnexae represent around 12% of all MALT lymphomas.The incidence of Chlamydia Psittaci infection in lymphoma patients may vary according to the geographic area, for instance 0% in Florida and 80% in Italy.The incidence of amyloïdosis in lymphoma patients arises from 0.85% to 7.5%. However amyloïd deposits are often not systematically looked for, and therefore may remain misdiagnosed.The handling of orbito‐palpebral lymphomas requires the collaboration of a multidisciplinary team involving pathologists, biological and clinical haematologists, microbiologists and orbital surgeons.