Orbital lymphoma

Abstract

Orbital lymphomas constitute 50-60% of ocular adnexal lymphomas. A total of 2211 cases of orbital lymphoma with a known subtype have been reported in the last 24 years (1994-2017). The vast majority of orbital lymphomas are of B-cell origin (97%), of which extranodal marginal zone B-cell lymphoma (EMZL) (59%) is the most common subtype, followed by diffuse large B-cell lymphoma (23%), follicular lymphoma (9%), and mantle cell lymphoma (5%). Orbital lymphoma is primarily a disease of the elderly. Gender distribution varies according to lymphoma subtype. However, extranodal marginal zone B-cell lymphoma (53%) and follicular lymphoma (75%) show a female predominance, whereas diffuse large B-cell lymphoma shows an even gender distribution. Mantle cell lymphoma has a striking male predominance of 80%. The histopathological subtype and the clinical stage of the disease are the best indicators of prognosis and patient outcome. Low-grade lymphomas such as extranodal marginal zone B-cell lymphoma and FL have a good prognosis, whereas high-grade lymphomas (diffuse large B-cell lymphoma and mantle cell lymphoma) are associated with a poor prognosis. When managing solitary low-grade lymphomas, radiotherapy is the treatment of choice. Chemotherapy, with or without radiotherapy, should be chosen for disseminated and high-grade lymphomas.

A pan‐inflammatory microRNA‐cluster is associated with orbital non‐Hodgkin lymphoma and idiopathic orbital inflammation

“…NHOL covers several disease subtypes with varying disease course and underlying molecular circuitry . Most NHOL were of the EMZL subtype in this study (Table ) and we were therefore unable to compare NHOL subtypes.…”

“…Non‐Hodgkin orbital lymphoma (NHOL) is the most common orbital malignancy in adults . Early and accurate diagnosis is essential to reduce metastatic spread, and avoid disease‐related morbidity and mortality.…”

“…Generally, NHOL first presents as a non‐specific mass within the orbit or ocular adnexa, which may be difficult to differentiate from idiopathic orbital inflammation (IOI), the most common non‐thyroid associated orbital inflammatory condition . Consequently, an incisional biopsy is required to accurately diagnose both diseases . However, a surgical biopsy can be technically challenging in case of deep orbital localization of the mass, which increases the risk for complications and nonrepresentative tissue biopsies.…”

Linfoma extranodal de zona marginal en órbita: Revisión de Caso

“…Este es un grupo muy donde tenemos 40 diferentes tipos de linfoma [1]. Predomina en pacientes mayores de 50 años, en el sexo femenino, con una incidencia de 1,4 /1.000.000 casos por año y con una media de 65 años de edad [4,5].…”

“…También se asocia con la mutación del gen A20. Este gen es un inhibidor del NF-κB y se ha visto que se encuen EMZL [4] s extranodales de células B de zona , tal y como el que se diagnosticó en el caso clínico. En el resto se incluye el Linfoma difuso de células grandes B (DLBCL) y otros subtipos menos frecuentes [2,3].…”