Lymphoma of ocular adnexa

Ocular lymphomas constitute
- 1-2% of all lymphomas
- 8% of all extranodal lymphomas
5% of all patients with lymphoma will develop involvement of the ocular adnexa
Lymphomas account for 10-20% of all ocular masses

Epidemiology

Patients have a wide age range, including children, with a mean of 64 years1.

Clinical features

Presentation is with a combination of a mass, exophthalmos, ptosis, pain, diplopia, nasolacrimal duct obstruction and glaucoma. Conjunctival involvement produces a salmon pink plaque.

Histopathology

Most lymphomas of the ocular adnexa are B-cell lymphomas.

Reference 1	number of cases
marginal zone lymphoma	182	14/182 patients of patients with ocular marginal zone lymphoma have prior lymphoma at other sites. Lymphoepithelial lesions are only apparent if there is lacrimal gland involvement. Aggregates of plasma cells are common. Polykaryocytes with cohesive moulded nuclei may be present. There may be perivascular hyalinisation. Amyloid deposition may occur.
follicular lymphoma	80	25/80 patients had a prior diagnosis of lymphoma.
mantle cell lymphoma	19	12/19 patients had a prior diagnosis of lymphoma and four more had widespread disease at presentation. One case was a blastoid variant.
CLL	13	8/13 patients had a prior diagnosis of CLL and one had a known blood lymphocytosis. Two cases had small aggregates of monotypic plasma cells.
Lymphoplasmacytic lymphoma	4	3 patients had a history of Waldenstrom's macroglobulinaemia, the fourth had a history of disseminated lymphoma. One case showed amyloid deposition.
Splenic marginal zone lymphoma	2	both had secondary ocular involvement
Low grade B-cell lymphoma, unclassified	19
Diffuse large B-cell lymphoma	27	5/27 patients had a prior diagnosis of lymphoma.
Precursor B lymphoblastic lymphoma	3
High grade B-cell lymphoma, unclassified	1
Peripheral T-cell lymphoma, unspecified type	1
Extranodal NK/T cell lymphoma, nasal type	1
Hodgkin lymphoma	1