Background-Primary oculocerebral large cell malignant non-Hodgkin's ly
mphoma, formerly called ocular reticulum cell sarcoma, runs a uniforml
y fatal course. Once the central nervous system (CNS) is involved, sur
vival without treatment is very limited. Although treatment does not s
ubstantially improve the long term survival, it provides short term im
provement in these patients. Methods-The charts of all patients with o
cular involvement of non-Hodgkin's lymphoma followed during the period
1984-93 were reviewed. The diagnosis of non-Hodgkin's lymphoma was ma
de by different diagnostic approaches: CNS biopsy, anterior chamber ta
p, vitrectomy, haematology, and necropsy. Results-Eight patients had o
culocerebral large cell and one had small cell non-Hodgkin's lymphoma.
Five patients with pure ocular localisation had initially received st
eroid treatment for intermediate uveitis. First diagnosis was made on
CNS biopsy in three, anterior chamber tap in one, vitreous aspirate in
three, haematology in one, and necropsy in one case. Conclusion-Ocula
r non-Hodgkin's lymphoma is a difficult diagnosis. Vitrectomy allows c
ytological diagnosis in most but not all cases. When no treatment is g
iven, patients survive for only a few weeks once the CNS is involved.
Although the disease is eventually fatal, treatment by means of radiot
herapy, steroid administration, and vitrectomy can allow these patient
s to lead a normal professional and social life during the years betwe
en recurrences.