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Orbital lymphomas respond well to local, systemic
therapies, says study
Daniel
M. Keller PhD
in Philadelphia
BOTH local and systemic therapies provide good response rates and
low rates of recurrence in patients with orbital lymphomas, according
to a recent study.
Researchers at Long Island Jewish Medical Centre in New York reported
a series of 35 adult patients with orbital lymphomas identified
between 1991 and 2002 at their institution.
The results of the study, published as part of the proceedings of
the 44th annual meeting of the American Society of Haematology,
showed that at a median follow up of 44.5 months, the overall survival
among patients with low grade lymphomas (Group 1, N=23) was 70%.
For patients with intermediate to high grade lymphomas (Group 2,
N=12), survival was 67%.
Lead author Shahid Ahmed MD, a third-year haematology-oncology fellow
at Long Island Jewish Medical Centre in New Hyde Park, New York,
said most of the patients were elderly (median 75 years) and female
(74%).
Most cases were unilateral, fairly evenly divided between the two
eyes, with only one case of bilateral disease. Most of the tumours
were localised (Stage 1E) at presentation. Three patients had a
history of prior lymphoma.
“Most of them presented with swelling in the eyes or complained
of blurred or double vision, or they might have had redness and
tearing in the eye. Some of them had had proptosis when lymphoma
was retro-orbital,” Dr Ahmed said.
Lymphoma can be associated with constitutional symptoms such as
weight loss, fever and night sweats. But only one patient in the
series had constitutional symptoms and most had local and non-specific
symptoms, which make it difficult to differentiate from any other
eye problem, he emphasised.
Symptoms
The major symptoms at diagnosis were swelling and/or a mass in 12
cases; blurred vision and/or diplopia in 11; and tearing and/or
redness in 11 cases.
In 22 cases, the dominant site of involvement was the orbit, five
of them with involvement of the lacrimal glands. Other dominant
sites were the conjunctive in 14 cases and eyelid in one case.
All of the lymphomas were of B-cell origin and non-Hodgkin histology,
with low-grade histology in two-thirds of the cases. All were confirmed
to be of monoclonal origin. The two groups comprised a variety of
histological types (Group 1: follicular, small lymphocytic, marginal
zone and unclassified low-grade lymphomas; Group 2: large cell,
mantle cell, undifferentiated and mixed histologies).
In Group 1, 70% of patients received radiation therapy, 22% surgery,
and 13% chemotherapy, giving a complete response rate of 91% and
a partial response rate of 9%.
For Group 2, the proportions receiving each therapy were 75%, 33%
and 33% respectively, resulting in a 71% complete response and 29%
partial response rates. Some patients in each group received more
than one treatment modality. Response rates are based on overall
data available for 18 patients.
“Those patients who were treated with the local treatment
for low grade responded very well. Those who had intermediate or
high-grade lymphoma were treated with systemic chemotherapy. Some
of them had received local treatment as well, but the majority received
systemic chemotherapy,” Dr Ahmed said. He added that vision
was preserved in most patients.
Seven patients with localised disease experienced recurrence (four
in Group 1; three in Group 2). Two recurrences occurred locally
and five at distant sites. Large cell histology appeared to increase
the risk of central nervous system (CNS) involvement. Two such patients
with this histology had CNS recurrence and died of progressive disease.
“That's one of the things that needs to be noted when you
are dealing with a patient with large cell lymphoma of the eye.
They can recur in the CNS and may need intrathecal chemotherapy,”
Dr Ahmed advised.
Careful follow-up is necessary because distant recurrence can occur
after local treatment. He said it could occur almost anywhere and
cited in particular the lungs and gastrointestinal tract when histology
was Malt (marginal zone ) lymphoma. All the patients in this cohort
had initially been seen by an ophthalmologist and were then referred
to an oncologist after a biopsy.
Dr Ahmed said some were treated initially for conjunctivitis for
a short time before the biopsy. But for others, a delay in diagnosis
or a misdiagnosis led to longer waits, up to several months before
receiving a definitive diagnosis of orbital lymphoma.
“Ophthalmologists want to remember the possibility of lymphoma
and should keep this entity in their differential diagnosis,”
he cautioned. Overall, Dr Ahmed said that the response rate with
any therapeutic modality, including local or systemic was high,
and the recurrence rate was low. Only seven patients recurred, although
large cell histology was associated with high recurrence rate in
the CNS.
He said his findings are in keeping with previous series of orbital
lymphoma cases, although some of the earlier studies did not have
such good histological classification of the tumours. Those studies,
too, found mostly localised presentations and predominantly low
grade histologies, with similar responses to treatment.
Shahid Ahmed
MD
Long Island Jewish Medical Centre, New York, US
Email: shahidahmed00@yahoo.com
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