Extraocular muscle involvement in marginal zone B-cell lymphomas of the orbit.
BACKGROUND
Marginal zone B-cell lymphoma is the most frequent ocular adnexal lymphoma. It may involve the conjunctiva, lacrimal gland, eyelid, extraocular muscle, and orbital connective tissue. Extraocular muscle infiltration is rare.


METHODS
We report here a series of 5 patients presenting with extraocular muscle infiltration due to marginal zone B-cell lymphoma, extracted from a retrospective study of 39 patients with primitive ocular adnexal marginal zone B-cell lymphoma presenting within a 15-year period, from 1993 to 2007, at two university hospitals.


RESULTS
Out of 39 patients, two females and three males presented with extraocular muscle involvement (one levator muscle, one medial rectus muscle, one lateral rectus muscle, one inferior rectus muscle, and one inferior oblique muscle). In 4 cases, the right eye was involved. The median age of presentation was 60 years. Proptosis and diplopia were the main clinical signs. A mean duration of symptoms was 12 months before diagnosis was given. Three patients were stage IE at the diagnosis, according to Ann Arbor classification, and one was at stage IV. Three patients received radiotherapy and two received chemotherapy. Three patients underwent local relapses at a median time of 40 months. The patients with relapses were treated with chemotherapy alone in one case, radiotherapy alone in one case, and immunotherapy and chemotherapy in one case. The mean follow-up period was 54 months.


CONCLUSIONS
When compared to other locations of marginal zone B-cell lymphomas of the orbit, extraocular involvement occurred in younger patients and had similar prognosis.


CONCLUSIONS
Extraocular muscle involvement is a rare location of marginal zone B-cell lymphoma that had to be known and can simulate thyroid orbitopathy.
Izambart
Céline
C
Department of Ophthalmology, Saint Roch Hospital, Nice, France. celine.izambart@tele2.fr
Robert
Pierre-Yves
PY
Petellat
Fabien
F
Petit
Barbara
B
Gastaud
Pierre
P
Lagier
Jacques
J
Labrousse
François
F
Adenis
Jean-Paul
JP
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D004172:Diplopia / Q000175:diagnosis; D005094:Exophthalmos / Q000175:diagnosis; D005260:Female; D006801:Humans; D007167:Immunotherapy; D018442:Lymphoma, B-Cell, Marginal Zone / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009801:Oculomotor Muscles / Q000000981:diagnostic imaging / Q000187:drug effects / Q000473:pathology* / Q000528:radiation effects; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D011878:Radiotherapy; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed