Orbital mucosa-associated lymphoid tissue (MALT)-type lymphoma in a patient with relapsing polychondritis.
Relapsing polychondritis is characterized by recurrent inflammation of the cartilaginous tissues of the ears, nose, peripheral joints, and the tracheobronchial tree. The eye is also a frequent target organ in relapsing polychondritis, and proptosis is a well-recognized manifestation of eye involvement. Similar to other rheumatologic diseases, an association of relapsing polychondritis with malignancy has been reported. We describe a patient with relapsing polychondritis who presented with exophthalmos. When treatment directed toward control of her underlying disease was only partially effective, further investigation revealed that she had an orbital mucosa-associated lymphoid tissue (MALT)-type B cell lymphoma. We hypothesize that the lymphoma resulted from malignant transformation of the relapsing polychondritis-induced inflammatory pseudotumor and emphasize that neoplastic disease should be considered in the differential diagnosis in patients with relapsing polychondritis presenting with exophthalmos.
Lichauco
J J
JJ
Albert Einstein College of Medicine, Bronx, New York 10461, USA.
Lauer
S
S
Shigemitsu
H H
HH
Bello
J A
JA
Bhattacharyya
P K
PK
Barland
P
P
Putterman
C
C
D000328:Adult; D003937:Diagnosis, Differential; D005094:Exophthalmos / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications* / Q000473:pathology; D009918:Orbital Neoplasms / Q000150:complications / Q000473:pathology; D011081:Polychondritis, Relapsing / Q000150:complications* / Q000473:pathology; D014057:Tomography, X-Ray Computed