Ocular adnexal IgG4-producing mucosa-associated lymphoid tissue lymphoma mimicking IgG4-related disease.
IgG4-related disease is a recently proposed clinical entity with several unique clinicopathological features. A chronic inflammatory state with marked fibrosis, which can often be mistaken for malignancy, especially by clinical imaging analyses, unifies these features. In the present report, we describe a case of IgG4-producing mucosa-associated lymphoid tissue lymphoma mimicking IgG4-related disease. The patient was a 55-year-old male who was being followed for right orbital tumor over 1.5 years. The lesion had recently increased in size, so a biopsy was performed. Histologically, the lesion was consistent with IgG4-related disease ; however, IgG4+plasma cells showed immunoglobulin light-chain restriction and immunoglobulin heavy chain gene rearrangement was detected in the lesion. Therefore, the lesion was diagnosed as IgG4-producing mucosa-associated lymphoid tissue lymphoma. In conclusion, in histological diagnosis of IgG4-related disease, it is important to examine not only IgG4-immunostain but also immunoglobulin light-chain restriction.
Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan. satou-y@cc.okayama-u.ac.jp
Yasuharu
Y
Sato
Koh-Ichi
K
Ohshima
Katsuyoshi
K
Takata
Xingang
X
Huang
Wei
W
Cui
Kyotaro
K
Ohno
Tadashi
T
Yoshino
D001706:Biopsy; D003607:Dacryocystitis / Q000378:metabolism / Q000473:pathology; D003937:Diagnosis, Differential; D005134:Eye Neoplasms / Q000378:metabolism / Q000473:pathology; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007074:Immunoglobulin G / Q000096:biosynthesis*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D008882:Mikulicz' Disease / Q000378:metabolism / Q000473:pathology; D009363:Neoplasm Proteins / Q000096:biosynthesis*
D007074:Immunoglobulin G; D009363:Neoplasm Proteins