Clinical Aspects of IgG4-Related Orbital Inflammation in a Case Series of Ocular Adnexal Lymphoproliferative Disorders

Masayuki Takahira,Yoh Zen,Shoko Hamaoka,Mitsuhiro Kawano,Kazuhisa Sugiyama,Kazunori Yamada,Yoshiaki Ozawa

doi:10.1155/2012/635473

Abstract

The most frequent ocular adnexal tumors and simulating lesions are lymphoproliferative disorders (LPDs), including malignant lymphomas and orbital inflammation with lymphoid hyperplasia or infiltration. IgG4-related orbital inflammation (IgG4-ROI) often involves lacrimal glands and other orbital tissues and is an important differential diagnosis. The present study evaluated clinical aspects of IgG4-ROI in a case series of orbital LPD. Sixty-two consecutive cases of orbital LPD, pathologically diagnosed from November, 2004, through March, 2011, were investigated. Histological types were 22 cases with MALT lymphoma, 11 cases with diffuse large B-cell lymphoma (DLBCL), 3 cases with other malignant lymphomas, 16 cases with IgG4-ROI, and 10 cases with non-IgG4-ROI. Ages of the IgG4-ROI group (56 ± 10 yrs) were significantly lower than the MALT lymphoma (71 ± 12 yrs) and DLBCL (75 ± 14 yrs) groups. Orbital lesions other than lacrimal glands were present in six cases including extraocular muscle swelling, mass lesions surrounding the optic nerve, and supraorbital and infraorbital nerves enlargements. Although none of the malignant lymphomas were related to IgG4, previous evidence suggested that malignant lymphomas can arise from IgG4-ROI. Based on this study (26%) and another report (33%), it is likely that nearly a quarter of orbital LPD are IgG4-ROI.

Highlights

The most frequent tumors and simulating lesions in ocular adnexa are lymphoproliferative disorders (LPDs), including malignant lymphomas and orbital inflammation with lymphoid hyperplasia or infiltration, some of which are historically called “orbital pseudotumors.” In Japanese studies, Goto [1] reported that the rate of lymphoproliferative diseases (LPD) among 409 cases with orbital tumors and simulating lesions was 43%, while Ohtsuka et al [2] described that the rate was 49% out of 213 cases
In the United States, LPD is a common orbital disease the rate seems to be somewhat lower: 24% of LPD out of 703 cases with orbital lesions was reported by Shields et al [3], and 26% of LPD out of 268 cases was reported by Shinder et al [4]
None of the malignant lymphomas showed a relationship with IgG4 in this case series

Summary

Introduction

The most frequent tumors and simulating lesions in ocular adnexa are lymphoproliferative disorders (LPDs), including malignant lymphomas and orbital inflammation with lymphoid hyperplasia or infiltration, some of which are historically called “orbital pseudotumors.” In Japanese studies, Goto [1] reported that the rate of LPD among 409 cases with orbital tumors and simulating lesions was 43%, while Ohtsuka et al [2] described that the rate was 49% out of 213 cases. The most frequent tumors and simulating lesions in ocular adnexa are lymphoproliferative disorders (LPDs), including malignant lymphomas and orbital inflammation with lymphoid hyperplasia or infiltration, some of which are historically called “orbital pseudotumors.”. In Japanese studies, Goto [1] reported that the rate of LPD among 409 cases with orbital tumors and simulating lesions was 43%, while Ohtsuka et al [2] described that the rate was 49% out of 213 cases. When patients with suspected orbital LPD are encountered, tissue biopsy is preferred since image examinations alone cannot distinguish inflammatory lesions from malignant lymphomas. It was elucidated that IgG4related orbital inflammatory lesions include other ocular adnexal tissues such as extraocular muscles [9] and periorbital membrane [10]. An orbital LPD case series was investigated and clinical

Methods

Results

Conclusion