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Ocular Adnexal Lymphoma Associated With IgG4+ Chronic Sclerosing Dacryoadenitis: A Previously Undescribed Complication of IgG4-related Sclerosing Disease

Cheuk, Wah MBBS*; Yuen, Hunter K. L. MBChB; Chan, Alexander C. L. MBBS*; Shih, Lee-Yung MD; Kuo, Tseng-Tong MD§; Ma, Ming-Wai MBBS; Lo, Yan-Fai MBChB; Chan, Wai-Kong MBChir; Chan, John K. C. MBBS*

The American Journal of Surgical Pathology: August 2008 - Volume 32 - Issue 8 - p 1159-1167
doi: 10.1097/PAS.0b013e31816148ad
Original Articles

IgG4-related sclerosing disease is a recently recognized inflammatory lesion frequently involving pancreas, submandibular gland, lacrimal gland, and lymph node. We report 3 cases of ocular adnexal lymphoma arising in IgG4-related chronic sclerosing dacryoadenitis, a phenomenon that has not been previously reported. The patients presented with bilateral or unilateral ocular adnexal mass usually present for many years. One patient also had asymptomatic diffuse lymphadenopathy. Two patients had biopsy-proven IgG4-related chronic sclerosing dacryoadenitis before the current presentation, and 1 had systemic involvement by IgG4-related sclerosing disease as evidenced by increased IgG4+ cells in a prior nasopharyngeal biopsy. Two cases showed features of extranodal marginal zone lymphoma of mucosa-associated lymphoid-tissue type (1 with large cell transformation) and 1 follicular lymphoma. Thus, the lymphoid hyperplasia of IgG4-related sclerosing disease can provide a substrate for the emergence of lymphoma. In addition, we report 3 cases of ocular adnexal extranodal marginal zone B-cell lymphoma that show sclerosing inflammation in the background and numerous IgG4+ monotypic plasma cells. In the absence of prior biopsies or information on serum IgG4 titer, it is unclear whether these cases represent lymphoma complicating IgG4-related sclerosing disease or de novo lymphoma. Nonetheless, these cases are distinctive in that the neoplastic cells express IgG4 (light chain restricted), whereas unselected cases of ocular adnexal lymphomas do not show IgG4 expression.

*Department of Pathology, Queen Elizabeth Hospital

Department of Ophthalmology and Visual Sciences, Chinese University of Hong Kong, Hong Kong Eye Hospital

Department of Pathology, Pamela Youde Nethersole Eastern Hospital

Department of Pathology, Caritas Medical Center, Hong Kong

Department of Pathology, Hong Kong Sanatorium and Hospital

Departments of Hematology-Oncology

§Pathology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taiwan

Correspondence: Dr Wah Cheuk, MBBS, Department of Pathology, Queen Elizabeth Hospital, Wylie Road, Kowloon, Hong Kong (e-mail:

© 2008 Lippincott Williams & Wilkins, Inc.