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IgG4-related Disease of the Ovary: A First Description

Sekulic, Miroslav M.D., M.A.; Pichler Sekulic, Simona M.D.; Movahedi-Lankarani, Saeid M.D.

International Journal of Gynecological Pathology: March 2017 - Volume 36 - Issue 2 - p 190–194
doi: 10.1097/PGP.0000000000000293

IgG4-related disease has been previously described to involve numerous organs and anatomic sites, however, involvement of the ovary has not yet been reported in the literature. In this case report we describe an ovary involved with an inflammatory process with histopathologic features supportive of involvement by IgG4-related disease: a dense lymphoplasmacytic infiltrate with an eosinophilic component, obliterative phlebitis, and a prominent proportion of IgG-positive cells with IgG4 expression by immunohistochemistry (40%–50%). The differential diagnosis for this case would include eosinophilic perifolliculitis involving the ovary, another rare entity that shares the eosinophilic component of IgG4-related disease. In short, we present the first description of IgG4-related disease of the ovary providing morphologic characterization and immunohistochemical studies supporting the diagnosis.

Department of Laboratory Medicine and Pathology (M.S., S.P.S., S.M.L.), University of Minnesota

Department of Pathology (S.M.L.), Abbott Northwestern Hospital, Minneapolis, Minneapolis

The authors declare no conflict of interest.

Address correspondence and reprint requests to Miroslav Sekulic, MD, MA, Department of Laboratory Medicine and Pathology, University of Minnesota, 420 Delaware Street SE, MMC 609 Mayo D185, Minneapolis, MN 55455. E-mail:

©2017International Society of Gynecological Pathologists