1. Mass-forming immunoglobulin G4-related disease shows indolent clinical course after surgical resection, clinicopathological analysis of a series of 15 cases.
- Author
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Shi R, Farah BL, Xu C, Yeong JPS, Kuick CH, Goh JY, Chang KTE, and Takano A
- Subjects
- Female, Fibrosis, Humans, Immunoglobulin G, Male, Middle Aged, Plasma Cells pathology, Retrospective Studies, Immunoglobulin G4-Related Disease diagnosis, Immunoglobulin G4-Related Disease pathology
- Abstract
The purpose of this study is to characterize the clinicopathological features of mass-forming immunoglobulin G4-related disease (IgG4-RD). A retrospective search for cases of mass-forming IgG4-RD diagnosed at Singapore General Hospital between 2008 and 2019 was performed. A total of 15 cases of mass-forming IgG4-RD were identified. The male-to-female ratio was 2.5:1, and the median age was 61 years old. The majority of cases showed a solitary lesion (12/15) with a mean size of 35 mm. IgG4-RD was considered as a clinical differential diagnosis only in one case (1/15) prior to the surgical resection. Diagnostic histopathological features, such as dense lymphoplasmacytic infiltrate positive for IgG4 plasma cells (15/15), storiform fibrosis (15/15), and obliterative phlebitis (9/15), were observed in most cases. These findings were distributed heterogeneously within the lesions. Cases with single organ involvement showed a low relapse rate (2/10) and normal serum IgG4 level after surgical resection. Mass-forming IgG4-RD has a male predilection and involves various organ systems. It may be initially misdiagnosed as malignancy and undergo surgical resection. The diagnostic histological features of IgG4-RD are readily identified in different organs. However, they may be distributed heterogeneously within a single lesion. Cases of single organ involvement show an indolent clinical course and normal serum IgG4 level after surgical resection., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
- Published
- 2022
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