Immunoglobulin G4-related disease mimicking an appendiceal neoplasm: a case report

Author:

Kim Dongwoo1,Lee Jongwon1,Jung Minsoo1,Lee Su Lim2,Yim Kwangil3,Chong Yosep3,Alam Mohammad Rizwan3,Seo Kyung Jin3ORCID

Affiliation:

1. College of Medicine, The Catholic University of Korea, Seoul, Korea

2. Department of Radiology, College of Medicine, The Catholic University of Korea, Seoul, Korea

3. Department of Hospital Pathology, College of Medicine, The Catholic University of Korea, Seoul, Korea

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by chronic activation of the immune system and a tendency to form tumorous lesions. IgG4-RD is frequently characterized by the presence of tumor-like masses affecting multiple organs and is easily mistaken for a malignant neoplasm. However, IgG4-RD affecting the appendix is extremely rare, with only seven cases reported previously. We report the case of a woman in her early 60s who presented with insidious abdominal pain and radiological findings mimicking appendiceal neoplasms. After diagnosing appendiceal neoplasms, surgery was performed. The patient had a serum IgG4 concentration of <1.35 g/L, which did not satisfy one of the three revised comprehensive diagnostic criteria for IgG4-RD. A pathological examination was conducted, and the patient was diagnosed with appendiceal IgG4-RD. To the best of our knowledge, there have been no previously reported cases of IgG4-RD affecting the appendix in patients with low serum IgG4 concentrations. This report may prove beneficial for the future understanding of IgG4-RD and for the revision of diagnostic and treatment strategies.

Funder

College of Medicine, Catholic University of Korea

Publisher

SAGE Publications

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