IgG4-Related Disease and Malignant Tumor
IgG4-related disease (IgG4-RD) is considered to be a benign disease responsive to glucocorticoid therapy, but recently cases complicated by malignant tumors have been described. The potential association between IgG4-RD and malignancy that is suggested by these cases has attracted growing attention. We describe a patient with mucinous cystadenocarcinoma of the appendix, during the course of which autoimmune pancreatitis developed and improved with glucocorticoid therapy. After cessation of the glucocorticoid treatment, the appendix cancer recurred, accompanied by the return of an elevated serum IgG4 concentration. The patient ultimately succumbed to the peritoneal dissemination of his appendiceal cancer. A postmortem examination revealed numerous IgG4-positive plasma cells within the kidneys, and findings consistent with IgG4-RD were also noted in the pancreas, submandibular glands, and prostate. This case and others similar to it in the literature suggest that the potential relationship between IgG4-RD and malignancy requires further investigation.
KeywordsCommon Bile Duct Peritoneal Dissemination Autoimmune Pancreatitis Bile Duct Wall Mucinous Cystadenocarcinoma
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