Clinicopathological Characteristics of IgG4-Related Disease Involving Uterus and Abdominopelvic Lymph Nodes: A Case Report.
Gong Qingyun, Wang Yuanyuan
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Case in brief
A 51-year-old woman with prolonged menstruation had PET/CT findings of hypermetabolic uterine masses and multiple abdominopelvic lymph nodes, initially raising concern for malignancy. After total hysterectomy and lymph node biopsy, histology showed storiform fibrosis, obliterative phlebitis, and dense lymphoplasmacytic infiltrates containing abundant IgG4-positive plasma cells. Markedly elevated serum IgG4 and IgG supported IgG4-related disease (IgG4-RD). Following postoperative treatment with dexamethasone, cyclophosphamide, and bortezomib, serum IgG4 levels substantially decreased.
What made this case unusual
IgG4-RD involved both the uterus and abdominopelvic lymph nodes, with imaging that initially suggested malignancy.
Diagnostic / clinical pearl
In atypical pelvic masses, consider IgG4-RD and assess tissue morphology, IgG4-positive plasma cells, and serum immunoglobulin levels together.