Type 1 autoimmune pancreatitis (AIP1) is an IgG4-related systemic disease that mimics tumors. We report a rare case of AIP1 accompanied by mucinous cystic neoplasm (MCN). A pancreatic lesion was incidentally detected in a woman in her 60s. After 6 years of follow-up, the lesion abruptly increased in size. Computed tomography showed a 3.5 cm unilocular cyst in the tail of the pancreas and distal pancreatectomy was performed. On microscopic examination, the cyst was lined by mucinous and non-mucinous epithelial cells with mild cytologic atypia. The surrounding stroma comprised ovarian-type spindle cells with progesterone receptor positivity. The pericystic pancreas exhibited multifocal lymphoid follicles, lymphoplasmacytic infiltrations, obliterative phlebitis, and storiform fibrosis. IgG4-positive plasma cell infiltration (215 cells high-power field) and the IgG4/IgG ratio (57%) were increased. Cases of MCN coexisting with AIP1 are extremely rare; only two such cases have been reported in the English-language literature. This third case featured low-grade MCN with AIP1.
Citations
Citations to this article as recorded by
Utilizing Immunoglobulin G4 Immunohistochemistry for Risk Stratification in Patients with Papillary Thyroid Carcinoma Associated with Hashimoto Thyroiditis Faridul Haq, Gyeongsin Park, Sora Jeon, Mitsuyoshi Hirokawa, Chan Kwon Jung Endocrinology and Metabolism.2024; 39(3): 468. CrossRef
Histological features of autoimmune pancreatitis and IgG4-related sclerosing cholangitis with a correlation with imaging findings Kenji NOTOHARA Choonpa Igaku.2023; 50(1): 55. CrossRef
Imaging Features and Risk Factors of Pancreatic Cystic Lesions Complicating
Autoimmune Pancreatitis: A Retrospective Study Bin-Bin Zhang, Xin-Meng Hou, Yu-Qi Chen, Jian-Wei Huo, Er-Hu Jin Current Medical Imaging Formerly Current Medical Imaging Reviews.2023;[Epub] CrossRef
Histological features of autoimmune pancreatitis and IgG4-related sclerosing cholangitis with a correlation with imaging findings Kenji Notohara Journal of Medical Ultrasonics.2021; 48(4): 581. CrossRef
Mucinous cystic neoplasm of the pancreas with type-1 autoimmune pancreatitis-like lesion Kevin Gowing, David F. Schaeffer, Hui-Min Yang Human Pathology: Case Reports.2019; 18: 200339. CrossRef
Mucious cystic neoplasm of pancreas is a cystic neoplasm composed of columnar, mucin-producing epithelium and is supported by ovarian-type stroma. The key to the cytologic evaluation of pancreatic cystic lesions is to recognize the cytologic components as being diagnostic of a mucin-producing cystic neoplasm, as all of these neoplasms need to be resected. We report the use of fine needle aspiration cytology in the diagnosis of an invasive mucinous cystic carcinoma confirmed by partial pancreatectomy. The cytologic specimen showed a abundant mucin background and sheets or papillae of neoplastic cells. There are mucin-containing columnar cells that show a variable degree of cytologic atypia.