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Case Study
- Coexisting Mucinous Cystic Neoplasm of the Pancreas and Type 1 Autoimmune Pancreatitis
- Mee-Jeong Kim, Tae Jun Song, Hyoung Jung Kim, Song-Cheol Kim, Myung-Hwan Kim, Seung-Mo Hong
- J Pathol Transl Med. 2019;53(2):125-128. Published online November 14, 2018
- DOI: https://doi.org/10.4132/jptm.2018.10.25
- 7,843 View
- 115 Download
- 2 Web of Science
- 5 Crossref
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Abstract
PDF - 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.
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Citations
Citations to this article as recorded by
- 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 - 自己免疫性膵炎診療ガイドライン2020
Suizo.2020; 35(6): 465. 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
- Histological features of autoimmune pancreatitis and IgG4-related sclerosing cholangitis with a correlation with imaging findings
Original Article
- Idiopathic Duct Centric Pancreatitis in Korea: A Clinicopathological Study of 14 Cases.
- Hyo Jeong Kang, Tae Jun Song, Eunsil Yu, Jihun Kim
- Korean J Pathol. 2011;45(5):491-497.
- DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.5.491
- 3,393 View
- 17 Download
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Abstract
PDF
- BACKGROUND
Idiopathic duct centric pancreatitis (IDCP) is a subtype of autoimmune pancreatitis (AIP) that is histologically characterized by granulocytic epithelial lesion and scarce IgG4-positive cells. This subtype of AIP has not been documented in Asian countries.
METHODS
We reviewed 38 histologically confirmed AIP cases and classified them into lymphoplasmacytic sclerosing pancreatitis (LPSP) and IDCP. Then, clinicopathological characteristics were compared between LPSP and IDCP.
RESULTS
Fourteen cases (36.8%) were IDCP. IDCP affected younger patients more than LPSP. IDCP was associated with ulcerative colitis in 35.7% of cases, whereas LPSP was associated with IgG4-related sclerosing diseases such as cholangitis, retroperitoneal fibrosis or sialadenitis in 41.7% of cases. IDCP was microscopically characterized by neutrophilic ductoacinitis with occasional granulocytic epithelial lesions, whereas LPSP was characterized by storiform inflammatory cell-rich fibrosis and obliterative phlebitis. IgG4-positive cells were not detected in any IDCP case but more than 20 IgG4-positive cells per high-power-field were invariably detected in LPSP cases. All patients with IDCP responded dramatically to steroids without recurrence, whereas 33.3% of patients with LPSP developed recurrences.
CONCLUSIONS
IDCP is clinicopathologically distinct from LPSP and can be diagnosed when neutrophilic ductoacinitis or granulocytic epithelial lesions are observed in a pancreatic biopsy under the appropriate clinical setting.
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