- Coexisting Mucinous Cystic Neoplasm of the Pancreas and Type 1 Autoimmune Pancreatitis
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Mee-Jeong Kim, Tae Jun Song, Hyoung Jung Kim, Song-Cheol Kim, Myung-Hwan Kim, Seung-Mo Hong
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J Pathol Transl Med. 2019;53(2):125-128. Published online November 14, 2018
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DOI: https://doi.org/10.4132/jptm.2018.10.25
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- 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 
- 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 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
- Rosai-Dorfman Disease: Report of a Case Associated with IgG4-Related Sclerotic Lesions
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Bong-Hee Park, Da Hye Son, Myung-Hwan Kim, Tae Sun Shim, Hee Jin Lee, Jooryung Huh
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Korean J Pathol. 2012;46(6):583-586. Published online December 26, 2012
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DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.6.583
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7,755
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53
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We describe a rare case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) associated with a six-year history of autoimmune pancreatitis, which was controlled by steroid treatment. The patient presented with multiple, cervical and thoracic lymphadenopathy and abnormal, nodular opacities in the lung. Histologically, Rosai-Dorfman disease with numerous IgG4-positive cells was identified in a subcutaneous lymph node in the patient's left forearm. The patient recovered uneventfully with steroid treatment.
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Citations
Citations to this article as recorded by 
- Lung Involvement in Destombes-Rosai-Dorfman Disease
Quentin Moyon, Samia Boussouar, Philippe Maksud, Jean-François Emile, Frédéric Charlotte, Nathalie Aladjidi, Grégoire Prévot, Jean Donadieu, Zahir Amoura, Philippe Grenier, Julien Haroche, Fleur Cohen Aubart Chest.2020; 157(2): 323. CrossRef - Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature
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