J Pathol Transl Med.  2019 Mar;53(2):125-128. 10.4132/jptm.2018.10.25.

Coexisting Mucinous Cystic Neoplasm of the Pancreas and Type 1 Autoimmune Pancreatitis

Affiliations
  • 1Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. smhong28@gmail.com
  • 2Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
  • 3Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
  • 4Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Abstract

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.

Keyword

Pancreas; Mucinous cystic neoplasm; Autoimmune pancreatitis

MeSH Terms

Epithelial Cells
Female
Fibrosis
Follow-Up Studies
Humans
Mucins*
Pancreas*
Pancreatectomy
Pancreatitis*
Phlebitis
Plasma Cells
Receptors, Progesterone
Tail
Mucins
Receptors, Progesterone

Figure

  • Fig. 1. (A) Coronal contrast-enhanced computed tomography image showing a unilocular cyst in the pancreas tail. (B) Six years later, the cyst had grown, with its longest diameter increasing from 2.2 to 3.5 cm, as well as thickening of the cyst wall (arrowheads). (C) The unilocular cyst in the body and tail of the pancreas showing irregular thickening of the cyst wall. (D) Scanning power image showing lymphoid follicles and lymphoplasmacytic infiltration around the cyst wall. (E) The cyst wall was lined mostly by non-mucinous and focally mucinous epithelial cells with mild cytologic atypia. (F) Progesterone receptor nuclear labeling highlighted ovarian-type stromal cells. (G) The irregularly thickened cyst wall contained multiple lymphoid follicles in a background of chronic inflammatory cell infiltrations. (H) Some areas showed dense periductal lymphoplasmacytic infiltrations. (I, J) Several foci of obliterative phlebitis were noted by hematoxylin and eosin staining (I) and elastic staining (J). (K, L) Areas of dense lymphoplasmacytic infiltrations (K) showed numerous IgG4-positive plasma cell infiltrations (L).


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