Korean J Gastroenterol.  2020 Jun;75(6):356-361. 10.4166/kjg.2020.75.6.356.

Metachronous Development of Peritoneal Carcinomatosis in a Patient with Autoimmune Pancreatitis

• Lee KW
• Chang JH
• Kim J
• Kim TH
• Kim CW
• Kim JK
• Han SW

• KMID: 2504379
• DOI: http://doi.org/10.4166/kjg.2020.75.6.356

Abstract

Autoimmune pancreatitis (AIP) is a rare and unique type of chronic pancreatitis. The prognosis of AIP, particularly when associated with pancreatic cancer or a related malignancy, is not known. Only a few cases, where metachronous pancreas-related cancer developed during follow-up, have been reported. Most of these patients either underwent surgery or steroid therapy. This paper reports a case of a 66-year-old woman with untreated type I AIP who developed peritoneal carcinomatosis more than 2 years later. Initially, the patient had a markedly elevated serum IgG4 level and a diffuse, infiltrative mass-like lesion in the pancreatic head, in which the biopsy results were consistent with type I AIP. The patient was not treated with steroids because of a cerebellar infarction. Twenty-eight months after the diagnosis of AIP, peritoneal carcinomatosis developed without noticeable changes in the pancreas from the initial findings.

Keyword

Autoimmune pancreatitis; Immunoglobulin G4-related disease; Pancreatic neoplasms; Peritoneal neoplasms

Figure

• 

  Fig. 1 At the initial diagnosis, (A, B) computed tomography revealed the diffuse enlargement of the pancreatic head and body with peripancreatic infiltration, and (C) magnetic resonance cholangiopancreatography shows multiple strictures of the main pancreatic duct (arrow) without marked upstream dilatation. Histopathological findings of the pancreatic head showing (D) storiform fibrosis, (E) periductal lymphoplasmacytic infiltration, (F) obliterative phlebitis (D-F: H&E, ×400), and (G) positive immunoglobulin G4 staining (×400).

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  Fig. 2 Abdominal computed tomography (CT) findings at 14 months. A CT scan revealed some resolution of the peripancreatic infiltration, but there was still prominent pancreatic enlargement of the pancreatic head with a diffuse narrowing of the superior mesenteric vein and splenic vein and the collateral vessels.

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  Fig. 3 At 27 months, (A, B) abdominal computed tomography revealed no obvious change in the pancreas during follow-up, but (C) peritoneal nodularities (arrows) were identified. (D) The laparoscopy shows multiple whitish nodules on the peritoneum. (E, F) The histopathology of the nodules shows well-differentiated adenocarcinoma of a pancreaticobiliary origin (E: H&E, ×100; F: cytokeratin-7, ×200).

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  Fig. 4 Timeline of patient events. mo, months.

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