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Presented by Ralph Hruban, M.D. and prepared by Matthew Karafin, M.D.
Case 3: This patient presented with abdominal symptoms and was found to have a diffuse enlargement of the head of the pancreas.
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1. Question
Week 501: Case 3
This patient presented with abdominal symptoms and was found to have a diffuse enlargement of the head of the pancreas. A Whipple was performed.images/1alex/102411case3image2.jpg
images/1alex/102411case3image1.jpg
images/1alex/102411case3image4.jpg
images/1alex/102411case3image3.jpgCorrect
Answer: Autoimmune pancreatitis
Histology: Several dilated vascular spaces with thin walls are present in the duodenum (image 1). These are filled with uniform cells with abundant cytoplasm. The pancreas is remarkable for a duct-centric mixed inflammatory infiltrate and a venulitis (image 2, 3, and 4). Immunolabeling for CD68 labels the cells within the dilated vascular spaces, and an immunostain for IgG4 labels increased numbers of cells in the pancreas (not shown).
Discussion: The changes in the pancreas, including the increased numbers of IgG4 expressing plasma cells, are diagnostic of autoimmune pancreatitis. The cells in the dilated vessels are striking and mimic Langerhans cell histiocytosis, but were proven to be macrophages on immunolabeling. We have seen several other cases like this, and believe that the accumulation of macrophages within vessels is secondary to the autoimmune pancreatitis, perhaps local vascular obstruction from the venulitis.
Incorrect
Answer: Autoimmune pancreatitis
Histology: Several dilated vascular spaces with thin walls are present in the duodenum (image 1). These are filled with uniform cells with abundant cytoplasm. The pancreas is remarkable for a duct-centric mixed inflammatory infiltrate and a venulitis (image 2, 3, and 4). Immunolabeling for CD68 labels the cells within the dilated vascular spaces, and an immunostain for IgG4 labels increased numbers of cells in the pancreas (not shown).
Discussion: The changes in the pancreas, including the increased numbers of IgG4 expressing plasma cells, are diagnostic of autoimmune pancreatitis. The cells in the dilated vessels are striking and mimic Langerhans cell histiocytosis, but were proven to be macrophages on immunolabeling. We have seen several other cases like this, and believe that the accumulation of macrophages within vessels is secondary to the autoimmune pancreatitis, perhaps local vascular obstruction from the venulitis.
