Pancreatic involvement in von Hippel–Lindau disease

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Pancreatic involvement in von Hippel–Lindau disease Pascal R. Hammel, Valerie Vilgrain, Benoit Terris, Alfred Penfornis, Alain Sauvanet, Jean–Michel Correas, Dominique Chauveau, Axel Balian, Catherine Beigelman, Dermot O'Toole, Pierre Bernades, Philippe Ruszniewski, Stephane Richard Gastroenterology Volume 119, Issue 4, Pages 1087-1095 (October 2000) DOI: 10.1053/gast.2000.18143 Copyright © 2000 American Gastroenterological Association Terms and Conditions

Fig. 1 (A) CT scan. Multiple pancreatic cysts in a 34-year-old woman with VHL disease. (B) CT scan of the same patient 5 years later. The cysts have increased markedly (arrowhead), causing gastric compression and appearance of NET (white arrows) in the head of the pancreas. This figure illustrates the clear difference on CT after contrast injection between NET and serous cystadenomas, which were multifocal in this patient. (C) Macroscopic resected specimen (same patient as in A and B) shows an NET invading the duodenal wall (arrowhead), accompanied by lymph node metastases. Multifocal serous cystadenomasare are also present (white arrows). Gastroenterology 2000 119, 1087-1095DOI: (10.1053/gast.2000.18143) Copyright © 2000 American Gastroenterological Association Terms and Conditions

Fig. 2 (A) Suspicion of a vascularized tumor in the head of the pancreas (arrows) in a 27-year-old patient with VHL disease. Intraoperative biopsy results were negative. (B) CT portography in the same patient 6 years later shows significant progression of the pancreatic tumor (arrows) and multiple liver metastases. The diagnosis of NET was established after analysis of endoscopic biopsy specimens obtained from a large ulcer of the second part of duodenum resulting from tumor extension. Gastroenterology 2000 119, 1087-1095DOI: (10.1053/gast.2000.18143) Copyright © 2000 American Gastroenterological Association Terms and Conditions

Fig. 3 CT scan (after contrast injection) showing a large cyst of the pancreas (*) resulting in abdominal pain, leading to a diagnosis of VHL disease. A left renal tumor was also present. Gastroenterology 2000 119, 1087-1095DOI: (10.1053/gast.2000.18143) Copyright © 2000 American Gastroenterological Association Terms and Conditions

Fig. 4 Example of histologic analysis (original magnification 3×) of a resected specimen showing the multicentricity of NETs in a patient with VHL. Adenomas of various size are indicated by black arrows. Gastroenterology 2000 119, 1087-1095DOI: (10.1053/gast.2000.18143) Copyright © 2000 American Gastroenterological Association Terms and Conditions