Abstract
Background and aim
Von Hippel-Lindau disease is associated with serous cysts in the pancreas and kidneys.
In this study we determined the prevalence of pancreatic cysts occurring concurrently
with other abdominal cysts and tested the hypothesis that these patients might represent
a forme fruste of Von Hippel-Lindau disease and be more likely to be serous cysts.
Methods
A retrospective chart review of patients undergoing endoscopic ultrasound of pancreatic
cysts.
Results
A total of 156 patients were included in the study. Eighty-five patients (54.8%) had
cyst(s) in the pancreas and at least one other intra-abdominal cyst. These cysts included
24 (27.9%) serous cysts, 30 (34.9%), mucinous cysts, 6 (7%) adenocarcinoma and 25
(29.4%) unknowns. Seventy-one patients (45.2%) had isolated pancreatic cysts. These
included 17 (23.9%) serous cysts, 28 (39.5%) mucinous cysts, 4 (5.6%) adenocarcinoma
and 22 (31%) unknowns. The odds of serous cysts with concurrent extra-pancreatic and
pancreatic cysts compared to odds of serous cysts with pancreatic cysts alone were
1.3 (95% CI: 0.6–2.9).
Conclusions
Pancreatic cysts are associated with cysts in other abdominal organs in 54.8% patients.
The prevalence of serous cysts was not higher amongst individuals with multiple organ
cysts compared to those with only pancreatic cysts.
Abbreviations:
CEA (carcinoembryonic antigen), CI (confidence interval), CT (computerized tomography), EUS (endoscopic ultrasound), FNA (fine needle aspiration), IPMN (intrapapillary mucinous neoplasm), MCN (mucinous cystic neoplasm), MRI (magnetic resonance imaging), MRCP (magnetic resonance cholangiopancreatography), SCA (serous cystadenoma), VHL (Von Hippel-Lindau disease)Keywords
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References
- Pancreatic cysts.Gastrointest Endosc. 2009; 69: S110-S116
- Cystic neoplasms of the pancreas.Pancreatology. 2001; 1: 641-647
- Pancreatic involvement in von Hippel-Lindau disease: the Groupe Francophone d’Etude de la Maladie de von Hippel-Lindau.Gastroenterology. 2000; 119: 1087-1095
- Allelic deletion and mutation of the von Hippel-Lindau (VHL) tumor suppressor gene in pancreatic microcystic adenomas.Am J Pathol. 1997; 151: 951-956
- Molecular characterization of pancreatic serous microcystic adenomas: evidence for a tumor suppressor gene on chromosome 10q.Am J Pathol. 2001; 158: 317-321
- Histopathology and molecular genetics of multiple cysts and microcystic (serous) adenomas of the pancreas in von Hippel-Lindau patients.Am J Pathol. 2000; 157: 1615-1621
- Pancreatic involvement in von Hippel-Lindau disease: report of two cases and review of the literature.Am J Gastroenterol. 2006; 101: 2655-2658
- Diagnosis of pancreatic cystic neoplasms: a report of the cooperative pancreatic cyst study.Gastroenterology. 2004; 126: 1330-1336
- Germline mutation profile of the VHL gene in von Hippel-Lindau disease and in sporadic hemangioblastoma.Hum Mutat. 1998; 12: 424-430
- Mutations in the RET proto-oncogene and the von Hippel-Lindau disease tumour suppressor gene in sporadic and syndromic phaeochromocytomas.J Med Genet. 1995; 32: 934-937
- The incidence of simple renal cyst by computed tomography.Tada Clin Radiol. 1983; 34: 437-439
- Rapid MR imaging detection of renal cysts: age-based standards.Radiology. 2001; 221: 628-632
- Characterization of cystic pancreatic masses: relative accuracy of CT and MRI.Am J Roentgenol. 2007; 189: 648-656
- Comparative performance of MDCT and MRI with MR cholangiopancreatography in characterizing small pancreatic cysts.Am J Roentgenol. 2009; 193: 722-731
Article info
Publication history
Published online: June 17, 2011
Accepted:
May 3,
2011
Received:
July 26,
2010
Identification
Copyright
© 2011 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Inc. All rights reserved.
