Multi-visceral resection of pancreatic VIPoma in a patient with sinistral portal hypertension
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Open Access
Case report
Multi-visceral resection of pancreatic VIPoma in a patient with sinistral portal hypertension David L Joyce1, Kelvin Hong2, Elliot K Fishman3, Joshua Wisell4 and Timothy M Pawlik*1 Address: 1Departments of Surgery, Johns Hopkins School of Medicine, Baltimore, MD, 22187-6681, USA, 2Department of Interventional Radiology, Johns Hopkins School of Medicine, Baltimore, MD, 22187-6681, USA, 3Department of Radiology, Johns Hopkins School of Medicine, Baltimore, MD, 22187-6681, USA and 4Department of Pathology, Johns Hopkins School of Medicine, Baltimore, MD, 22187-6681, USA Email: David L Joyce - [email protected]; Kelvin Hong - [email protected]; Elliot K Fishman - [email protected]; Joshua Wisell - [email protected]; Timothy M Pawlik* - [email protected] * Corresponding author
Published: 28 July 2008 World Journal of Surgical Oncology 2008, 6:80
doi:10.1186/1477-7819-6-80
Received: 16 April 2008 Accepted: 28 July 2008
This article is available from: http://www.wjso.com/content/6/1/80 © 2008 Joyce et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract Background: VIPomas are rare neuroendocrine tumors poorly described in the literature. Aggressive resection of patients with advanced VIPoma neuroendocrine tumors has rarely been reported. Case presentation: A 46 year old women presented with abdominal pain and diarrhea. A threedimensional (3-D) pancreas protocol computed tomography scan revealed an 18 × 12 cm pancreatic VIPoma abutting the liver, stomach, spleen, left adrenal, colon that also invaded the distal duodenum – proximal jejunum at the ligament of Treitz in association with sinistral portal hypertension. Following preoperative proximal splenic artery embolization, the patient with underwent successful en bloc resection of the locally advanced VIPoma in conjunction with a diaphragmatic resection, total gastrectomy, splenectomy, left adrenalectomy, as well as small and large bowel resection. The estimated blood loss was 500 ml. All margins were negative (R0 resection). The patient is alive and disease-free. Conclusion: This case illustrates the role of aggressive resection of pancreatic neuroendocrine tumors and highlights several key technical points that allowed for successful resection.
Background VIPomas are rare neuroendocrine tumors with an annual incidence of about 1 per 10,000,000 individuals.[1] The majority of VIPomas in adults (> 90%) are primary tumors of the pancreas.[2] As with other neuroendocrine tumors of the pancreas, on occasion these lesions can be exceptionally large with invasion of adjacent visceral and vascular structures. As such, accurate preoperative imaging is critical. In particular, assessment of the relationship between the tumor and adjacent vascular structures, such
as the portal and superior m
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