Precious Data: Interim Report from the Jefferson Pancreas Tumor Registry.

Purpose: The Jefferson Pancreas Tumor Registry (JPTR) is a voluntary hospital-based registry of persons with pancreas and related periampullary cancers, premalignant lesions, and nonaffected family members (NAFMs). The ultimate goals of the JPTR are to provide a link between family history, gene mutations, and precision medicine therapy, and to identify high-risk NAFMs for potential surveillance screening. Methods: The JPTR is an Institutional Review Board approved longitudinal epidemiological study housed in the Department of Surgery at Thomas Jefferson University Hospital. Individuals who met the eligibility criteria and signed informed consent provide information on hereditary conditions, family history of cancers, environmental exposures, and occupational risk factors. Data are collected using a self-administered questionnaire, the electronic medical record, and the molecular analysis of tumor specimens. Results: Established in 2008, >725 persons have enrolled in the JPTR. The cohort is mostly composed of sporadic pancreas cancer, with 13% of enrollees having familial pancreas cancer and a control group comprising nonaffected persons. Data from the registry have been utilized to inform clinical studies, molecular investigations, and to shed light on and gain insight into the lived experience of persons with these conditions. Conclusion: The JPTR contains precious qualitative data and is an invaluable repository of information about persons with pancreatic and related tumors.

Congenital Variants of Gastrointestinal Rotation Found at Resection of Hepatopancreatobiliary Tumors: A Case Series with Review of the Literature.

Background: Gastrointestinal malrotation arises from intrauterine events that occur early in the first trimester of gestation, and can result in a midgut volvulus that classically presents in the neonatal period with bilious emesis. Gastrointestinal malrotation can present clinically with symptoms such as chronic abdominal pain or bowel obstruction, or remain completely asymptomatic only to be discovered as an incidental finding much later in life during surgical exploration for other diseases. We sought to identify the prevalence of gastrointestinal malrotation in patients undergoing surgical exploration for hepatopancreatobiliary (HPB) malignancy and describe the operative considerations of these cases. Case Presentation: We performed a retrospective review of our prospectively acquired HPB surgery database from January 1, 2006, to December 1, 2013. We identified three cases of gastrointestinal malrotation out of a total of 1220 HPB cases reviewed, which represents 0.2%. We found two cases of gastrointestinal malrotation in the setting of pancreatic ductal adenocarcinoma and one case in the setting of cholangiocarcinoma. All three patients underwent exploratory laparotomy with resection of their respective primary tumors. We searched the English literature for cases of HPB malignancy in the setting of gastrointestinal malrotation. Conclusion: Our case series and review of the literature underscore the rarity and complexity of these cases.

Safety and efficacy of LigaSure usage in pancreaticoduodenectomy.

BACKGROUND: Over recent years, use of the LigaSure™ vessel sealing device has increased in major abdominal surgery to include pancreaticoduodenectomy (PD). LigaSure™ use during PD has expanded to include all steps of the procedure, including the division of the uncinate margin. This introduces the potential for thermal major vascular injury or margin positivity. The aim of the present study was to evaluate the safety and efficacy of LigaSure™ usage in PD in comparison to established dissection techniques. METHODS: One hundred and forty-eight patients who underwent PD from 2007 to 2012 at Robert Wood Johnson University Hospital were identified from a retrospective database. Two groups were recognized: those in which the LigaSure™ device was used (N = 114), and in those it was not (N = 34). Peri-operative outcomes were compared. RESULTS: Vascular intra-operative complications directly caused by thermal injury from LigaSure™ use occurred in 1.8% of patients. Overall vascular intra-operative complications, uncinate margin positivity, blood loss, length of stay, and complication severity were not significantly different between groups. The mean operative time was 77 min less (P < 0.010) in the LigaSure™ group. Savings per case where the LigaSure™ was used amounted to $1776.73. CONCLUSION: LigaSure™ usage during PD is safe and effective. It is associated with decreased operative times, which may decrease operative costs in PD.

The novel triad of dorsal agenesis of the pancreas with concurrent pancreatic ductal adenocarcinoma and nonalcoholic chronic calcific pancreatitis: a case series and review of the literature.

INTRODUCTION: Dorsal agenesis of the pancreas (DAP) is a rare congenital anomaly, with only 44 cases having been reported in the English literature since 1966. MATERIALS AND METHODS: A retrospective review of our IRB-approved pancreatic surgery database was performed from November 2005 to November 2010 searching for cases of DAP. DISCUSSION: Disorders in the retinoic acid (Raldh) and hedgehog (Hh) signaling pathways, which appear to play a role in the development of DAP, have been implicated in other diseases of the pancreas such as pancreatic ductal adenocarcinoma (PDA) and nonalcoholic chronic calcific pancreatitis (NCCP). CONCLUSION: In this report, we describe three cases of DAP in the setting of PDA, two of which include the third component of NCCP. We provide a discussion of the clinical features of this novel triad and address the molecular pathways that relate to these respective diseases.

Reconstruction following the pylorus preserving Whipple resection: PJ, HJ, and DJ.

INTRODUCTION: Pancreaticoduodenectomy is one of the most challenging procedures performed by general surgeons. DISCUSSION: Many studies have been performed looking at the technical aspects of reconstruction after pancreaticoduodenal resection. Multiple randomized trials have failed to convincingly demonstrate the superiority of any single approach or technique. Here, we illustrate our approach to reconstruction, with an emphasis on technical aspects and details. CONCLUSION: The fine points can help avoid technical errors that result in anastomotic failure.