Background: Synchronous cystic lesions of the pancreas with different pathophysiology in the same patient are a rare occurrence.. Case Presentation: We report the incidental finding of a multicystic lesion within the pancreatic head in a morbidly obese woman during workup for bariatric surgery. The lesion contained an intraductal papillary mucinous neoplasm (IPMN) with high-grade dysplasia within an acinar cell cystadenoma (ACA). ACAs are rare tumors first described in 2002. Conclusion: To date, there have been no published reports of synchronous IPMN within an ACA. This case report intends to increase provider awareness of these lesions as well as highlight the importance of surveillance and careful histological examination of heterogeneous cystic lesions of the pancreas.
Background: Paraganglionic neoplasms that originate in the adrenal medullas are referred to as pheochromocytomas, but if they arise from other paraganglia scattered throughout the body, they are referred to as paragangliomas. Pancreatic paragangliomas are an extremely rare entity as only 20 cases have been reported in the literature. They tend to be nonfunctional and typically occur in the fourth to fifth decade of life without a gender predilection. We describe in this study a case of a pancreatic paraganglioma and its CT appearance. Case Presentation: A 62-year-old woman undergoing presurgical evaluation for an olfactory groove meningioma resection was incidentally found to have a pancreatic mass. Multiple fine needle aspirations of the mass through endoscopic ultrasound yielded only atypical epithelial cells. The mass demonstrated avid enhancement on serial CTs with mild interval growth over a period of 5 years. No lymphadenopathy was ever found. The patient's complete blood count, complete metabolic panel, and plasma carcinoembryonic antigen levels were all within normal limits. Urine catecholamine metabolite levels were never checked as the patient demonstrated no symptoms of catecholamine excess. The patient underwent a laparoscopic distal pancreatectomy and splenectomy, and the mass was eventually diagnosed as a pancreatic paraganglioma through pathology. While the patient tolerated the surgery well, she did require a biliary sphincterotomy and placement of a pancreatic duct stent postoperatively for treatment of a pancreatic duct leak, which completely resolved. She showed no evidence of disease recurrence on multiple subsequent CTs and continues to do well. Conclusion: Pancreatic paragangliomas are usually incidentally discovered and typically demonstrate avid homogenous enhancement on contrast-enhanced CT or MR. Aggressive surgical resection is necessary to maximize the chances of disease-free survival. Pancreatic paragangliomas are similar histologically, whether benign or malignant, to paragangliomas that occur anywhere else in the body, with ∼70% in the abdomen and 30% in the chest.
Background: Intraoperative frozen section analysis is frequently used to obtain a histological diagnosis at the time of resection and to assess resection margins. Although many surgeons perceive a clinical benefit, particularly with respect to the transected resection margins, the limitations and pitfalls of frozen section analysis have not been well documented. Case: Here, we report a case of serous cystadenoma with background pancreatitis masquerading on frozen section as an invasive pancreatic ductal adenocarcinoma. This interpretation was a surprise in light of preoperative imaging that was highly suggestive of a benign cystic tumor, but nevertheless prompted intraoperative consideration of a more radical operation to ensure a complete resection was achieved. Conclusions: Frozen section analysis is an imperfect test, and misdiagnoses can potentially impact patient outcomes adversely. Intraoperative decisions must carefully integrate the preliminary pathological interpretation with the overall clinical context. Further studies are warranted to more fully characterize the accuracy, utility, and cost-effectiveness of intraoperative frozen section analysis for pancreatic surgery.
Background: More than 100 years after its conception, the pancreaticoduodenectomy (PD) remains a challenging procedure with significant morbidity, often due to a postoperative pancreatic fistula (POPF). Factors related to patient physiology, tumor anatomy/pathology, and surgeon/surgical technique have been studied, yielding results at times conflicting and difficult to reproduce. We present a case of a late POPF along with a brief review of the current literature. Case Presentation: The patient is a 55-year-old female with a 20 pack-year smoking history and no history of alcohol abuse, who presented for evaluation of new nausea. Her laboratory tests and computed tomography (CT) imaging were suggestive of biliary obstruction. She was found to have an invasive ampullary adenocarcinoma and subsequently underwent a classic PD. She developed a POPF, managed through a closed suction drain placed intraoperatively. Her course was complicated by the development of an intra-abdominal abscess, managed percutaneously through CT-guided placement of two drains, subsequently removed without issues. She recovered uneventfully until 8 months after the operation, when she presented with abdominal pain and pancreatitis. She was found to have an intra-abdominal collection, again managed percutaneously via CT-guided drainage. This time, the amylase and lipase levels of the drainage fluid were 21,860 and 86,650 U/L, respectively, and cultures were sterile. Upon workup of her pancreatic fistula, a severe stricture at the pancreaticojejunostomy (PJ) was identified. She underwent endoscopic placement of a Hobbs stent by the GI service. Conclusion: Although commonly diagnosed in the days to weeks after a PD, we present a case of a POPF that manifested 8 months after the initial operation in association with a PJ stricture. This case highlights the importance of considering the diagnosis even months after the operation in a patient who presents with symptoms of pancreatitis and/or imaging findings consistent with an intra-abdominal collection.
Background: Vegetative transjugular intrahepatic portosystemic shunt (TIPS) infections are a rare complication of TIPS placement. Cases have been reported in the literature and one study estimated incidence to be 1%.1 The vast majority of cases were reported in the setting of cirrhosis. Here, we report a case of vegetative polymicrobial TIPS infection refractory to broad spectrum antibiotics in a patient with a prior hepaticojejunostomy anastomosis as part of a Whipple procedure for a pancreatic neuroendocrine tumor. Case Presentation: A 40-year-old gentleman with pancreatic neuroendocrine tumor underwent neoadjuvant chemoradiation therapy and became eligible for tumor resection. A pancreaticoduodenectomy (Whipple resection) with en bloc superior mesenteric vein (SMV) and portal vein-splenic vein confluence resection was performed. The patient developed SMV stenosis, and a TIPS was placed to access the SMV for stent placement. The patient eventually developed recurrent fevers because of Escherichia coli and Enterococcal bacteremia that did not resolve with extended courses of various antibiotics, including meropenem, vancomycin, daptomycin, ertapenem, caspofungin, and piperacillin-tazobactam. The TIPS was eventually removed with an interventional radiology procedure; however, the patient ultimately succumbed to sepsis from antibiotic-resistant bacteria. Conclusion: Here we present a case of endotipsitis in a patient with a biliary enteric anastomosis who did not respond to antibiotic therapy. We caution the use of TIPS in patients with this anatomy, as the biliary tree is inevitably colonized with enteric bacteria and in contact with the intraparenchymal hardware of the TIPS.
Background: Pancreatic neuroendocrine tumors (PNETs) are relatively rare, and data guiding management of metastatic lesions are scarce. Hepatic metastases are most common; here we describe a case of metastatic PNET implanted into the posterior gastric cardia. Case Presentation: This case study describes the progression of a 44-year-old man with a history of pancreatic neuroendocrine tumor (PNET) resected through distal pancreatectomy and splenectomy who developed recurrent disease in his stomach with extension into the left adrenal fossa 17 months after initial resection. He subsequently underwent a total gastrectomy and left adrenalectomy with en bloc resection of this recurrence without complication. Final pathology revealed a morphologically similar PNET with positivity for CAM5.2, chromogranin A, and synaptophysin. Conclusion: The unusual location of his recurrence could suggest that his preoperative endoscopic ultrasound and fine-needle aspiration may have had a role in seeding the posterior gastric wall, highlighting the risk of performing this diagnostic procedure in the setting of suspected pancreatic malignancy.
Background: The modified Appleby procedure has been developed for cancer of the pancreatic body or tail with celiac axis invasion, historically classified as unresectable disease. Post-Appleby resection, the source of arterial blood to the liver is the superior mesenteric artery, which supplies the gastroduodenal artery and ultimately feeds the proper hepatic artery. In cases of inadequate collateralization, preoperative coiling of the common hepatic artery (CHA) or intraoperative reconstruction via an aorto-hepatic bypass has been described. Method: We describe a 74-year-old female with a pancreatic mass that was initially determined to be unresectable. She underwent extensive combination neoadjuvant chemotherapy. A favorable response was evidenced by a decrease in serum CA 19-9 levels. After 7 months, she was restaged and offered a distal pancreatectomy (DP) with the possibility of a modified Appleby procedure due to potential tumor involvement of the proximal CHA. Results: Intraoperatively, tumor was identified along the CHA traveling proximally to the celiac axis. Therefore, a modified Appleby procedure with DP and splenectomy was performed without the need for reconstruction of the CHA. Postoperative specimen pathology showed residual pancreatic ductal adenocarcinoma with marked treatment effects. The pathology confirmed an R0 resection. The patient followed our postpancreatic surgery care pathway. She remains well 7 months postoperatively. Conclusion: A pancreatic body or tail mass encasing the celiac vessels should not be an immediate referral for palliative care. Recent evidence shows that successful R0 resection can be achieved following neoadjuvant therapy. In fact, patients who have undergone a successful modified Appleby procedure show survival outcomes similar to patients with less advanced cancer who underwent standard DP. The modified Appleby procedure used in conjunction with neoadjuvant therapy can achieve complete resection in select patients previously thought to be unresectable.
Background: Acinar cell carcinoma is a rare form of pancreatic cancer, accounting for 1-2% of all cases of exocrine pancreatic neoplasms in adults. Due to its rarity, no randomized controlled trials have been performed to determine the optimal treatment options. As such, high-quality case reports and case series are needed to help guide clinicians in the management of this deadly disease. Case Presentation: A 56-year-old Caucasian male presenting with abdominal pain and weight loss was diagnosed with stage III acinar cell carcinoma of the pancreatic body with celiac axis involvement. Although initially deemed unresectable, the patient responded favorably to nine cycles of 5-fluorouracil-based neoadjuvant chemotherapy. The tumor was successfully resected through distal pancreatectomy with en bloc splenectomy and en bloc celiac artery resection (Appleby procedure). Final pathology analysis showed negative resection margins and complete chemotherapeutic response within the pancreas, with residual tumor cells detected in only a single peripancreatic lymph node. Conclusion: 5-fluorouracil-based chemotherapy may be a promising option for the neoadjuvant treatment of locally unresectable acinar cell carcinoma. With sufficient expertise, negative surgical resection margins are possible even with vascular involvement. Due to the generally poor prognosis associated with acinar cell carcinoma, such aggressive treatment measures are warranted.