How to treat gastrinomas in patients with multiple endocrine neoplasia type1: surgery or long-term proton pump inhibitors?

In patients with multiple endocrine neoplasia type 1 syndrome (MEN 1) and Zollinger-Ellison syndrome (ZES), gastrinomas arise from the duodenum, about 60% are multiple, and about 15% of patients have coexisting pancreatic gastrinomas, which can be localized by the selective arterial secretagogue injection test (SASI test). The guidelines (GLs) by the Japanese Neuroendocrine Tumor Society (JNETS) recommend surgical resection for functioning duodenopancreatic neuroendocrine tumors (NETs), including gastrinomas, in patients with MEN1 (Grade A, 100% agreement among members). Conversely, the GLs of the National Comprehensive Cancer Network (NCCN) in the USA recommend observation and treatment with proton pump inhibitors (PPIs) or exploratory surgery for occult gastrinomas. An international Consensus Statement (ICS) from the European Union (EU) also does not recommend resection of gastrinomas in patients with MEN1, despite some surgeons having reported surgery being curative for gastrinomas in MEN1 patients. In this review, we discuss the serious side effects and tumorigenic effects of the prolonged use of PPIs and the safety and curability of surgery, supported by our results of curative surgery for gastrinomas in 20 patients with MEN1 over 30 years. We conclude that surgery should be the first-line treatment for gastrinomas in MEN1 patients.

Pancreas-Preserving Total Duodenectomy: A Systematic Review.

BACKGROUND: The management of the pancreas in patients with duodenal trauma or duodenal tumors remains a controversial issue. Pancreas-preserving total duodenectomy (PPTD) requires a meticulous surgical technique. The most common indication is familial duodenal adenomatous polyposis (FAP). The aims of this study are to carry out a systematic review of the literature on the indications for PPTD and to highlight the risks and benefits compared with other more aggressive procedures. SUMMARY: A systematic literature review was performed following PRISMA recommendations of studies published in PubMed, Embase, and Cochrane library until May 2019. Thirty articles describing 211 patients were chosen. The mean age was 48 years. The surgical indication in 75% of patients was FAP. The mean operating time was 329 min and mean intraoperative bleeding 412 mL. Postoperative morbidity rate was 49.7% (76% Clavien-Dindo 97.8%. Key Messages: PPTD is indicated for patients with benign and premalignant duodenal lesions without involvement of the pancreatic head. It is a feasible procedure offering an alternative to other more aggressive procedures in selected patients. Mortality is below 1.5%.

Postoperative outcome and quality of life after surgery for FAP-associated duodenal adenomatosis.

INTRODUCTION: Prophylactic colon surgery has increased life expectancy of familial adenomatous polyposis patients. Extracolonic manifestations are life limiting, above all duodenal adenomas. Severe duodenal adenomatosis or cancer may necessitate pancreas-preserving total duodenectomy or partial pancreatico-duodenectomy, mostly after previous proctocolectomy and often after limited local resections of duodenal adenomas. Scarce information on long-term postoperative outcome and quality of life after surgery for duodenal adenomatosis is available. Aim of the present study was to analyze perioperative and long-term outcome after PD and PPTD for FAP-associated duodenal adenomatosis, including QoL and recurrence of adenomas in the neoduodenum after PPTD. MATERIAL, METHODS AND PATIENTS: Thirty-eight patients, 27 after pancreas-preserving duodenectomy and 11 after partial pancreaticoduodenectomy, were included. RESULTS: Pancreas-preserving total duodenectomy was associated with shorter operation time and less blood loss than partial pancreatico-duodenectomy. Clinically relevant pancreatic fistula occurred in 31.5%. In-hospital mortality was 5.3%. Long-term follow-up revealed recurrent pancreatitis after pancreas-preserving total duodenectomy in 22% of patients, two (7.4%) required re-operation. Recurrent adenomatosis was detected in 26% of patients. Quality of life was comparable to the German normal population after both surgical procedures. Patients with postoperative complications showed worse results than those without complications. Disease-specific 10-year survival rate with respect to duodenal adenomatosis was 100%. CONCLUSION: Surgery for FAP-associated duodenal adenomatosis and cancer can be carried out with reasonable morbidity rates despite previous proctocolectomy. Long-term outcome, quality of life, and survival rates are favorable.

Pancreas-preserving duodenectomy after living donor liver transplantation for invasive cytomegalovirus disease.

CMV infection plays an important role in the postoperative course following solid organ transplantation. We present the case of an 11-year-old male patient who underwent LDLT due to severe hepatopulmonary syndrome and biliary cirrhosis. Four weeks after LDLT, he developed persistent GI bleeding and was subjected to repeated endoscopic treatment and radiological arterial embolization to stop the bleeding from duodenal ulcers. Diagnostic workup was negative for CMV disease. Because the bleeding persisted, surgical treatment was indicated, and a pancreas-preserving duodenectomy was performed. Immunohistochemical staining of the surgical specimen demonstrated diffuse endothelial infiltration by CMV. Despite ganciclovir treatment, the patient developed new erosions in the jejunal mucosa and melena; ganciclovir was discontinued, and foscarnet was started, resulting in clinical improvement and the cessation of bleeding. This case highlights the technical aspects of performing a complex upper GI resection in a patient recently subjected to LDLT, taking care to avoid injury to the previous liver graft anastomosis and restore GI continuity. Moreover, CMV tissue-invasive disease compartmentalized in the GI tract may be difficult to diagnose, as indicated by the negative results of antigenemia and PCR assays and endoscopic superficial mucosal biopsies.

Pancreas-preserving management in reinterventions for severe pancreatic fistula after pancreatoduodenectomy: a systematic review.

BACKGROUND: Life-threatening postoperative pancreatic fistula (LTPOPF) is the most feared complication after pancreatoduodenectomy (PD). Although completion pancreatectomy (CP) is usually performed when radiological management fails, the associated morbidity and mortality rates remain high. Here, we reviewed pancreas-preserving alternatives to CP. METHODS: The PubMed database was systematically searched for publications between 1983 and 2014, describing pancreas-preserving surgical treatment of the pancreas remnant (PR) after reintervention in a context of post-PD LTPOPF. RESULTS: A total of 12 articles including 140 patients were reviewed. Six different types of pancreas-preserving treatment were described: external wirsungostomy, simple drainage of the PF, closure of pancreatic stump, internal wirsungostomy, partial CP, and salvage pancreatogastrostomy after major leakage of a pancreatojejunostomy. The overall median survival rate was 75 % but rose to 83 % when patients undergoing only surgical drainage of the fistula were excluded. The median complication rate was 75 %, and the median length of hospital stay was 41.5 days. Further reintervention was required for 25 % of the patients. The median incidence of late diabetes was 22.5 %. The incidence of exocrine insufficiency ranged from 0 to 100 % depending on the intervention. CONCLUSION: Pancreas-preserving surgical management of the PR after LTPOPF can be performed with acceptable mortality and morbidity. These data suggest that CP should have a more precisely specified role in the management algorithm and should not be performed systematically.

Pancreas preserving duodenectomy (PPrD).

BACKGROUND: Pancreaticoduodenectomy has been the standard of care for managing duodenal neoplasms, but recent studies show similar overall and disease-specific survival after pancreas-preserving duodenectomy (PPrD) with potentially less morbidity. METHODS: Retrospective cohort of all adult (age >18) patients who underwent PPrD with curative intent of a neoplasm in or invading into the duodenum at our institution from 2011 to 2022 (n â€‹= â€‹29), excluding tumors involving the Ampulla of Vater or the pancreas. Statistical analyses were performed using STATA. RESULTS: R0 resection was achieved in 93 â€‹% patients. Ten (34.4 â€‹%) experienced postoperative complications (13.7 â€‹% within Clavien-Dindo III-V). PPrD patients had lower rates of pancreatic leak, delayed gastric emptying, and deep surgical site infection. CONCLUSIONS: In this case series, we demonstrate PPrD is safe and effective, with a high rate of complete resection and lower complication rate than that seen in pancreaticoduodenectomy.

Indocyanine green-fluorescent imaging for a detection of accessory pancreatic duct in pancreas-preserving duodenectomy.

Pancreas-preserving duodenectomy is indicated for select patients with a duodenal tumor in the second portion. In this procedure, identification and closure of the accessory pancreatic duct is important to prevent postoperative pancreatic fistula. A 63-y-old man was diagnosed with duodenal mucosal carcinoma in the second portion, with invasion of the major ampullary. We performed pancreas-preserving duodenectomy. Intraoperatively, indocyanine green-fluorescent imaging identified the accessory pancreatic duct clearly and it was successfully closed. Postoperative pancreatic fistula did not occur. Indocyanine green-fluorescent imaging is effective in identifying the accessory pancreatic duct in pancreas-preserving duodenectomy.

Robotic Parenchymal-Sparing Pancreatectomy: A Systematic Review.

BACKGROUND: Parenchymal-sparing approaches to pancreatectomy are technically challenging procedures but allow for preserving a normal pancreas and decreasing the rate of postoperative pancreatic insufficiency. The robotic platform is increasingly being used for these procedures. We sought to evaluate robotic parenchymal-sparing pancreatectomy and assess its complication profile and efficacy. METHODS: This systematic review consisted of all studies on robotic parenchymal-sparing pancreatectomy (central pancreatectomy, duodenum-preserving partial pancreatic head resection, enucleation, and uncinate resection) published between January 2001 and December 2022 in PubMed and Embase. RESULTS: A total of 23 studies were included in this review (n = 788). Robotic parenchymal-sparing pancreatectomy is being performed worldwide for benign or indolent pancreatic lesions. When compared to the open approach, robotic parenchymal-sparing pancreatectomies led to a longer average operative time, shorter length of stay, and higher estimated intraoperative blood loss. Postoperative pancreatic fistula is common, but severe complications requiring intervention are exceedingly rare. Long-term complications such as endocrine and exocrine insufficiency are nearly nonexistent. CONCLUSIONS: Robotic parenchymal-sparing pancreatectomy appears to have a higher risk of postoperative pancreatic fistula but is rarely associated with severe or long-term complications. Careful patient selection is required to maximize benefits and minimize morbidity.

Fully Laparoscopic Pancreas-Preserving Resection of the Third and Fourth Portion of the Duodenum for Adenocarcinomas and Gastrointestinal Stromal Tumors: Technical Report on a Case Series.

Background: Infra-ampullary duodenal lesions are rare and surgical management is controversial. The commonly accepted treatment, which allows radical resection, is pancreaticoduodenectomy, but segmental duodenal resection has been considered as alternative. Aim of the study was to describe the effectiveness of minimally invasive resection of the third/fourth portion of the duodenum for both benign and malignant lesions, with pancreas preservation and reconstruction through end-to-side duodenojejunostomy. Methods: Data from patients undergoing elective laparoscopic curative duodenal resection with pancreas preservation between June 2005 and June 2019 were prospectively collected. Results: A total of 5 patients were identified (3M/2F), median age 73 years (range: 54-83). Lesions were all located in the third or fourth portion of the duodenum and were adenocarcinoma in 2 patients (pT2N0 and pT3N2, both 3 cm in diameter) and gastrointestinal stromal tumor in 3 patients (two pT1N0 and one pT2N0, low-risk according to Miettinen, of 3, 2, and 5 cm in diameter, respectively). The operations lasted a median of 225 minutes (range: 180-300). Digestive continuity was restored with fully laparoscopic side-to-side duodenojejunostomy in all cases. One patient developed pneumonia after surgery (20%) and required also postoperative blood transfusions. Reoperation and mortality rate was nil. Median postoperative stay was 11 days (range: 10-13). The median follow-up was 30 months. Conclusions: Fully laparoscopic pancreas-preserving duodenal resection with duodenojejunal reconstruction can be a safe and feasible option for both benign and malignant lesions of the third and fourth portion of the duodenum. It brings good oncological results, but it needs to be validated with larger number of patients.

Pancreas-preserving partial duodenectomy for non-ampullary duodenal neoplasms: three case reports.

BACKGROUND: There are multiple surgical procedures for resecting non-ampullary duodenal neoplasms (NADNs), and the appropriate method is selected depending on the tumor location and diagnosis. We herein report 3 cases of NADNs that were resected using pancreas-preserving partial duodenectomy (PPD). CASE REPORTS: The first patient, a 73-year-old woman with a circumferential duodenal adenoma in the supra-ampullary duodenum, underwent surgery. After laparotomy, the duodenum proximal to the tumor was confirmed using intraoperative endoscopy and dissected. The duodenum distal to the tumor was dissected under direct visualization, and the specimen was removed. The distal stump of the duodenum was closed, and duodenojejunostomy was performed as described by Billroth II. The tumor was diagnosed as an adenoma 75 mm in size. She was discharged 12 days after surgery without any complications. The second patient, a 48-year-old man, was diagnosed with a neuroendocrine neoplasm (NEN) with a diameter of 14 mm in the supra-ampullary duodenum. Laparoscopic PPD was performed. He was diagnosed with NEN G1 and discharged the 11th day after surgery. The third patient, a 71-year-old man with a 0-Is + IIa lesion in the horizontal duodenum, underwent surgery. After laparotomy, the horizontal duodenum and proximal jejunum were resected, and duodenojejunostomy was performed. The patient was diagnosed with stage I adenocarcinoma and discharged on the 15th day after surgery. CONCLUSION: PPD is useful for avoiding the morbidity of pancreatoduodenectomy in the management of NADNs without invasion to the ampulla of Vater or pancreas.

Mesenteric approach in pancreas-preserving partial duodenectomy for aortic graft-duodenal fistula: a case report.

An aortic graft-duodenal fistula commonly requires graft replacement and duodenectomy. However, the appropriate surgical approach to the duodenum with aortic graft fistula remains unclear. Herein, we describe the case of an 85-year-old male patient who underwent a pancreas-preserving partial duodenectomy using the mesenteric approach for aortic graft-duodenal fistula. The patient presented with hemorrhagic shock and duodenal bleeding 2 years after undergoing open aortic graft replacement. He first underwent emergent endovascular aortic repair with an artificial vascular graft to achieve hemostasis. Although his general condition stabilized following endovascular treatment, duodenal endoscopy revealed an aortic graft-duodenal fistula, exposing the artificial vascular graft via the third portion of the duodenum. As the radical treatment for aortic graft-duodenal fistula, open graft replacement and pancreas-preserving partial duodenectomy were performed using the mesenteric approach which helps to divide the pancreas and duodenum. The patient recovered without any major complications, such as postoperative pancreatic fistula, and was discharged. In conclusion, the mesenteric approach in partial duodenectomy for aortic graft-duodenal fistula could be safely performed. This procedure is useful to approach the duodenum fixed by fistula formation, which may help reduce intraoperative blood loss, operative time, and surgical invasiveness.

Pancreas-preserving duodenal resections vs pancreatoduodenectomy for groove pancreatitis. Should we revisit treatment algorithm for groove pancreatitis?

BACKGROUND: The management of cystic dystrophy of the duodenal wall (CDDW), or groove pancreatitis (GP), remains controversial. Although pancreatoduodenectomy (PD) is considered the most suitable operation for CDDW, pancreas-preserving duodenal resection (PPDR) has also been suggested as an alternative for the pure form of GP (isolated CDDW). There are no studies comparing PD and PPDR for this disease. AIM: To compare the safety, efficacy, and short- and long-term results of PD and PPDR in patients with CDDW. METHODS: A retrospective analysis of the clinical, radiologic, pathologic, and intra- and postoperative data of 84 patients with CDDW (2004-2020) and a comparison of the safety and efficacy of PD and PPDR. RESULTS: Symptoms included abdominal pain (100%), weight loss (76%), vomiting (30%) and jaundice (18%) and data from computed tomography, magnetic resonance imaging, and endoUS led to the correct preoperative diagnosis in 98.8% of cases. Twelve patients were treated conservatively with pancreaticoenterostomy (n = 8), duodenum-preserving pancreatic head resection (n = 6), PD (n = 44) and PPDR (n = 15) without mortality. Weight gain was significantly higher after PD and PPDR and complete pain control was achieved significantly more often after PPDR (93%) and PD (84%) compared to the other treatment modalities (18%). New onset diabetes mellitus and severe exocrine insufficiency occurred after PD (31% and 14%), but not after PPDR. CONCLUSION: PPDR has similar safety and better efficacy than PD in patients with CDDW and may be the optimal procedure for the isolated form of CDDW. The pure form of GP is a duodenal disease and PD may be an overtreatment for this disease. Early detection of CDDW provides an opportunity for pancreas-preserving surgery.

Pancreas-preserving duodenectomy for treatment of a duodenal papillary tumor: A case report.

BACKGROUND: Duodenal papillary tumor is a rare tumor of the digestive tract, accounting for about 0.2% of gastrointestinal tumors and 7% of periampullary tumors. The clinical manifestations of biliary obstruction are most common. Some benign tumors or small malignant tumors are often not easily found because they have no obvious symptoms in the early stage. Surgical resection is the only treatment for duodenal papillary tumors. At present, the methods of operation for duodenal papillary tumors include pancreatoduodenectomy, duodenectomy, ampullectomy, and endoscopic resection. CASE SUMMARY: A 47-year-old man was admitted to because of a duodenal mass that had been discovered 2 mo previously. Electronic gastroscopy at another hospital revealed a duodenal papillary mass that had been considered to be a high-grade intraepithelial neoplasia. Therefore, we conducted a multidisciplinary group discussion and decided to perform a pancreas-preserving duodenectomy and a R0 resection was successfully performed. After surgery, the patient underwent a follow-up period of 5 yr. No recurrence or metastasis occurred. CONCLUSION: According to our experience with a duodenal papillary tumor, compared with pancreaticoduodenectomy, the use of pancreas-preserving duodenectomy can preserve pancreatic function, maintain gastrointestinal structure and function, reduce tissue damage and complications, and render the postoperative recovery faster. Pancreas-preserving duodenectomy for treatment of a duodenal papillary tumor is feasible under strict control of surgical indications.

Central Pancreatectomy for Central Pancreatic Lesions: A Single-Institution Experience.

Background Pancreaticoduodenectomy and distal pancreatectomy are radical procedures for pancreatic lesions with high postoperative morbidity and mortality even in experienced hands. Central pancreatectomy is an alternative less radical procedure for centrally located pancreatic lesions that are benign or have a low malignant potential. It involves removing the central portion of the pancreas and has the advantage of preserving the pancreatic parenchyma, thereby decreasing the postoperative endocrine and exocrine insufficiencies. Methods We conducted a prospective study of six cases of central pancreatectomy in the Department of Surgical Gastroenterology and Liver Transplant, Government Stanley Medical College, India, between the years 2015 and 2019. All patients with lesions in the neck and proximal body of the pancreas were clinically and radiologically evaluated, and those with benign or borderline malignant lesions underwent central pancreatectomy by a standardized technique. Results The mean age of the patients was 27.8 years (range: 14 years - 37 years). Most of the patients were females (66.6%). The most common presenting symptom was abdominal pain, and the most common diagnosis was solid pseudopapillary neoplasm (83.3%). The mean diameter of the lesion was 6.1 cm. All patients underwent pancreaticojejunostomy of the distal stump. The median operative time and the blood loss were 310 minutes and 85 ml, respectively. Two patients developed biochemical postoperative pancreatic fistula, and in the long-term follow-up, none of them developed endocrine or exocrine insufficiency. Conclusion Central pancreatectomy is a safe and effective alternative for benign and low-grade lesions in the neck and body of the pancreas in which the head of the pancreas and a significant portion of the distal body and tail of the pancreas is uninvolved. Standardization of this pancreas-preserving procedure will result in better outcomes.

A Common Concern With a Rare Diagnosis: Pancreatic Neuroendocrine Neoplasms.

Pancreatic neuroendocrine neoplasms (pNENs) are rare, representing only a small percentage of all pancreatic tumors. We report the clinical and radiological features of pNENs. Intraoperative pathology confirmed pNENs with clear margins and the patient did not require adjuvant chemoradiation. The patient is currently doing well and being closely monitored due to the high risk of relapse.

Pancreas-preserving double pancreaticogastrostomy after traumatic injury to the head of the pancreas: a case report.

Traumatic injury to the main pancreatic duct requires surgical treatment, but optimal management strategies have not been established. In patients with isolated pancreatic injury, the pancreatic parenchyma must be preserved to maintain long-term quality of life. We herein report a case of traumatic pancreatic injury with main pancreatic duct injury in the head of the pancreas. Two years later, the patient underwent a side-to-side anastomosis between the distal pancreatic duct and the jejunum. Eleven years later, he presented with abdominal pain and severe gastrointestinal bleeding from the Roux limb. Emergency surgery was performed with resection of the Roux limb along with central pancreatectomy. We attempted to preserve both portions of the remaining pancreas, including the injured pancreas head. We considered the pancreatic fluid outflow tract from the distal pancreatic head and performed primary reconstruction with a double pancreaticogastrostomy to avoid recurrent gastrointestinal bleeding. The double pancreaticogastrostomy allowed preservation of the injured pancreatic head considering the distal pancreatic fluid outflow from the pancreatic head and required no anastomoses to the small intestine.

Hepatocellular carcinoma with duodenal invasion resected subsequent to multimodal therapies: A case report.

INTRODUCTION: Gastrointestinal (GI) involvement in hepatocellular carcinoma (HCC) is uncommon. In particular, HCC with duodenal invasion is known to be a rare condition. In such cases, surgical indication has been generally negative except in few reported cases. To our knowledge, this report describes the first case of HCC with duodenal invasion, resected by hepatectomy accompanied by pancreas-preserving partial duodenectomy (HPPD) following multimodal therapies including systemic sorafenib administration. CASE PRESENTATION: A 65-year-old man had been repeatedly treated for multiple HCCs by transarterial chemoembolization (TACE) and sorafenib. However, the main tumor formerly ruptured began to involve his duodenum, causing GI bleeding. The collateral vessels from the pancreatic and omental branches entered the tumor and nullified the transarterial hemostatic embolization. Hence, HPPD was performed to preserve the major Vater papilla. Histopathological examination revealed poorly-to -moderately differentiated HCC cells invading the duodenum. DISCUSSION AND CONCLUSION: HPPD treatment successfully removed HCC with duodenal invasion achieving viable tumor clearance status (R0). We underline the importance of achieving viable tumor clearance status at any time during the treatment course of patients with advanced HCC as this approach may be the only approach to enable HCC patients with duodenal invasion to resume a healthy life.

Non-Whipple Operations in the Management of Benign, Premalignant and Early Cancerous Duodenal Lesions.

AIM: We reviewed our 20-year experience with non-Whipple operations (pancreas-preserving duodenectomy and transduodenal ampullectomy) for the treatment of benign, premalignant or early-stage malignant duodenal lesions. PATIENTS AND METHODS: Twenty-four patients who underwent non-Whipple operations between January 1996 and December 2015 were identified from an institutional database and retrospectively analyzed. RESULTS: Between 1996 and 2015, 10 patients underwent pancreas-preserving duodenectomy and 14 patients underwent transduodenal ampullectomy. The mean follow-up was 25.8 months (range=6-54 months) and no patient was lost to follow-up. Eighteen patients had preoperative diagnosis of duodenal adenomatosis, three patients had preoperative diagnosis of duodenal adenocarcinoma, one had a bleeding polyp and two had localized inflammation. Average operative time was 145 min (range=127-168 min) for transduodenal ampullectomy and 183 min (range=173-200 min) for pancreas-preserving duodenectomy (p<0.05). The estimated blood loss for transduodenal ampullectomy was 85 vs. 125 ml for pancreas-preserving duodenectomy (p<0.05). Early postoperative complications were noted in 13 cases (54.17%). There were no postoperative (90-day) deaths observed in this series and there were no recurrences during follow-up for the patients operated on with neoplastic lesions. CONCLUSION: For carefully selected patients, transduodenal ampullectomy and pancreas-preserving duodenectomy may be used in place of the Whipple operation for benign and occasionally early-stage malignant (Tis and T1) duodenal and ampullary disease.

Pancreas-preserving resection of lower biliary tract adenocarcinoma: A coring-out technique.

Surgical resection for distal cholangiocarcinoma is usually carried out using pancreaticoduodenectomy (PD). However, because PD is a complex procedure with a high rate of postoperative complications, the surgical indications should be carefully considered, especially for patients with a decreased performance status, significant comorbidities, and/or anatomical anomalies. If curatively carried out, a less invasive, local resection may be an alternative procedure for such patients. In the current study, we present pancreas-preserving resection of the lower biliary tract in a patient with early-stage distal cholangiocarcinoma. This procedure was selected to avoid PD with arterial reconstruction because of arterial anomalies. After an abdominal exploration, a cholecystectomy was carried out and the common hepatic duct was transected. The bile duct was dissected from the pancreatic parenchyma without pancreatic resection, downward to the biliopancreatic ductal confluence. Next, a duodenotomy was done opposite Vater's ampulla. The duodenal mucosa around Vater's ampulla was incised and dissected, and the main pancreatic duct (MPD) was divided. The bile duct was completely separated from the pancreatic parenchyma, and the lower biliary tract was totally "cored-out". After resection, the MPD was re-implanted into the duodenal wall, and the duodenotomy was closed. Finally, a Roux-en-Y hepaticojejunostomy was created. Postoperative course was uneventful. No tumor recurrence has been observed for 21 months after the operation. Thus, pancreas-preserving resection of the lower biliary tract appeared to be appropriate for our patient. This organ-preserving approach can be a useful, alternative procedure in selected patients.

A primary hepatic gastrinoma accompanied by hyperplasia of multi-nodular Brunner's glands.

BACKGROUND: Primary hepatic gastrinoma causing severe ulcerogenic syndrome is extremely rare. Herein, we report a case of primary hepatic gastrinoma accompanied by hyperplasia of multi-nodular Brunner's glands in a patient who instead, preoperatively, was suspected of having multiple duodenal gastrinomas and hepatic metastasis. CASE PRESENTATION: A 57-year-old woman consulted a clinic complaining of melena, intermittent abdominal pain, diarrhea, and vomiting which had persisted for about 3 years. Six months before her presentation, she underwent segmental resection of the jejunum for acute peritonitis due to the spontaneous jejunal perforation. A blood test revealed that her serum immunoreactive gastrin (IRG) level was 12,037 pg/mL. Subsequently, she was transferred to our hospital. On computed tomography (CT), a hypervascular tumor of 23 mm in the segment 5 (S5) region of the liver was visualized. A selective arterial secretagogue injection test (SASI test) was performed twice. The first SASI test revealed that the hepatic tumor was a gastrinoma, and there was no gastrinoma in the duodeno-pancreatic region. Additionally, somatostatin receptor scintigraphy only visualized the tumor in the liver. However, the second SASI test, which was performed during the administration of a proton pump inhibitor and a somatostatin analog (octreotide acetate), revealed that there may have been gastrinomas existing not only in the liver but also in the upper part of the duodenum or the head of the pancreas. Duodenal endoscopy revealed multiple submucosal tumors in the first and the second portion of the duodenum, although a pathological examination of biopsied specimens obtained from the duodenal lesions was negative for malignant cells. Multiple endocrine neoplasia type 1 (MEN1) was excluded from her family history, and serum levels of both intact parathyroid hormone (iPTH) and calcium were within normal ranges. An anterior segmentectomy of the liver and pancreas-preserving total duodenectomy were performed on September 9, 2013. Postoperatively, her serum immunoreactive gastrin level decreased to less than 50 pg/mL. Pathological study of the resected specimens revealed a gastrinoma in the liver, but no gastrinoma in the duodenum. Interestingly, the duodenal submucosal tumor-like lesions were hyperplastic Brunner's glands. Postoperatively, she has been well without recurrence of hypergastrinemia for 4 years. CONCLUSION: We report a case of primary hepatic gastrinoma in a patient who has been cured for 4 years postoperatively. The diagnosis was somewhat difficult due to the coexisting, multiple hyperplastic Brunner's glands of the duodenum mimicking the submucosal neuroendocrine tumors, which might have developed due to long-term hypergastrinemia.