A Comparison between Open and Minimally Invasive Techniques for the Resection of Colorectal Liver Metastasis.

The liver is the most common site of colorectal cancer metastasis. Liver surgery is a cornerstone in treatment, with progressive expansion of minimally invasive surgery (MIS). This study aims to compare short- and long-term outcomes of open surgery and MIS for the treatment of colorectal adenocarcinoma liver metastasis during the first three years of increasing caseload and implementation of MIS use in liver surgery. All patients treated between November 2018 and August 2021 at Careggi Teaching Hospital in Florence, Italy, were prospectively entered into a database and retrospectively reviewed. Fifty-one patients were resected (41 open, 10 MIS). Considering that patients with a significantly higher number of lesions underwent open surgery and operative results were similar, postoperative morbidity rate and length of hospital stay were significantly higher in the open group. No differences were found in the pathological specimen. The postoperative mortality rate was 2%. Mean overall survival and disease-free survival were 46 months (95% CI 42-50) and 22 months (95% CI 15.6-29), respectively. The use of minimally invasive techniques in liver surgery is safe and feasible if surgeons have adequate expertise. MIS and parenchymal sparing resections should be preferred whenever technically feasible.

Evaluating the best treatment for multifocal hepatocellular carcinoma: A propensity score-matched analysis.

BACKGROUND: Hepatocellular carcinoma (HCC) is a common tumour often diagnosed with a multifocal presentation. Patients with multifocal HCC represent a heterogeneous group. Although Trans-Arterial ChemoEmbolization (TACE) is the most frequently employed treatment for these patients, previous data suggested that liver resection (LR) could be a safe and effective procedure. AIM: To compare LR and TACE in patients with multifocal HCC in terms of procedure-related morbidity and oncologic outcomes. METHODS: All patients with multifocal HCC who underwent LR or TACE as the first procedure between May 2011 and March 2021 were enrolled. The decision to perform surgery or TACE was made after a multidisciplinary team evaluation. Only patients in Child-Pugh class A or B7 and stage B (according to the Barcelona Clinic Liver Cancer staging system, without severe portal hypertension, vascular invasion, or extrahepatic spread) were included in the final analysis. Propensity score matching was used to adjust the baseline differences between patients undergoing LR and the TACE group [number and diameter of lesions, presence of cirrhosis, alpha-fetoprotein (AFP) levels, and Model for End-Stage Liver Disease score]. The Kaplan-Meier method was used to estimate overall survival (OS) and disease-free survival (DFS). The outcomes of LR and TACE were compared using the log-rank test. RESULTS: After matching, 30 patients were eligible for the final analysis, 15 in each group. Morbidity rates were 42.9% and 40% for LR and TACE, respectively (P = 0.876). Median OS was not different in the LR and TACE groups (53 mo vs 18 mo, P = 0.312), while DFS was significantly longer with LR (19 mo vs 0 mo, P = 0.0001). Subgroup analysis showed that patients in the Italian Liver Cancer (ITA.LI.CA) B2 stage, with AFP levels lower than 400 ng/mL, less than 3 lesions, and lesions bigger than 41 mm, benefited more from LR in terms of DFS. Patients classified as ITA.LI.CA B3, with AFP levels higher than 400 ng/mL and with more than 3 lesions, appeared to receive more benefit from TACE in terms of OS. CONCLUSION: In a small cohort of patients with multifocal HCC, LR confers longer DFS compared with TACE, with similar OS and post-procedural morbidity.

Multiple hepatocellular carcinoma: Long-term outcomes following resection beyond actual guidelines. An Italian multicentric retrospective study.

BACKGROUND: Hepatocellular carcinoma (HCC) is frequently diagnosed as multinodular. This study aims to assess prognostic factors for survival and identify patients with multiple HCC who may benefit from surgery beyond the Barcelona Clinic Liver Cancer classification indications. METHODS: This retrospective study included all the consecutive patients from 4 Italian tertiary centers receiving liver resection for naive multiple HCC between 1990 and 2012 to have a potential follow-up of 5 years. RESULTS: Included patients were 144. Ninety-day morbidity and mortality rates were 38.3% and 8.3%, respectively. The 5-year overall and disease-free survival rates were 33.3% and 19.1%, respectively. Tumor size <3 cm, bilirubin, Child-Pugh A, BCLC-A stage, being within "up-to-7" criteria, and minor resections resulted in prognostic factors. The Child-Pugh score resulted in an independent prognostic factor. CONCLUSIONS: Surgery may be related to good outcomes in selected patients with multiple HCC.

Robotic Versus Open Liver Resection in Hepatocarcinoma: Surgical and Oncological Outcomes.

BACKGROUND: Minimally invasive approaches are spreading in every field of surgery, including liver surgery. However, studies comparing robotic hepatectomy with the conventional open approach regarding oncologic outcomes for hepatocellular carcinoma are limited. MATERIALS AND METHODS: We retrospectively reviewed demographics characteristics, pathologic features, surgical, and oncological outcomes of patients who underwent robotic and conventional open liver resection for hepatocellular carcinoma. RESULTS: No significant differences in demographics features, tumor size, tumor location, and type of liver resection were found. The morbidity rate was similar, 23% for the open group versus 17% of the robotic group (P=0.605). Perioperative data analysis showed a greater estimated blood loss in patients who underwent open resection, if compared with robotic group (P=0.003). R0 resection and disease-free resection margins showed no statistically significant differences. The 3-year disease-free survival of the robotic group was comparable with that of the open group (54% vs. 37%; P=0.592), as was the 3-year overall survival (87% vs. 78%; P=0.203). CONCLUSIONS: The surgical and the oncological outcomes seem to be comparable between minimally invasive and open hepatectomy. Robotic liver resections are effective, and do not compromise the oncological outcome, representing a reasonable alternative to the open approach.

The modern vision of the vascular anatomy of the liver by Leonardo da Vinci.

Leonardo da Vinci's analysis and artistic representation of the hepatic vascular anatomy, performed more than 500 years ago, has not yet been fully recognized nor appreciated. Leonardo modified the anatomic concepts of Galen, up until then in vogue, and described for the first time the intrahepatic distribution of the proper hepatic artery, the portal vein, and the hepatic veins. The depiction of these structures is surprising for its clarity and perspective and reproduces the anatomic situation almost exactly. The segmentary division of the liver which several centuries later became the basis of modern resection hepatic surgery was extremely clear in Leonardo's mind.

Analysis of Prognostic Factors for Resected Synchronous and Metachronous Liver Metastases from Colorectal Cancer.

BACKGROUND: Surgical treatment is the cornerstone in the management of colorectal cancer (CRC) liver metastases. The aim of this study is to identify clinicopathological factors affecting disease-free (DFS) and overall survival (OS) in patients undergoing potentially curative liver resection for CRC metastasis. METHODS: All consecutive patients undergoing liver resection for first recurrence of CRC from February 2006 to February 2018 were included. Prognostic impact of factors related to the patient, primary and metastatic tumors, was retrospectively tested through univariate and multivariate analyses. RESULTS: Seventy patients were included in the study. Median postoperative follow-up was 37 months (range 1-119). Median DFS and OS were 15.2 and 62.7 months, and 5-year DFS and OS rates were 16% and 53%. In univariate analysis, timing of metastasis presentation/treatment (combined colorectal and liver resection, "bowel first" approach or metachronous presentation) (p < 0.0001), ASA score (p = 0.003), chemotherapy after liver surgery (p = 0.028), T stage (p = 0.021), number of resected liver lesions (p < 0.0001), and liver margin status (p = 0.032) was significantly associated with DFS while peritoneal resection at colorectal surgery (p = 0.026), ASA score (p = 0.036), extension of liver resection (p = 0.024), chemotherapy after liver surgery (p = 0.047), and positive nodes (p = 0.018) with OS. In multivariate analysis, timing of metastasis presentation/treatment, ASA score, and chemotherapy (before and after liver surgery) resulted significantly associated with DFS and timing of metastasis presentation/treatment, positive nodes, peritoneal resection at colorectal surgery, and surgical approach (open or minimally invasive) of colorectal resection with OS. CONCLUSIONS: Surgery may provide good DFS and OS rates for CRC liver metastasis. Patient selection for surgery and correct timing of intervention within a multidisciplinary approach may be improved by taking into account negative prognostic factors which stress the importance of systemic therapy.

Natural History of MEN1 GEP-NET: Single-Center Experience After a Long Follow-Up.

BACKGROUND: The multiple endocrine neoplasia type 1 syndrome (MEN1) natural history is poorly evaluated, and few single-institution experiences about hereditary gastroenteropancreatic neuroendocrine tumors (GEP-NET) are reported. Our purpose is to analyze the role of GEP-NET in MEN1-related death, as well as the behavior of these lesions during follow-up. METHODS: The study population consists of 77 patients diagnosed with MEN1 GEP-NET, regularly followed up since 1990. Extensive clinical data were prospectively recorded. Statistical analysis was performed both on the whole population of 77 patients and on two subgroups including patients who, during the long lasting study period, underwent GEP-NET surgery (50 pts) and who did not (27 pts), respectively. RESULTS: Twenty-five males (32.5%) and 52 females (67.5%) were enrolled. Sixty-four patients had MEN1 family history (83.1%), and genetic mutation was detected in 67 cases (87%). The mean age at GEP-NET diagnosis was 41.4 years (SD = 13.6); 16 patients (20.8%) had GEP-NET diagnosed before age 30 and 12 cases (15.6%) before 1996. The mean interval time between MEN1 diagnosis and GEP-NET detection was 5.7 years (range -11/37; SD = 8.1 years). Overall, the mean follow-up time from MEN1 diagnosis was 15.8 years (SD = 9.7 years) and from GEP-NET diagnosis was 9.6 years (SD = 6.9 years). Gastrinoma was the most frequent functioning GEP-NET and pancreatoduodenectomy the most adopted surgery. GEP-NET progression affected 12 patients within the non-surgical group, while 18 subjects developed progression after surgery. CONCLUSIONS: Our single-center data provide information on epidemiologic, clinical and pathological features of GEP-NET in MEN1 making possible to clarify their natural history.

Results of Surgical Salvage Treatment for Anal Canal Cancer: A Retrospective Analysis with Overview of the Literature.

BACKGROUND AND AIM: Chemoradiotherapy (CRT) is the gold standard treatment for anal cancer, which permits the maintenance of the anal function. However, about 30-40% of patients develop local disease progression, for which surgery represents a good salvage therapy. The aim of this study is to evaluate survival and morbidity rate in patients who undergo salvage surgery in our single institution, with an overview of the literature. METHODS: A retrospective study was carried out on patients who underwent surgical treatment of anal canal cancer after failure of CRT. We evaluated overall survival at 1, 3, and 5 years and postoperative morbidity rate. RESULTS: Twenty patients who underwent radical surgery with abdominoperineal resection were included in the study. The survival rates at 1, 3, and 5 years were 75, 60, and 37.4%; with a disease-free survival of 67, 53, and 35%, respectively. There was no postoperative mortality. The morbidity rate was 35%. CONCLUSION: Surgery represents the recommended therapy for persistent or recurrent anal canal cancer after CRT, with a good survival rate and an acceptable morbidity.

Operation for insulinomas in multiple endocrine neoplasia type 1: When pancreatoduodenectomy is appropriate.

BACKGROUND: Distal pancreatectomy is the most frequent operation for insulinomas complicating multiple endocrine neoplasia type 1 insulinoma, although there are conditions for which a different operative approach might be preferable. In this article, we report the operative experience of a referral center for multiple endocrine neoplasia type 1 insulinoma. METHODS: Twelve patients underwent operations between 1992 and 2015: 8 underwent a distal pancreatic resection, and 4 underwent a pancreatoduodenectomy. Enucleation of other macroadenomas present in the remnant pancreas was performed in 9 out of these 12 patients. RESULTS: Operative complications (2 pancreatic fistulas and 2 cases of pancreatitis) occurred in 4 of the 8 distal pancreatic resections. In 1 patient, reoperation was required to resolve the complications of the first operation. At pathologic analysis, multiple insulinomas were found in 5 patients, lymph-nodal metastasis positive for insulin in 2 patients, multiple nonfunctioning pancreatic tumors in all patients, glucagonoma in 4 patients, and gastrinoma in the duodenum or lymph nodes in 4 patients. All the patients were treated successfully for the hypoglycemic/hyperinsulinemic syndrome with no clinical recurrence at a mean follow-up of 85 months (range 4-242 months). Recurrent nonfunctioning pancreatic tumor macroadenomas in the remnant pancreas occurred in only 1 of the 12 patients, and no progression of the gastrinomas was observed. None of the patients developed diabetes mellitus. CONCLUSION: Resection of the most severely affected part of the pancreas, whether left or right, associated with enucleation of concomitant macroadenomas in the preserved pancreas is recommended for the treatment of hypoglycemic/hyperinsulinemic syndrome and to prevent malignant progression of nonfunctioning pancreatic tumors in patients with multiple endocrine neoplasia type 1. If the head of the pancreas is the most affected site and the Zollinger-Ellison syndrome is concomitant, then pancreatoduodenectomy should be preferred over distal pancreatectomy.

Safer intestinal invagination for a solid pancreatico-jejunal anastomosis in presence of a soft texture pancreatic remnant and non-dilated duct.

Pancreatico-jejunal anastomosis after pancreatoduodenectomy still represents the Achilles' heel of the procedure: the failure of this anastomosis is relatively common and it is the main cause of post-operative morbidity and mortality. Studies have described different reconstruction strategies for the control of the development of post-operative pancreatic fistula, but the strategy to obtain a safer pancreatico-jejunal anastomosis is still far from satisfaction. We report a novel variation of the invagination technique based on preliminary clinical experience in 8 patients who underwent pancreatico-jejunal anastomosis after pancreatoduodenectomy in our hepatobiliopancreatic center from 2008 to 2014. The variation could obtain a safer intestinal invagination for a solid pancreatico-jejunal anastomosis even in the presence of soft pancreatic remnant.

Albumin Cys34 adducted by acrolein as a marker of oxidative stress in ischemia-reperfusion injury during hepatectomy.

The aim of this study was to measure and identify the reactive carbonyl species (RCSs) released in the blood of humans subjected to hepatic resection. Pre-anesthesia malondialdehyde (MDA) plasma content (0.36 ± 0.11 nmol/mg protein) remained almost unchanged immediately after anaesthesia, before clamping and at the 10th min after ischemia, while markedly increased (to 0.59 ± 0.07 nmol/mg; p < 0.01, Tukey's post test) at the 10th min of reperfusion. A similar trend was observed for the protein carbonyls (PCs), whose pre-anesthesia levels (0.17 ± 0.13 nmol/mg) did not significantly change during ischemia, while increased more than fourfold at the 10th min of reperfusion (0.75 ± 0.17 nmol/mg; p < 0.01, Tukey's post test). RCSs were then identified as covalent adducts to the albumin Cys34, which we previously found as the most reactive protein nucleophilic site in plasma. By using a mass spectrometry (MS) approach based on precursor ion scanning, we found that acrolein (ACR) is the main RCS adducted to albumin Cys34. In basal conditions, the adducted albumin was 0.6 ± 0.4% of the native form but it increased by almost fourfold at the 10th min of reperfusion (2.3 ± 0.7%; p < 0.01, t-test analysis). Since RCSs are damaging molecules, we propose that RCSs, and ACR in particular, are new targets for novel molecular treatments aimed at reducing the ischemia/reperfusion damage by the use of RCS sequestering agents.

Hepatocellular carcinoma on cirrhosis complicated with tumoral thrombi extended to the right atrium: results in three cases treated with major hepatectomy and thrombectomy under hypothermic cardiocirculatory arrest and literature review.

BACKGROUND: Hepatocellular carcinoma (HCC) with the presence of tumor thrombus in hepatic veins and vena cava, until the atrium (RATT), is correlated with poor prognosis and with risk of tricuspid valve occlusion, congestive heart failure, and pulmonary embolism. METHODS: Three patients with HCC on cirrhotic liver with RATT were studied. Operative technique, pre-operative and post-operative liver function tests, blood loss and transfusions, post-operative morbidity and mortality, and the overall survival and the disease free survival were analyzed. RESULTS: Mean operative time was 336 ± 66 min. Intra-operative blood loss was 926.6 ± 325.9 ml. No major complications occurred. The times of hospital stay were 10, 21, and 19 days, respectively. The survival times were 90, 161, and 40 days, and the disease-free survival times were 30, 141, and 30 days, respectively. CONCLUSIONS: The complete removal of HCC with RATT may be achieved with cardiopulmonary by-pass (CPB) and total hepatic vascular exclusion (THVE). Adding the hypothermic cardiocirculatory arrest (HCCA) to the use of CPB allowed us to have minimal blood loss and hemostasis of the resectional plane. So the use of CPB and HCCA should be considered a good therapeutic alternative to the normothermic CPB with THVE.

Liver resection with thrombectomy as a treatment of hepatocellular carcinoma with major vascular invasion: results from a retrospective multicentric study.

BACKGROUND: The role of liver resection (LR) of hepatocellular carcinoma with macroscopic vascular thrombosis (MVT) remains controversial. The aim of this study is to evaluate whether the presence of MVT should still be considered a contraindication for LR. METHODS: Retrospective study was carried out on 62 patients who underwent LR and thrombectomy for hepatocellular carcinoma complicated by MVT. Of the 62 patients, 15 (36.5%) had tumor thrombus (TT) in the peripheral portal vein (Vp1), 5 (12.2%) in second branch (Vp2), and 21 (51.3%) in the first branch/portal vein trunk (Vp3), while on the hepatic/cava vein side, 8 (12.9%) had TT in the main trunk of the hepatic veins (Vv2) and 3 (4.8%) had TT reaching the vena cava/right atrium (Vv3). RESULTS: Perioperative major morbidity was 14.5%, while in-hospital mortality was 4.8%. Overall, 1, 3, and 5-year survival rates were 53.3%, 30.1%, and 20%, and disease-free survival rates were 31.7%, 20.8%, and 15.6%, respectively. There were no differences in survival about the MVT localized in Vp1, Vp2, or Vp3 (P = .77), while we found a statistical trend between patients with Vv2 and Vv3 (P = .06). CONCLUSION: Surgical resection seems to be justified in these patients, and the presence of MVT should no longer be considered an absolute contraindication for LR.

Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome.

AIM: To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic. METHODS: Between January 1992 and June 2012, 28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1 (MEN1) syndrome underwent surgery at our institution. This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome (ZES). Surgical treatment consisted of duodenopancreatectomy (DP) or total pancreatectomy (TP). Regional lymphadenectomy was always performed. Any hepatic tumoral lesions found were removed during surgery. In MEN1 patients, removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia. One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors. This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree. RESULTS: Seventeen MEN1 patients affected with ZES were analyzed. The mean age was 40 years. Fifteen patients underwent DP and two TP. On histopathological examination, duodeno pancreatic endocrine tumors were found in all 17 patients. Eighty-one gastrinomas were detected in the first three portions of the duodenum. Only one gastrinoma was found in the pancreas. The mean number of gastrinomas per patient was 5 (range 1-16). Malignancy was established in 12 patients (70.5%) after lymph node, liver and omental metastases were found. Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s). In two cases, the ectopic gastrinoma was removed at the same time as pancreatic surgery, while in the third case, the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES. CONCLUSION: These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.

Radiofrequency on the liver remnant after liver resection to reach the haemostasis not otherwise achievable with conventional techniques.

INTRODUCTION: During liver resection, in same case of inflamed, steatotic or neo-vascularized liver parenchyma, reaching of haemostasis on the liver resection surface could be very difficult for the surgeon because of the presence of fragile tissue that does not allows the proper placement of stitches, and the conventional method fail. PRESENTATION OF CASE: The authors describe a novel technique in which, after a formal liver resection, liver haemostasis is achieved using radiofrequency energy on the resected surface. A patient affected by a hystiocytic sarcoma localized on the VI-V and IVa segments was scheduled for liver resection. During the resection a diffuse bleeding from the resected surface started with little success obtained with conventional method. So we decided to use the coagulative necrosis generated by the radiofrequency, using a cool type cluster needle, hand-piece with 3 needle, bending 2 needles in a way resembling a "fork", to reach a complete and definitive haemostasis. DISCUSSION: Haemostasis remains a critical issue in liver surgery not only for the catastrophic effect of haemorrhage but also because it is correlated to complications rate and to survival. The coagulative necrosis generated by the radiofrequency could be used to facilitate the creation of a necrotic plane to be transacted. CONCLUSION: The use of the radiofrequency energy, delivered through needles, is suggested when the conventional techniques fail to reach a proper haemostasis after a liver resection or, to consider its use, prior to resect the liver in presence of fragile parenchyma.

Iterative surgical resection of a recurrent gallbladder carcinoma with long-term survival: report of a case.

Gallbladder carcinoma is a rare, but often lethal disease. Unfortunately, at the time of diagnosis, patients usually have advanced disease (T3-T4) and long-term survival is dismal, ranging from 5 to 12% in the literature. However, this cancer can be successfully treated when the tumour is organ-confined (T1-T2 tumours), as happens in the case of incidental diagnosis at the time of cholecystectomy for gallstones. Here we describe a patient with recurrent gallbladder carcinoma who, treated with iterative surgical resection, is alive and disease-free at 5 years after the final surgical procedure.

Cavo-atrial thrombectomy combined with left hemi-hepatectomy for vascular invasion from hepatocellular carcinoma on diseased liver under hypothermic cardio-circulatory arrest.

Vascular invasion of supra-hepatic veins (SHV) is a major complication of primary liver tumours. The tumorous thrombus, when extended to the vena cava and right atrium, may produce occlusion of the tricuspid valve or pulmonary embolism with sudden cardiac death. The presence of macroscopic vascular infiltration represents an advanced stage of the tumour contraindicating liver transplantation, thus liver resection with thrombectomy is the only therapeutic option in this setting despite the concerns of postoperative liver failure and the dismal results at distance. A 45-year-old male with chronic active hepatitis/cirrhosis was referred to our department for a tumour in the left hemi-liver with infiltration of the left-middle hepatic veins and a tumour thrombus extension to the right atrium. We reported a successful cavo-atrial thrombectomy, along with left hemi-hepatectomy, under hypothermic cardio-circulatory arrest (HCA). To our knowledge, this technique has been used only once for primary liver cancer on chronic liver disease, this being the second case reported in literature. We conclude that this technique should be considered for atrial thrombi removal in patients affected by liver tumours in the presence of a healthy liver or of a well compensated liver cirrhosis in order to prolong the patient's life span.

The role of cytoreductive surgery alone for the treatment of peritoneal carcinomatosis of colorectal origin. A retrospective analysis with regard to multimodal treatments.

BACKGROUND/AIMS: Patients with peritoneal carcinomatosis (PC) of colorectal origin have a poor prognosis (median survival of 6 months). Cytoreductive surgery (CS) with intra-peritoneal chemotherapy with or without hyperthermia (HIPEC or EPIC) allows encouraging survivals rates of 22-60 months to be obtained, with an acceptable mortality and morbidity. Nevertheless, the role of cytoreductive surgery alone is little explored in literature. The aim of this study was to better understand the role of CS alone in the treatment of PC of colorectal origin. METHODOLOGY: The outcome of 27 patients with PC of colorectal origin who underwent surgery with curative intent without combined treatments from 1996 to 2006, has been retrospectively analyzed. RESULTS: the median overall survival rate was 15 months; there was a significant statistical difference between patients who had CCR0-1 surgery (N=22) and those who had CCR2 or no resection (N=5) (15.8 vs. 9.6 months respectively, p=0.02). The mortality and the morbidity rates were 7.3% and 29%. CONCLUSIONS: This study suggests that CCR0-1 surgery alone as well as the extension of the disease are important variables influencing survival of patients with PC of colorectal origin. When a very aggressive procedure is needed to achieve a CCR0 resection, surgery should be considered rigorously because of the high risk of severe and potentially lethal complications even without chemohyperthermia. A prospective study should be realized to determine whether or not patients with PC could mostly benefit from combined treatments.

Pancreatectomy in multiple endocrine neoplasia type 1-related gastrinomas and pancreatic endocrine neoplasias.

OBJECTIVE: The aim of this study was to evaluate the results of pancreatic resection in pancreatic endocrine neoplasias (PENs) in patients affected by multiple endocrine neoplasia type 1 (MEN1) syndrome. BACKGROUND: Since these tumors often show an indolent course, the role of diagnostic procedures and type of surgical approach are controversial. Experience with new diagnostic approaches and more aggressive surgery is still limited. METHODS: Sixteen MEN1 patients were referred to our Surgical Unit (1992-2003) and were operated on for the indications of hypergastrinism, hypoglycemia, and/or pancreatic endocrine neoplasias larger than 1 cm. Zollinger-Ellison syndrome (ZES) was present in 13 patients, 2 of whom experienced a recurrence after previous surgery. Preoperative tumor localization was carried out using ultrasonography (US), computed tomography (CT), endoscopic ultrasonography (EUS), somatostatin receptor scintigraphy (SSRS), or selective arterial secretin injection (SASI). Rapid intraoperative gastrin measurement (IGM) was carried out in 8 patients, and 1 patient also underwent an intraoperative secretin provocative test. RESULTS: Either pancreatoduodenectomy (PD) or total pancreatectomy (TP) or distal pancreatectomy was performed. There was no postoperative mortality; 37% complications included pancreatic (27%) and biliary (6%) fistulas, abdominal collection (6%), and acute pancreatitis (6%). EUS and SSRS were the most sensitive preoperative imaging techniques. At follow-up, 10 of 13 hypergastrinemic patients (77%) are currently eugastrinemic with negative secretin provocative test, while 3 are showing a recurrence of the disease. All patients affected by insulinoma were cured. CONCLUSIONS: MEN1 tumors should be considered surgically curable diseases. IGM may be of value in the assessment of surgical cure. Our experience suggests that PD is superior to less radical surgical approaches in providing cure with limited morbidity in MEN1 gastrinomas and pancreatic neoplasias.