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Multiple Endocrine Neoplasia type 1 (MEN1) Clinical Practice Guidelines (2012) are predominantly based on expert opinion due to limited available evidence at the time, leaving room for interpretation and variation in practices. Evidence on the natural course of MEN1-related neuroendocrine tumours (NET) and the value of screening programs has increased and new imaging techniques have emerged. The aim of this study is to provide insight in the current practices of screening and surveillance for MEN1-related NETs in ENETS Centers of Excellence (CoEs). A clinical practice questionnaire was distributed among all 65 ENETS CoEs. Response rate was 91% (59/65). In 14% of CoEs <10 patients, in 50% 10-49, in 31% 50-100 and in 3 centres (5%) >100 patients with MEN1 are seen. Practices with regard to screening and surveillance of NETs were markedly heterogeneous. Differences between countries were noted in the use of gut hormones for biochemical screening and the choice for imaging modality for screening/surveillance of pancreatic NETs (PanNETs). Magnetic resonance imaging (MRI) is the preferred modality for screening and surveillance of PanNETs, whereas this is computed tomography (CT) for thoracic NETs. Practices regarding screening for thoracic NETs were more homogeneous among larger volume CoEs, with longer screening intervals. The majority of CoEs tailored the surveillance of small pancreatic and lung NETs to observed growth rate. 68% of CoEs advise patients with clinical MEN1 with negative genetic testing to undergo periodic screening like mutation-positive patients. In conclusion, there is still marked heterogeneity in practice, although there are also common trends. Differences were sometimes associated with volume or country, but often no association was found. This underscores the need for clear and evidence-based practice recommendations., (© 2024 The Author(s). Journal of Neuroendocrinology published by John Wiley & Sons Ltd on behalf of British Society for Neuroendocrinology.)

Introduction: The 2015 American Thyroid Association guidelines recommend to de-escalate treatment such as Thyroid lobectomy instead of total thyroidectomy for 1-4 cm papillary thyroid cancer (PTC). Dutch guidelines endorse restricted work-up for thyroid incidentalomas recommending only fine needle aspiration in case of a 'palpable thyroid nodule'. This diagnostic work-up algorithm may result in the identification of less indolent PTCs and may lead to a patient population with relatively more aggressive PTCs. This study aims to retrospectively analyze recurrence rates of low-risk 1-4 cm PTC in the Netherlands., Methods: From the national cancer registry, patients with low-risk 1-4 cm PTC between 2005 and 2015 were included for analysis. Disease free survival (DFS) and overall survival were compared between patients who underwent TT ± RAI and TL without RAI. Post-hoc propensity score analysis was performed correcting for age, sex, T-stage, and N-stage., Results: In total 901 patients were included, of which 711 (78.9%) were females, with a median follow-up of 7.7 years. TT was performed in 893 (94.8%) patients. Recurrence occurred in 23 (2.6%) patients. Multivariable analysis showed no significant correlation between extent of surgery and DFS (p = 0.978), or overall survival (p = 0.590). After propensity score matching, multivariable analysis showed no significant difference on extent of surgery and recurrence., Conclusion: Low-risk PTC patients with 1-4 cm tumor who underwent TL showed similar recurrence rates as those who underwent TT ± adjuvant RAI, which suggests that TL can be sufficient in treating low-risk 1-4 cm PTC, possibly reducing morbidity of these patients in the Netherlands., (© 2022. The Author(s).)

The incidence of differentiated thyroid cancer (DTC) has been increasing worldwide, mostly, as an increase in the incidental detection of micro papillary thyroid carcinomas (microPTCs), many of which are potentially overtreated, as suggested by the unchanged mortality. Several international guidelines have suggested a less aggressive approach. More recently, it has been shown that active surveillance or minimally invasive treatments (MIT) are good alternatives for the management of these patients. In this context, patient participation in the decision-making process is paramount. The Endocrine Task Force of the European Organisation for Research and Treatment of Cancer (EORTC) has undertaken the task to establish consensus and define its position based on the scientific evidence concerning, 1) the current state of diagnostic and management options in microPTCs, including the current opinion of physicians about shared decision making (SDM), 2) the available evidence concerning patients' needs and the available decision instruments, and 3) to provide practical suggestions for implementation of SDM in this context. To improve SDM and patients' participation, knowledge gaps and research directions were highlighted., Competing Interests: Conflict of interest statement The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objective: Recent studies have found aristaless-related homeobox gene (ARX)/pancreatic and duodenal homeobox 1 (PDX1), alpha-thalassemia/mental retardation X-linked (ATRX)/death domain-associated protein (DAXX) and alternative lengthening of telomeres (ALT) to be promising prognostic biomarkers for non-functional pancreatic neuroendocrine tumours (NF-PanNETs). However, they have not been comprehensively evaluated, especially among small NF-PanNETs (≤2.0 cm). Moreover, their status in neuroendocrine tumours (NETs) from other sites remains unknown., Design: An international cohort of 1322 NETs was evaluated by immunolabelling for ARX/PDX1 and ATRX/DAXX, and telomere-specific fluorescence in situ hybridisation for ALT. This cohort included 561 primary NF-PanNETs, 107 NF-PanNET metastases and 654 primary, non-pancreatic non-functional NETs and NET metastases. The results were correlated with numerous clinicopathological features including relapse-free survival (RFS)., Results: ATRX/DAXX loss and ALT were associated with several adverse prognostic findings and distant metastasis/recurrence (p<0.001). The 5-year RFS rates for patients with ATRX/DAXX-negative and ALT-positive NF-PanNETs were 40% and 42% as compared with 85% and 86% for wild-type NF-PanNETs (p<0.001 and p<0.001). Shorter 5-year RFS rates for ≤2.0 cm NF-PanNETs patients were also seen with ATRX/DAXX loss (65% vs 92%, p=0.003) and ALT (60% vs 93%, p<0.001). By multivariate analysis, ATRX/DAXX and ALT status were independent prognostic factors for RFS. Conversely, classifying NF-PanNETs by ARX/PDX1 expression did not independently correlate with RFS. Except for 4% of pulmonary carcinoids, ATRX/DAXX loss and ALT were only identified in primary (25% and 29%) and NF-PanNET metastases (62% and 71%)., Conclusions: ATRX/DAXX and ALT should be considered in the prognostic evaluation of NF-PanNETs including ≤2.0 cm tumours, and are highly specific for pancreatic origin among NET metastases of unknown primary., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

1: ESGE recommends endoscopic ultrasonography (EUS) as the best tool to characterize subepithelial lesion (SEL) features (size, location, originating layer, echogenicity, shape), but EUS alone is not able to distinguish among all types of SEL.Strong recommendation, moderate quality evidence. 2: ESGE suggests providing tissue diagnosis for all SELs with features suggestive of gastrointestinal stromal tumor (GIST) if they are of size > 20 mm, or have high risk stigmata, or require surgical resection or oncological treatment.Weak recommendation, very low quality evidence. 3: ESGE recommends EUS-guided fine-needle biopsy (EUS-FNB) or mucosal incision-assisted biopsy (MIAB) equally for tissue diagnosis of SELs ≥ 20 mm in size.Strong recommendation, moderate quality evidence. 4: ESGE recommends against surveillance of asymptomatic gastrointestinal (GI) tract leiomyomas, lipomas, heterotopic pancreas, granular cell tumors, schwannomas, and glomus tumors, if the diagnosis is clear.Strong recommendation, moderate quality evidence. 5: ESGE suggests surveillance of asymptomatic esophageal and gastric SELs without definite diagnosis, with esophagogastroduodenoscopy (EGD) at 3-6 months, and then at 2-3-year intervals for lesions < 10 mm in size, and at 1-2-year intervals for lesions 10-20 mm in size. For asymptomatic SELs > 20 mm in size that are not resected, ESGE suggests surveillance with EGD plus EUS at 6 months and then at 6-12-month intervals.Weak recommendation, very low quality evidence. 6: ESGE recommends endoscopic resection for type 1 gastric neuroendocrine neoplasms (g-NENs) if they grow larger than 10 mm. The choice of resection technique should depend on size, depth of invasion, and location in the stomach.Strong recommendation, low quality evidence. 7: ESGE suggests considering removal of histologically proven gastric GISTs smaller than 20 mm as an alternative to surveillance. The decision to resect should be discussed in a multidisciplinary meeting. The choice of technique should depend on size, location, and local expertise.Weak recommendation, very low quality evidence. 8: ESGE suggests that, to avoid unnecessary follow-up, endoscopic resection is an option for gastric SELs smaller than 20 mm and of unknown histology after failure of attempts to obtain diagnosis.Weak recommendation, very low quality evidence. 9: ESGE recommends basing the surveillance strategy on the type and completeness of resection. After curative resection of benign SELs no follow-up is advised, except for type 1 gastric NEN for which surveillance at 1-2 years is advised.Strong recommendation, low quality evidence. 10: For lower or upper GI NEN with a positive or indeterminate margin at resection, ESGE recommends repeating endoscopy at 3-6 months and another attempt at endoscopic resection in the case of residual disease.Strong recommendation, low quality evidence., Competing Interests: J.Y. Blay’s department has received research support from Novartis, Bayer, Deciphera, and Roche (all from 2018–2021). P.H. Deprez has received lecture fees from Olympus (2010–2021) and Erbe (2010–2020). R. Gincul has received honoraria for lectures and speaker’s fees from Olympus (2013–2014, 2018–2019) and from Boston Scientific (2016), and for training workshops from IPSEN (2015–2021). J.E. van Hooft’s department has received research grants from Cook Medical (2014–2019) and Abbott (2014–2017); she has received lecture fees from Medtronics (2014–2015, 2019), Cook Medical (2019), and Abbvie (2021), and consultancy fees from Boston Scientific (2014–2017) and Olympus (2021). L.M.G. Moons has provided consultancy on new products to Boston Scientific (1 Jan 2021–31 Dec 2021). M. Polkowski had a speaker and consultancy agreement with Olympus (2018–2021) and a consultancy agreement with Boston Scientific (2018–2019); he is a Co-Editor of Endoscopy. I. Borbath, G. Fernandez Esparrach, T.G. Moreels, E. Nieveen van Dijkum, P.P. Nunes, D. O’Toole, A. Seicean, and M. Vieth have no competing interests., (European Society of Gastrointestinal Endoscopy. All rights reserved.)

Background: Despite the low recurrence rate of resected nonfunctional pancreatic neuroendocrine tumors (NF-pNETs), nearly all patients undergo long-term surveillance. A prediction model for recurrence may help select patients for less intensive surveillance or identify patients for adjuvant therapy. The objective of this study was to assess the external validity of a recently published model predicting recurrence within 5 years after surgery for NF-pNET in an international cohort. This prediction model includes tumor grade, lymph node status and perineural invasion as predictors., Methods: Retrospectively, data were collected from 7 international referral centers on patients who underwent resection for a grade 1-2 NF-pNET between 1992 and 2018. Model performance was evaluated by calibration statistics, Harrel's C-statistic, and area under the curve (AUC) of the receiver operating characteristic curve for 5-year recurrence-free survival (RFS). A sub-analysis was performed in pNETs >2 cm. The model was improved to stratify patients into 3 risk groups (low, medium, high) for recurrence., Results: Overall, 342 patients were included in the validation cohort with a 5-year RFS of 83% (95% confidence interval [CI]: 78-88%). Fifty-eight patients (17%) developed a recurrence. Calibration showed an intercept of 0 and a slope of 0.74. The C-statistic was 0.77 (95% CI: 0.70-0.83), and the AUC for the prediction of 5-year RFS was 0.74. The prediction model had a better performance in tumors >2 cm (C-statistic 0.80)., Conclusions: External validity of this prediction model for recurrence after curative surgery for grade 1-2 NF-pNET showed accurate overall performance using 3 easily accessible parameters. This model is available via www.pancreascalculator.com., (© 2021 The Author(s) Published by S. Karger AG, Basel.)

Background: Surgery for small bowel neuroendocrine neoplasms (SB-NEN) might result in vascular compromise of the remaining bowel due to resection of lymph node metastases in close proximity to main mesenteric vessels. Fluorescence angiography (FA) has been described as a safe technique to assess perfusion during gastro-intestinal surgery. This study aimed to evaluate the potential value of intraoperative FA during surgery for SB-NEN., Methods: This study included patients undergoing surgery for SB-NEN of any stage. The planned level of transection was marked by the surgeon, after which FA using indocyanine green (ICG) was performed. The primary study outcome was change in management due to FA., Results: Ten consecutive patients with SB-NEN were included, all with metastatic lymph nodes close to main mesenteric vessels. FA use led to management changes in eight patients (80%); four patients had less bowel resected with a preserved length of 5-35 cm. The other four patients had more extended bowel resections with an additional length varying from 3 to 25 cm. The median postoperative stay was 4 days (interquartile range 4-6). No anastomotic leakage occurred., Conclusion: This is the first known series describing preliminary results of FA during SB-NEN surgery. FA led to a management change in 80% of patients with better tailoring the extent of resection of small bowel. Structural implementation of FA to assess small bowel perfusion after dissection for small bowel NET results in change of management, either by preserving small bowel or resecting ill-perfused small bowel., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to disclose., (Copyright © 2020 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Background: Surgical safety may be improved using a medical data recorder (MDR) for the purpose of postoperative team debriefing. It provides the team in the operating room (OR) with the opportunity to look back upon their joint performance objectively to discuss and learn from suboptimal situations or possible adverse events. The aim of this study was to investigate the satisfaction of the OR team using an MDR, the OR Black Box®, in the OR as a tool providing output for structured team debriefing., Methods: In this longitudinal survey study, 35 gastro-intestinal laparoscopic operations were recorded using the OR Black Box® and the output was subsequently debriefed with the operating team. Prior to study, a privacy impact assessment was conducted to ensure alignment with applicable legal and regulatory requirements. A structured debrief model and an OR Back Box® performance report was developed. A standardized survey was used to measure participant's satisfaction with the team debriefing, the debrief model used and the performance report. Factor analysis was performed to assess the questionnaire's quality and identified contributing satisfaction factors. Multivariable analysis was performed to identify variables associated with participants' opinions., Results: In total, 81 team members of various disciplines in the OR participated, comprising 35 laparoscopic procedures. Mean satisfaction with the OR Black Box® performance report and team debriefing was high for all 3 identified independent satisfaction factors. Of all participants, 98% recommend using the OR Black Box® and the outcome report in team debriefing., Conclusion: The use of an MDR in the OR for the purpose of team debriefing is considered to be both beneficial and important. Team debriefing using the OR Black Box® outcome report is highly recommended by 98% of team members participating.

Background: Evidence for limiting the extent of surgery in patients with low-risk thyroid cancer is lacking., Methods: A systematic search was performed according to the PRISMA and MOOSE guidelines to assess the effect of total thyroidectomy (TT) with or without radioactive iodine (RAI) treatment versus hemithyroidectomy (HT) on recurrence and overall mortality in patients with differentiated (papillary or follicular) T1-2 N0 thyroid cancer. PubMed, Embase and Cochrane databases were searched, and two authors independently assessed the articles., Results: A total of ten eligible articles were identified. All were observational cohort series, representing a total of 23 134 patients, of which 17 699 were available for meta-analysis. Six studies included patients who had TT followed by RAI treatment. The pooled recurrence rate after TT ± RAI and HT was 2·3 and 2·8 per cent respectively (odds ratio (OR) 1·12, 95 per cent c.i. 0·82 to 1·53; P = 0·48). The pooled 20-year overall survival rate after TT ± RAI was 96·8 per cent, compared with 97·4 per cent for HT (OR 1·30, 0·71 to 2·37; P = 0·40). Overall, higher complication rates were found in the TT ± RAI group., Conclusion: Recurrence rates after HT for treatment of well differentiated T1-2 N0 thyroid cancer were similar to those after TT ± RAI, with a lower incidence of treatment-related complications., (© 2020 The Authors. BJS Open published by John Wiley & Sons Ltd on behalf of British Journal of Surgery Society.)

Background: Currently, there are no European recommendations for the management of pediatric thyroid cancer. Other current international guidelines are not completely concordant. In addition, medical regulations differ between, for instance, the US and Europe. We aimed to develop new, easily accessible national recommendations for differentiated thyroid carcinoma (DTC) patients <18 years of age in the Netherlands as a first step toward a harmonized European Recommendation., Methods: A multidisciplinary working group was formed including pediatric and adult endocrinologists, a pediatric radiologist, a pathologist, endocrine surgeons, pediatric surgeons, pediatric oncologists, nuclear medicine physicians, a clinical geneticist and a patient representative. A systematic literature search was conducted for all existing guidelines and review articles for pediatric DTC from 2000 until February 2019. The Appraisal of Guidelines, Research and Evaluation (AGREE) instrument was used for assessing quality of the articles. All were compared to determine dis- and concordances. The American Thyroid Association (ATA) pediatric guideline 2015 was used as framework to develop specific Dutch recommendations. Discussion points based upon expert opinion and current treatment management of DTC in children in the Netherlands were identified and elaborated., Results: Based on the most recent evidence combined with expert opinion, a 2020 Dutch recommendation for pediatric DTC was written and published as an online interactive decision tree (www.oncoguide.nl)., Conclusion: Pediatric DTC requires a multidisciplinary approach. The 2020 Dutch Pediatric DTC Recommendation can be used as a starting point for the development of a collaborative European recommendation for treatment of pediatric DTC.

Background: PTEN hamartoma tumor syndrome (PHTS) represents a group of syndromes caused by a mutation in the PTEN gene. Children with a germline PTEN mutation have an increased risk of developing differentiated thyroid carcinoma (DTC). Several guidelines have focused on thyroid surveillance in these children, but studies substantiating these recommendations are lacking., Objective: The present study intends to provide the available evidence for a thyroid carcinoma surveillance program in children with PHTS., Methods: An extensive literature search was performed to identify all studies on DTC in pediatric PHTS patients. Two pediatric cases are presented to illustrate the pros and cons of thyroid carcinoma surveillance. Recommendations for other patient groups at risk for DTC were evaluated. Consensus within the study team on recommendations for children with PHTS was reached by balancing the incidence and behavior of DTC with the pros and cons of thyroid surveillance, and the different surveillance methods., Results: In 5 cohort studies the incidence of DTC in childhood ranged from 4 to 12%. In total 57 cases of DTC and/or benign nodular disease in pediatric PHTS patients were identified, of which 27 had proven DTC, with a median age of 12 years (range 4-17). Follicular thyroid carcinoma (FTC) was diagnosed in 52% of the pediatric DTC patients. No evidence was found for a different clinical behavior of DTC in PHTS patients compared to sporadic DTC., Conclusions: Children with PHTS are at increased risk for developing DTC, with 4 years being the youngest age reported at presentation and FTC being overrepresented. DTC in pediatric PHTS patients does not seem to be more aggressive than sporadic DTC., Recommendations: Surveillance for DTC in pediatric PHTS patients seems justified, as early diagnosis may decrease morbidity. Consensus within the study team was reached to recommend surveillance from the age of 10 years onwards, since at that age the incidence of DTC seems to reach 5%. Surveillance for DTC should consist of yearly neck palpation and triennial thyroid ultrasound. Surveillance in children with PHTS should be performed in a center of excellence for pediatric thyroid disease or PHTS., Competing Interests: The authors have nothing to disclose., (Copyright © 2020 by S. Karger AG, Basel.)

Objective: To perform a nationwide population based study in ATC on incidence, treatment and survival., Design: Retrospective cohort study., Methods: All patients with primary ATC between 1989 and 2016 were identified in the Netherlands Cancer Registry (NCR). Of all these patients excerpts from the pathology reports from PALGA: Dutch Pathology registry were linked to the data of the NCR. Standardized incidences were calculated, survival was estimated using Kaplan-Meier method and univariable statistically significant factors were included in a multivariable regression model., Results: In total, 812 patients were included. Mean standardized incidence rates were 0.18/100 000 (range 0.11-0.27/100 000) with a significant trend over the years with an estimated annual percentage change of 1.3% per year (95% CI 0.4-2.1%). Median overall survival was 2.2 months, and estimated 1-year survival was 12%. Patients without distant metastases at diagnosis had an estimated 1-year survival of 21.6%. Prognostic factors for prolonged survival were double or triple therapy, age below 65 years, M0-status and absence of bilateral lymph node metastases., Conclusions: ATC is rare, but often lethal, form of thyroid cancer, with a median survival of 2 months and 1-year survival of approximately 10%. The incidence is slightly rising in the Netherlands over the past 3 decades. There appears to be a subgroup of patients that survive longer, mainly those with relatively limited disease who underwent double or triple therapy. Further research is needed to define these patients more distinctively.

Pancreatic parenchyma sparing surgery for insulinomas avoids the risk of endocrine and exocrine insufficiency, and potential high-risk anastomoses associated with pancreatic resection. Robotic surgery may be used as an alternative for open pancreatic enucleation without compromising dexterity and 3D-vision. We present the case of a 42-year old woman who presented with sweating, tremor and episodes of hypoglycemia. A fasting test confirmed endogenic insulin overproduction. After inconclusive CT- and MRI imaging, endoscopic ultrasonography showed a hypoechoic lesion, which was fully within the pancreatic head. Although consent was obtained for pancreatoduodenectomy, robotic enucleation seemed feasible. After mobilization, intraoperative ultrasonography was used to identify the lesion and its relation with the pancreatic duct. Dissection was performed using a traction suture, hot shears and bipolar diathermia. A sealant patch was applied for hemostasis and a drain placed. The patient developed a grade B pancreatic fistula for which endoscopic sphincterotomy was performed; the surgical drain could be removed in the outpatient clinic after 20 days. Prospective studies should confirm the short- and long-term benefits of robotic enucleation of insulinomas.

Appendiceal neuroendocrine neoplasms (ANEN) are mostly discovered coincidentally during appendicectomy and usually have a benign clinical course; thus, appendicectomy alone is considered curative. However, in some cases, a malignant potential is suspected, and therefore additional operations such as completion right hemicolectomy are considered. The existing European Neuroendocrine Tumour Society (ENETS) guidelines provide useful data about epidemiology and prognosis, as well as practical recommendations with regards to the risk factors for a more aggressive disease course and the indications for a secondary operation. However, these guidelines are based on heterogeneous and retrospective studies. Therefore, the evidence does not seem to be robust, and there are still unmet needs in terms of accurate epidemiology and overall prognosis, optimal diagnostic and follow-up strategy, as well as identified risk factors that would indicate a more aggressive surgical approach at the beginning and a more intense follow-up. In this review, we are adopting a critical approach of the ENETS guidelines and published series for ANEN, focusing on the above-noted "grey areas"., (© 2018 S. Karger AG, Basel.)

Pulmonary carcinoids (PCs) display the common features of all well-differentiated neuroendocrine neoplasms (NEN) and are classified as low- and intermediate-grade malignant tumours (i.e., typical and atypical carcinoid, respectively). There is a paucity of randomised studies dedicated to advanced PCs and management principles are drawn from the larger gastroenteropancreatic NEN experience. There is growing evidence that NEN anatomic subgroups have different biology and different responses to treatment and, therefore, should be investigated as separate entities in clinical trials. In this review, we discuss the existing evidence and limitations of tumour classification, diagnostics and staging, prognostication, and treatment in the setting of PC, with focus on unmet medical needs and directions for the future., (© 2018 S. Karger AG, Basel.)

Background: Despite evidence of different malignant potentials, postoperative follow-up assessment is similar for G1 and G2 pancreatic neuroendocrine tumors (panNETs) and adjuvant treatment currently is not indicated. This study investigated the role of Ki67 with regard to recurrence and survival after curative resection of panNET., Methods: Patients with resected non-functioning panNET diagnosed between 1992 and 2016 from three institutions were retrospectively analyzed. Patients who had G1 or G2 tumor without distant metastases or hereditary syndromes were included in the study. The patients were re-categorized into Ki67 0-5 and Ki67 6-20%. Cox regression analysis with log-rank testing for recurrence and survival was performed., Results: The study enrolled 241 patients (86%) with Ki67 0-5% and 39 patients (14%) with Ki67 6-20%. Recurrence was seen in 34 patients (14%) with Ki67 0-5% after a median period of 34 months and in 16 patients (41%) with Ki67 6-20% after a median period of 16 months (p < 0.001). The 5-year recurrence-free and 10-year disease-specific survival periods were respectively 90 and 91% for Ki67 0-5% and respectively 55 and 26% for Ki67 6-20% (p < 0.001). The overall survival period after recurrence was 44.9 months, which was comparable between the two groups (p = 0.283). In addition to a Ki67 rate higher than 5%, tumor larger than 4 cm and lymph node metastases were independently associated with recurrence., Conclusions: Patients at high risk for recurrence after curative resection of G1 or G2 panNET can be identified by a Ki67 rate higher than 5%. These patients should be more closely monitored postoperatively to detect recurrence early and might benefit from adjuvant treatment. A clear postoperative follow-up regimen is proposed.

Background: Large population-based studies give insight into the prognosis and treatment outcomes of patients with pancreatic neuroendocrine tumors (pNETs). Therefore, we provide an overview of the treatment and related survival of pNET in the Netherlands., Methods: Patients diagnosed with pNET between 2008 and 2013 from the Netherlands Cancer Registry were included. Patient, tumors and treatment characteristics were reported. Survival analyses with log-rank testing were performed to compare survival., Results: In total, 611 patients were included. Median follow-up was 25.7 months, and all-cause mortality was 42%. Higher tumor grade and TNM stage were significantly associated with worse survival in both the overall and metastasized population. The effect of distant metastases on survival was more significant in lower tumor stages (T1-3 p < 0.05, T4 p = 0.074). Resection of the primary tumor was performed in 255 (42%) patients. Patients who underwent surgery had the highest 5-year survival (86%) compared to PRRT (33%), chemotherapy (21%), targeted therapy and somatostatin analogs (24%) (all p < 0.001). Patients with T1M0 tumors (n = 115) showed favorable survival after surgical resection (N = 95) compared to no therapy (N = 20, p = 0.008). Resection also improved survival significantly in patients with metastases compared to other treatments (all p > 0.05). Without surgery, PRRT showed the best survival curves in patients with distant metastases. Grade 3 tumors and surgical resection were independently associated with survival (HR 7.23 and 0.12, respectively)., Conclusion: Surgical resection shows favorable outcome for all pNET tumors, including indolent tumors and tumors with distant metastases. Prospective trials should be initiated to confirm these results.

Radiation exposure to the thyroid gland during treatment of childhood, adolescent and young adult cancer (CAYAC) may cause differentiated thyroid cancer (DTC). Surveillance recommendations for DTC vary considerably, causing uncertainty about optimum screening practices. The International Late Effects of Childhood Cancer Guideline Harmonization Group, in collaboration with the PanCareSurFup Consortium, developed consensus recommendations for thyroid cancer surveillance in CAYAC survivors. These recommendations were developed by an international multidisciplinary panel that included 33 experts in relevant medical specialties who used a consistent and transparent process. Recommendations were graded according to the strength of underlying evidence and potential benefit gained by early detection and appropriate management. Of the two available surveillance strategies, thyroid ultrasound and neck palpation, neither was shown to be superior. Consequently, a decision aid was formulated to guide the health care provider in counseling the survivor. The recommendations highlight the need for shared decision making regarding whether to undergo surveillance for DTC and in the choice of surveillance modality., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

The use of intraoperative neuromonitoring (IONM) in thyroid surgery has steadily increased in recent years. This technique helps to identify different nerves in and around the operation site, such as the recurrent laryngeal nerve (RLN) and the external branch of the superior laryngeal nerve. Particularly in patients who undergo a second operation or a complex procedure for malignancy, the technique's value has been demonstrated. However, in non-complex thyroid operations the risk of vocal cord paresis due to iatrogenic injury of the RLN is 0.1%. Therefore, high quality research with sufficient power to assess the effect of IONM on prevention of vocal cord paresis is scarce, and the routine use of IONM in thyroid surgery is currently not indicated.

Background: Medullary thyroid cancer (MTC) comprises only 4% of all thyroid cancers and originates from the parafollicular C-cells. HIF-1α expression has been implied as an indicator of worse prognosis in various solid tumors. However, whether expression of HIF-1α is a prognosticator in MTC remained unclear. Our aim was to evaluate the prognostic value of HIF-1α in patients with MTC., Methods: All patients with MTC who were operated on between 1988 and 2014 in five tertiary referral centers in The Netherlands were included. A tissue microarray was constructed in which 111 primary tumors could be analyzed for expression of HIF-1α, CAIX, Glut-1, VEGF and CD31 and correlated with clinicopathologic variables and survival., Results: The mean age of patients was 46.3 years (SD 15.6), 59 (53.2%) were male. Of the 111 primary tumors, 49 (44.1%) were HIF-1α negative and 62 (55.9%) were HIF-1α positive. Positive HIF-1α expression was an independent negative indicator for progression free survival (PFS) in multivariate cox regression analysis (HR 3.1; 95% CI 1.3 - 7.3). Five-years survival decreased from 94.0% to 65.9% for the HIF-1α positive group (p=0.007). Even within the group of patients with TNM-stage IV disease, HIF-1α positivity was associated with a worse prognosis, shown by a decrease in 5-years survival of 88.0% to 49.3% (p=0.020)., Conclusion: Expression of HIF-1α is strongly correlated with adverse prognosis of MTC. This could open up new ways for targeted systemic therapy of MTC.

Surgery and anesthesia pose a threat to patients with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), because prolonged fasting, stress, and pain are known risk factors for the induction of metabolic derangement. The optimal perioperative management in these patients is unknown and the use of volatile agents and agents dissolved in fatty acids has been related to postoperative metabolic complications. However, the occurrence of metabolic derangement is multifactorial and depends, amongst others, on the severity of the mutation and residual enzyme activity. Current guidelines suggest avoiding both volatile anesthetics as well as propofol, which seriously limits the options for providing safe anesthesia. Therefore, we reviewed the available literature on the perioperative management of patients with VLCADD. We concluded that the use of some medications, such as volatile anesthetics, in patients with VLCADD might be wrongfully avoided and could in fact prevent metabolic derangement by the adequate suppression of pain and stress during surgery. We will illustrate this with a case report of an adult VLCADD patient undergoing minor surgery. Besides the use of remifentanil, anesthesia was uneventfully maintained with the use of sevoflurane, a volatile agent, and continuous glucose infusion. The patient was monitored with a continuous glucose meter and creatinine kinase measurements.

Aim: To determine whether somatostatin receptor scintigraphy (SRS) influences the preoperative staging and clinical management of non-functioning pancreatic neuroendocrine tumours (NF-pNETs)., Materials and Methods: All SRS examinations performed between 2002-2013 were selected. Patients with NF-pNET were included if both computed tomography (CT) and SRS was performed during preoperative staging. The diagnostic accuracy of CT and SRS for detecting NF-pNET metastases was analysed. Altered TNM classification and changed clinical management were calculated. Changed management was defined as a change from surgical resection into systemic treatment or vice versa. NF-pNETs were defined as tumours without clinical symptoms of hormonal hypersecretion., Results: Overall, 62 patients with NF-pNET were included with a mean age of 57 years (SD: 12.4)(2). In 28 patients (45%), CT and SRS were correct and in agreement in the detection of primary tumour/metastases. In 34 patients (55%), one of the techniques was incorrect and therefore, there was no agreement. SRS altered the TNM classification in 14 patients (23%) and clinical management in nine patients (15%). In patients without metastases on CT, SRS detected lymph node metastases in one patient. The sensitivity to detect the primary tumour with CT was 95% and with SRS was 73%. In detecting metastases, the sensitivity and specificity were both 85% for CT versus 80% and 90% for SRS., Conclusion: Overall, CT and SRS were in agreement in the detection of NF-pNET. In NF-pNET without suspicious metastatic lesions on CT, SRS has limited value. SRS may be indicated to confirm lesions suspicious for neuroendocrine tumours metastases., (Copyright © 2016 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Background: Treatment of differentiated thyroid carcinoma (DTC) often involves administration of radioactive iodine (I-131) for remnant ablation or adjuvant therapy. As DTC has favorable outcome and the incidence is increasing, concerns have been raised about the possible adverse effects of I-131 therapy. We systematically reviewed the literature to examine the risk of intermediate and long-term adverse effects of I-131 therapy in DTC patients., Methods: Multiple electronic databases were searched up to November 2014 for English-language, controlled studies that reported on the risk of salivary gland dysfunction, lacrimal gland dysfunction, gonadal dysfunction, female reproductive outcomes or second primary malignancies (SPM) after I-131 exposure. The certainty of the evidence found was assessed using GRADE., Results: In total, 37 articles met all inclusion criteria, no studies reporting on adverse effects after I-131 treatment focused solely on children. After exposure to I-131 for DTC, patients experienced significantly more frequently salivary gland dysfunction (prevalence range: 16-54%, moderate-level evidence), lacrimal gland dysfunction (prevalence: 11%, low-level evidence), transient male gonadal dysfunction (prevalence: 35-100%, high-level evidence), transient female gonadal dysfunction (prevalence: 28%, low-level evidence) and SPM (prevalence: 2.7-8.7%, moderate-level evidence) compared to unexposed patients. I-131 therapy seems to have no deleterious effects on female reproductive outcomes (very-low level evidence). The prevalence and severity of adverse effects were correlated to increasing cumulative I-131 activity., Conclusion: Treatment with I-131 for DTC may have significant adverse effects, which seem to be dose dependent. These adverse effects of treatment must be balanced when choosing for I-131 therapy in patients with DTC., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Objectives. The aim of this study was to investigate the detection rate of adrenal incidentalomas and subsequent workup. Design. Retrospective cohort study. Methods. Two investigators evaluated the adrenals on abdominal CT scans. Abnormalities were compared to the original radiology reports and an experienced abdominal radiologist reviewed the CT scans. All additional imaging and laboratory tests were assessed. Results. The investigators detected 44/356 adrenal incidentalomas (12%). In 25 patients an adrenal incidentaloma had been noted in the radiology report. The expert radiologist agreed on 19 incidentalomas in 17 patients, two with bilateral incidentalomas. Of the 25 incidentaloma patients, 4 (16%) patients were screened for hormonal overproduction and 2 (8%) patients had follow-up imaging studies. Conclusions. 12% of the patients had an adrenal incidentaloma (42 of 356). 17 (40%) had initially not been reported by the radiologist. When diagnosed with an adrenal incidentaloma, only a small percentage of patients (16%) is screened or undergoes repeated imaging (8%) as proposed in the National Institutes of Health (NIH) guidelines on adrenal incidentalomas.

Background: Subtotal thyroidectomy is a surgical procedure, in which the surgeon leaves a small thyroid remnant in situ to preserve thyroid function, thereby preventing lifelong thyroid hormone supplementation therapy., Aim: To evaluate thyroid function after subtotal thyroidectomy for Graves' hyperthyroidism., Subjects and Methods: We retrospectively reviewed the medical records of all patients (n = 62) who underwent subtotal thyroidectomy for recurrent Graves' hyperthyroidism between 1992 and 2008 in our hospital. Thyroid function was defined according to plasma TSH and free T4 values., Results: Median followup after operation was 54.6 months (range 2.1-204.2 months). Only 6% of patients were euthyroid after surgery. The majority of patients (84%) became hypothyroid, whereas 10% of patients had persistent or recurrent hyperthyroidism. Permanent recurrent laryngeal nerve palsy and permanent hypocalcaemia were noted in 1.6% and 3.2% of patients, respectively., Conclusion: In our series, subtotal thyroidectomy for Graves' hyperthyroidism was associated with a high risk of postoperative hypothyroidism and a smaller, but significant, risk of persistent hyperthyroidism. Our data suggest that subtotal thyroidectomy seems to provide very little advantage over total thyroidectomy in terms of postoperative thyroid function.

Introduction: Most leg ulcers are vascular based. Only if vascular therapy fails other causes are considered. We report the case of a female with incapacitating leg ulcers caused by a rare condition which was only diagnosed after failing treatment., Presentation of Case: The female had an extensive previous history including diabetes, renal insufficiency and cardiovascular disease and presented with three large and painful ulcers on her left lower leg. Standard treatment with antibiotics, wound excision and additional treatment with hyperbaric oxygen were ineffective. One month post hospital-admission calciphylaxis cutis caused by renal failure induced secondary hyperparathyroidism was diagnosed. Surgical treatment by a parathyroidectomy induced rapid regeneration of the ulcers., Discussion: Our patient's vast comorbidity and previous history had expanded differential considerations causing a delay in diagnosis. Our patient's previous history led us to believe her ulcers were vascular based, however her chronic renal failure appeared responsible for her condition., Conclusion: Although less probable than venous insufficiency and concomittant leg ulcers or other differential considerations, calciphylaxis cutis should be part of the differential diagnosis in any end stage renal disease-patient with unexplained ulcers as an effective therapy is readily available.

Background: Hepatic artery embolization (HAE) is a palliative treatment for patients with liver metastases from neuroendocrine tumours. HAE reduces hormonal symptoms, but its impact on survival has been questioned., Methods: Biochemical responses and survival in consecutive patients with disseminated liver metastases from midgut carcinoid tumours were studied after HAE. Repeat HAE was performed in selected patients with radiological and biochemical signs of progression., Results: Of 107 patients who had HAE, the median survival from the first procedure was 56 (range 1-204) months. Prolonged survival showed a strong correlation with reduction of urinary 5-hydroxyindoleacetic acid (P = 0.003) and plasma chromogranin A (P = 0.001) levels. The biochemical response to repeat HAE was similar to that for the first procedure (P = 0.002). The complication rate was low (7.5 per cent), as was the mortality rate (1.9 per cent) within 1 month of HAE., Conclusion: HAE is safe, provides good control of hormonal symptoms, and prolongs survival in biochemically responsive patients. It is a valuable palliative option for patients with midgut carcinoid syndrome due to liver metastases and can be repeated in patients with a favourable response to the first procedure., (2009 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.)

A 53-year-old woman presented with fever accompanied by chills and an extremely painful swelling of her right thyroid lobe. She was initially diagnosed as having subacute thyroiditis, but after 14 days her disease appeared to be caused by a destructive suppurative thyroiditis due to Salmonella group C. A pre-existing hyperplastic nodule in the right thyroid lobe was the predisposing factor. Antibiotics were given for several weeks and surgical drainage was performed. Finally a hemithyroidectomy was done to eliminate the predisposing factor.

Background: Quality of life (QOL) is an important outcome measure after treatment of pancreatic and periampullary carcinoma. The aim of this prospective longitudinal study was to analyse QOL after surgery for resectable pancreatic or periampullary carcinoma., Methods: Patients with potentially resectable tumours underwent pancreaticoduodenectomy (n = 72) or a double-bypass procedure (n = 42). They were asked to complete a questionnaire before laparotomy and at 2 weeks, 6 weeks, and 3, 6, 9 and 12 months after surgery. Fifty-nine patients completed a shortened questionnaire on a weekly basis., Results: There was a temporary decrease in physical and gastrointestinal functioning after pancreaticoduodenectomy. A similar decrease in QOL was observed after double bypass, as well as decreases in mental functioning and overall QOL. Almost all QOL scores returned to preoperative values by about 3 months after surgery, although only briefly so in patients who had a double-bypass procedure. There were no differences between groups in the terminal stages of disease. A rapid decrease on all QOL scales was observed in the last 8 weeks before death., Conclusion: Surgery for pancreatic and periampullary carcinoma was not associated with irreversible impairment or protracted recovery of QOL. The relatively long plateau phase after recovery supports the argument for surgical treatment, including surgical palliation in selected patients.

Objective: To investigate the role of diagnostic laparoscopy and laparoscopic ultrasonography in the staging of carcinoma of the gastric cardia that is involving the distal oesophagus., Design: Retrospective consecutive case series., Setting: Tertiary care centre, The Netherlands., Subjects: 48 patients (34 men and 14 women, median age 63 years, range 39-84) who presented with tumours of the gastric cardia that involved the distal oesophagus and in whom non-invasive staging had not shown unresectable locoregional disease or distant metastases., Interventions: In addition to laparoscopy and laparoscopic ultrasonography, biopsy of all suspected lesions outside the area of potential resection., Main Outcome Measures: Number of patients in whom the findings obviated the need for exploratory laparotomy., Results: There were no complications related to the laparoscopy. The investigation showed distant metastases (which were histologically verified) in 11 patients (23%, 95% confidence interval (CI) 16 to 30). These patients had non-operative palliation. Seven were identified by laparoscopy, and laparoscopic ultrasonography showed the other four. In three patients whose distant metastases had already been identified by laparoscopy, ultrasonography was omitted. Three additional patients had suspect lesions, but these were not confirmed histologically. However, these lesions were shown to be cancerous at laparotomy. One additional patient had an intra-abdominal metastasis which was missed by laparoscopy with ultrasonography., Conclusions: Laparoscopy with ultrasonography safely detected metastases that had not been shown by conventional staging investigations in 23% of 48 patients with carcinoma of the gastric cardia. The investigation should therefore be added to the standard staging procedures in patients with carcinoma of the gastric cardia that is involving the distal oesophagus.

Objective: Development of a prognostic tool for patients with unresectable pancreatic cancer to distinguish between with low or high probabilities of survival 3 to 9 months after diagnosis., Design: Data about individual patients from five studies were pooled. A multivariate proportional hazards model with time-dependent covariates was developed, including age, sex, and metastases. An extended model was developed on a subset of patients, including weight loss, pain, and jaundice at diagnosis., Setting: Multicentre study, The Netherlands, Norway, USA, UK, and Canada., Subjects: 1020 patients with unresectable pancreatic cancer., Main Outcome Measures: Prediction of prognosis., Results: Patients with metastases, pain, or weight loss at diagnosis had a significantly poorer prognosis than the others. Older men had a worse prognosis than younger men, while older women had a better prognosis than younger ones. Patients with jaundice had a relatively good prognosis. Differences in survival among the studies were incorporated in a prognostic score chart., Conclusion: The prognostic score chart can be used to select patients with relatively low expectation of survival for endoscopic palliation, and patients with relatively high expectation for surgical palliation.

Background: A disease-specific quality of life questionnaire is not available for patients with periampullary carcinoma, although cancer-specific questionnaires and the Gastrointestinal Quality of Life Index (GIQLI) have been used. The aim of this study was to validate the GIQLI for patients with periampullary tumours and to evaluate if subscales of the GIQLI could be identified to allow a more detailed assessment of the patients' quality of life., Methods: Patients with periampullary carcinoma, included in a study concerning diagnostic laparoscopy, were asked about symptoms and completed a questionnaire comprising the Medical Outcomes Study (MOS) 24 questionnaire, the GIQLI and one question of the Rotterdam Symptom Check List (RSCL). Clinical interpretation and statistical factor analysis were used to identify subscales of the GIQLI., Results: The GIQLI could be divided into four subscales, measuring physical well-being, mental well-being, digestion and defaecation. All four subscales had a good internal reliability and the construct validity was supported by the pattern of correlations with the MOS and RSCL as well as differences in subscale scores for patients with or without certain symptoms., Conclusion: In patients with periampullary tumours the GIQLI can be divided into four subscales, measuring different aspects of quality of life. These subscales provide insight into the different problems affecting the patient.

Background: Resection offers the only chance of cure to patients with esophageal, gastroesophageal junction, and hepatopancreatobiliary tumors. Staging is essential to select patients who will benefit from operation because palliation can also be performed nonoperatively. Several studies, including limited numbers of patients, have shown that laparoscopic staging prevents unnecessary laparotomies, but it is doubtful whether general application of this staging method can be advised. The aim of this study was to assess the benefit of diagnostic laparoscopy for staging patients with esophageal, gastroesophageal junction, and hepatopancreatobiliary tumors., Study Design: Between June 1992 and December 1996, 420 patients with a resectable tumor after conventional staging underwent diagnostic laparoscopy combined with laparoscopic ultrasonography. Histologic proof of metastases or ingrowth was used to cancel laparotomy., Results: Laparoscopic staging avoided laparotomy in 20% of patients (sensitivity 0.70): 5% with an esophageal tumor, 20% with a gastroesophageal junction tumor, 15% with a periampullary tumor, 40% with a proximal bile duct tumor, 35% with a liver tumor, and 40% with a pancreatic body or tail tumor. Complications and port-site metastases were seen in 4% and 2% of patients, respectively., Conclusions: Laparoscopic staging is a safe procedure with low morbidity and without mortality in this series. It has shown no benefit in esophageal cancer, but seems beneficial for staging tumors located at the gastroesophageal junction, proximal bile duct tumors, liver tumors, and pancreatic body and tail tumors. The value of laparoscopic staging for patients with periampullary tumors is not as great as stated in previous studies and is still the subject of investigation.

Controversy exists about the indication for a palliative pancreatoduodenectomy. A palliative resection for patients with a pancreatic carcinoma can be performed safely nowadays with low mortality and acceptable morbidity in centers with experience. The early results in terms of mortality and morbidity are not different from resections with curative intent or even after bypass surgery. The procedure seems effective for controlling symptoms of the disease, and the quality of life after a palliative resection is acceptable and not worse than after bypass surgery. It is, however, still doubtful whether the incidence of symptom recurrence, such as jaundice, obstruction, and pain, is lower after resection than after bypass surgery. The longer survival after palliative resection could also be due to patient selection and postoperative treatment. There are no randomized trials to prove the superiority of palliative resection over bypass surgery. The safety of pancreatic resection for cancer has already changed the policy in centers with experience, and surgeons are more willing to perform a resection because the results are better or at least the same as after bypass surgery. There are, however, no results to confirm that a palliative resection should be performed routinely or to justify resection as a debulking procedure.

The main benefit of laparoscopic staging (LLU) of (distal) bilio-pancreatic malignancy is the detection of small superficial metastases at the liver surface and peritoneum and local tumor ingrowth. The different aspects of laparoscopic staging have been described extensively in the literature and the benefit is generally expressed in terms of prevention of laparotomy. The impact of this final staging procedure on outcome for the patient in terms of the efficacy of the subsequent non-surgical palliative treatment have been analysed scarsely. In this review the results of laparoscopic staging of bilio-pancreatic tumors concerning the detection of metastases and tumor ingrowth as well as the results of long term follow up after subsequent non-surgical treatment and the preferred palliative treatment after laparoscopic staging are discussed.

Objective: To evaluate the potential benefit of cytology of the peritoneal lavage obtained during diagnostic laparoscopy for staging gastrointestinal (GI) malignancies., Summary Background Data: Peritoneal lavage is a simple procedure that can be performed during laparotomy for GI tumors. Tumor cells in the lavage fluid are thought to indicate intraperitoneal tumor seeding and to have a negative effect on survival. For this reason, peritoneal lavage is frequently added to diagnostic laparoscopy for staging GI malignancies., Methods: Patients who underwent peritoneal lavage during laparoscopic staging for GI malignancies between June 1992 and September 1997 were included. Lavage fluids were stained using Giemsa and Papanicolaou methods. Cytology results were correlated with the presence of metastases and tumor ingrowth found during laparoscopy and with survival., Results: Cytology of peritoneal lavage was performed in 449 patients. Tumor cells were found in 28 patients (6%): 8/87 with an esophageal tumor, 2/32 with liver metastases, 11/72 with a proximal bile duct tumor, 7/236 with a periampullary tumor, and none in 7 and 15 patients with a primary liver tumor or pancreatic body or tail tumor, respectively. In 19 of the 28 patients (68%) in whom tumor cells were found, metastatic disease was detected during laparoscopy, and 3 of the 28 patients had a false-positive (n = 1) or a misleading positive (n = 2) lavage result. Therefore, lavage was beneficial in only 6/449 patients (1.3%); in these patients, the lavage result changed the assessment of tumor stage and adequately predicted irresectable disease. Univariate analysis showed a significant survival difference between patients in whom lavage detected tumor cells and those in whom it did not, but multivariate analysis revealed that these survival differences were caused by metastatic or ingrowing disease., Conclusion: Cytology of peritoneal lavage with conventional staining should no longer be performed during laparoscopic staging of GI malignancies because it provides an additional benefit in only 1.3% of patients and has limited prognostic value for survival in this group of patients.

In recent years, laparoscopic ultrasonography has been introduced as an adjunct to diagnostic laparoscopy for staging of tumors of the upper gastrointestinal tract, liver, biliary tree, and pancreas. It has proved feasible to visualize most anatomic structures in the upper abdomen consistently and in detail with laparoscopic ultrasonography. Recent publications indicate that laparoscopic ultrasonography may be useful for detecting small liver metastases, lymph node metastases, small primary tumors of the pancreas and bile ducts, and for the assessment of the local extension of tumors of the pancreas and stomach. The ongoing improvements in US technology and the results of larger studies will in the near future determine the precise place of this new imaging modality for staging of abdominal tumors.

Background: The major advantage of diagnostic laparoscopy for patients with a gastrointestinal tumor is the prevention of unnecessary explorative laparotomies. However, it is doubtful whether this procedure also prevents late laparotomies that are necessary for palliative treatment during follow-up., Methods: From January 1992 to July 1995, 233 consecutive patients with gastrointestinal malignancies underwent laparoscopy and laparoscopic ultrasonography after routine diagnostic procedures had shown potential curative disease., Results: After diagnostic laparoscopy, laparotomy was not performed in 21% of all patients (47 of 226) because of histologically proven, unresectable, mainly metastatic disease; 6% had esophageal tumors (4 of 64 patients), 43% had liver tumors (10 of 23), 43% had proximal bile duct tumors (9 of 21), 15% had periampullary tumors (17 of 111), and 43% had pancreatic body and tail tumors (3 of 7). Nonoperative palliation was successful in all patients. However, late laparotomies were necessary in 7 of these 47 patients (15%): 5 patients with periampullary tumors and 2 patients with proximal bile duct tumors. All 7 patients underwent a surgical bypass, most due to duodenal obstruction, 1 to 13 months after diagnostic laparoscopy., Conclusions: In this study, diagnostic laparoscopy may have prevented unnecessary laparotomies for exploration or palliation in 18% of all patients (40 of 226). The procedure is of doubtful benefit for patients with esophageal tumors because the current findings show that only 6% of explorative laparotomies could be prevented. In patients with periampullary tumors, the initial benefit was 15%, but the risk of a late laparotomy is relatively high (30%).

The additional value of laparoscopic ultrasonography was evaluated prospectively in 35 patients undergoing diagnostic laparoscopy for a suspected potentially resectable proximal bile duct tumor. Findings were compared with transabdominal ultrasonography, laparoscopy, surgery, and pathology. Laparoscopic ultrasonography was able to visualize the presence and origin of small bile duct tumors or stones and small liver metastases, which could not be seen or could be visualized only doubtfully by ultrasonography and laparoscopy. Laparoscopic ultrasonography was more useful in staging of small tumors of the gallbladder or proximal common bile duct than in staging bifurcation (Klatskin) tumors. Additional information provided by laparoscopic ultrasonography led to a change in diagnosis or tumor stage in eight patients (23%) and to avoidance of laparotomy in three patients (9%).