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Objective: To evaluate the impact of multidisciplinary team (MDT) meetings on the management of patients with neuroendocrine neoplasms (NENs). Methods: All newly referred gastro‐entero‐pancreatic (GEP)‐NEN patients discussed from 1 April to 1 October 2017 in the MDT of seven European expert centres were prospectively included. The impact on patients' management was defined as a change in diagnosis, grade, stage or treatment. Results: A total of 292 patients were included, mainly small intestinal (siNENs) (32%) and pancreatic NENs (28%), with distant metastases in 51%. Patients had received prior surgery in 43% of cases and prior medical treatment in 32%. A significant change occurred in 61% of NENs: 7% changes in diagnosis, 8% in grade and 16% in stage. The MDT recommended a new treatment for 51% of patients, mainly surgery (9%) or somatostatin analogues (20%). A significant change was most frequently observed in patients with Stage IV disease (hazard ratio [HR] 3.6, 95% confidence interval [CI]: 1.9–6.9 vs. Stage I) and G2 NENs (vs. G1, HR 2.1 95% CI: 1.2–3.8). Conclusion: NEN‐dedicated MDT discussion in expert centres yields significant management changes in over 60% of patients and thus represents the gold standard for the management of these patients. [ABSTRACT FROM AUTHOR]

BACKGROUND: Clinical pathways are care plans established to describe essential steps in the care of patients with a specific clinical problem. They translate (inter)national guidelines into local applicable protocols and clinical practice. The purpose of this article is to establish a multidisciplinary integrated care pathway for specialists and allied health care professionals in caring for individuals with von Hippel–Lindau (VHL) disease. METHODS: Using a modified Delphi consensus‐making process, a multidisciplinary panel from 5 Dutch University Medical Centers produced an integrated care pathway relating to the provision of care for patients with VHL by medical specialists, specialized nurses, and associated health care professionals. Patient representatives cocreated the pathway and contributed quality criteria from the patients' perspective. RESULTS: The panel agreed on recommendations for the optimal quality of care for individuals with a VHL gene mutation. These items were the starting point for the development of a patient care pathway. With international medical guidelines addressing the different VHL‐related disorders, this article presents a patient care pathway as a flowchart that can be incorporated into VHL expertise clinics or nonacademic treatment clinics. CONCLUSIONS: Medical specialists (internists, urologists, neurosurgeons, ophthalmologists, geneticists, medical oncologists, neurologists, gastroenterologists, pediatricians, and ear‐nose‐throat specialists) together with specialized nurses play a vital role alongside health care professionals in providing care to people affected by VHL and their families. This article presents a set of consensus recommendations, supported by organ‐specific guidelines, for the roles of these practitioners in order to provide optimal VHL care. This care pathway can form the basis for the development of comprehensive, integrated pathways for multiple neoplasia syndromes. [ABSTRACT FROM AUTHOR]

Background Serotonin secretion occurs in approximately 1%-4% of patients with a pancreatic neuroendocrine tumour ( PNET), but the incidence is not well defined. The aim of this study was to determine the incidence of serotonin secretion with and without carcinoid syndrome and the prognostic value for overall survival ( OS). Methods Data were collected from 255 patients with a PNET if 24-hours urinary 5-hydroxyindoleacetic acid excretion (5- HIAA) was assessed. Patients were diagnosed with serotonin secretion if 24-hours urinary 5- HIAA excretion was more than 3× the upper limit of normal ( ULN) of 50 μmol/24 hours during follow-up. The effect of serotonin secretion on OS was estimated with uni- and multivariate analyses using a Cox regression. Results Two (0.8%) patients were diagnosed with carcinoid syndrome, and another 20 (7.8%) had a serotonin-secreting PNET without symptoms. These patients mostly had ENETS stage IV disease with high chromogranin A (CgA). Serotonin secretion was a negative prognostic factor in univariate analysis ( HR 2.2, 95% CI: 1.27-3.81), but in multivariate analysis, only CgA>10× ULN ( HR: 1.81, 95% CI: 1.10-2.98) and neuron-specific enolase ( NSE) > ULN ( HR: 3.51, 95% CI: 2.26-5.46) were predictors for OS. Immunohistochemical staining for serotonin was positive in 28.6% of serotonin-secreting PNETs (one with carcinoid syndrome) and negative in all controls. Conclusion Carcinoid syndrome is rare in patients with a PNET, but serotonin secretion occurs often. This is a negative prognostic factor for OS, but after correction for CgA and NSE, it is no longer a predictor and probably only a 'not-so innocent bystander' in patients with high tumour burden. [ABSTRACT FROM AUTHOR]

Introduction Methods Results Discussion The 2015 American Thyroid Association guidelines recommend de‐escalating surgical treatment for papillary thyroid cancer (PTC). We hypothesize that the Dutch PTC population might differ due to a restrictive diagnostic policy that mainly selects symptomatic and palpable thyroid nodules for further diagnostics, potentially selecting relatively more aggressive tumors. We aimed to describe the Dutch PTC population because differences in populations can have consequences for the adoption of foreign guidelines.From the Dutch national cancer registry, patients diagnosed with pT1–T3 PTC between 2005 and 2015 were included. Baseline characteristics, disease‐free interval, and overall survival were compared between low‐risk and non‐low risk PTC. Furthermore, the TNM stage of the Dutch and U.S. cohorts were compared via literature search.Of the 3368 pT1‐T3 patients included, 1813 (53.8%) had a low‐risk PTC, and 1555 (46.2%) had a non‐low‐risk PTC. In the Dutch PTC population, pT1 tumors occurred in 45.8%, pT2 and pT3 tumors occurred in 34.9% and 19.3% of the patients, respectively. Of all patients, 10.2% had central lymph node metastases and 16.6% had lateral lymph node metastasis. Distant metastasis only occurred in 18 (0.5%) of the patients. The 10‐year overall survival was 89.6%, with rates of 91.6% for low‐risk and 87.3% for non‐low‐risk patients (p = < 0.001). During the follow‐up period, 257 patients (7.6%) had a recurrence.The higher frequency of advanced tumors among the Dutch PTC population in contrast to the U.S. emphasizes the need for careful national data analyses before the adoption of surgical intervention de‐escalation protocols from other countries. [ABSTRACT FROM AUTHOR]