As incidence data on gastroenteropancreatic neuroendocrine tumours (GEP-NETs) have so far only been retrospectively obtained and based on inhomogeneous material, we conducted a prospective study in Austria collecting all newly diagnosed GEP-NETs during 1 year. Using the current WHO classification, the tumor, nodes, metastases (TNM) staging and Ki67 grading and the standard diagnostic procedure proposed by the European Neuroendocrine Tumor Society (ENETS), GEP-NETs from 285 patients (male: 148; female: 137) were recorded. The annual incidence rates were 2.51 per 100 000 inhabitants for men, 2.36 per 100 000 for women. The stomach (23%) was the main site, followed by appendix (21%), small intestine (15%) and rectum (14%). Patients with appendiceal tumours were significantly younger than patients with tumours in any other site. About 46.0% were classified as benign, 15.4% as uncertain, 31.9% as well differentiated malignant and 6.7% as poorly differentiated malignant. Patients with benign or uncertain tumours were significantly younger than patients with malignant tumours. Among the malignant tumours of the digestive tract, 1.49% arose from neuroendocrine cells. For malignant gastrointestinal NETs, the incidence was 0.80 per 100 000: 40.9% were ENETS stage I, 23.8% stage II, 11.6% stage III and 23.8% stage IV. The majority (59.7%) were grade 1, 31.2% grade 2 and 9.1% grade 3. NETs of the digestive tract are more common than previously reported; the majority show benign behaviour, are located in the stomach and are well differentiated. G3 tumours are very rare.

Background. The aim of this prospectively collected, retrospectively analyzed clinical investigation was to describe "unmasked" clinical symptoms and methods of diagnosis, treatment, and short-term follow-up of gastroenteropancreatic neuroendocrine tumors (GEPNETs) diagnosed during 1 year in Austria.Methods. In total, 277 patients with GEP-NETs were documented. All tumors were immunhistochemically defined according to recently summarized criteria (World Health Organization, European Neuroendocrine Tumour Society). A standardized questionnaire comprising 50 clinical and biochemical parameters (clinical symptoms, mode of diagnosis, treatment, follow-up) was completed by attending physicians.Results. The most common initial symptoms were episodes of abdominal pain, diarrhea, weight loss, gastrointestinal bleeding, flushing, and bowel obstruction. Overall, 48.1% of tumors were diagnosed by endoscopy, 43.7% were diagnosed during surgery, 5% were diag-

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BackgroundAt the time of diagnosis, one-third of medullary thyroid carcinoma (MTC) patients show lymph node (LN) or distant metastasis. A metastasized MTC requires different surgical strategies.ObjectiveThis study aimed to determine the value of ultrasound and [18F]fluoro-dihydroxyphenylalanine positron emission tomography with computed tomography (F-DOPA-PET-CT) in localizing MTC, as well as LN and distant metastasis.MethodsThe study included 50 patients (24 males/26 females) with preoperative ultrasound, F-DOPA-PET-CT, and histologically proven MTC. Imaging results were correlated with both preoperative basal calcitonin (bCt) levels and final histology.ResultsTumors were classified as pT1a:17 (diameter, mean ± standard deviation: 5.8 ± 3.0 mm), pT1b:15 (15.0 ± 3.2 mm), pT2:9 (27.3 ± 7.0 mm), and pT3:9 (38.3 ± 24.2 mm). The median bCt level was 202 pg/mL (lower/upper quartile: 82/1074 pg/mL). Ultrasound was positive for tumor in 45/50 (92%) patients (20.0 ± 16.0 mm) and negative in 5 patients (3.2 ± 2.2 mm). Overall, 43/50 (86%) patients had positive F-DOPA local scans (20.0 ± 16.4 mm), while 7 (14%) patients were negative (7.7 ± 8.1 mm). Lastly, 21/50 (42%) patients had LN metastasis; 8/21 (38%) patients had positive LNs suspected with ultrasound, and 12/21 (57%) patients had positive LNs suspected with F-DOPA. Tumor and LN sensitivity of ultrasound was 92% and 43%, respectively, and 86% and 57% of F-DOPA-PET-CT, respectively. In 3/50 (6%) patients and 3/50 (6%) patients, mediastinal LN metastasis and distant metastasis, respectively, were diagnosed only by F-DOPA-PET-CT.ConclusionUltrasound and F-DOPA-PET-CT are sensitive for the localization of MTC but not for the presence and location of LN metastasis (limitations: size/number). Only F-DOPA ensures the diagnosis of distant metastasis and influences the extent of LN surgery. Surgical strategy cannot be predicted based on neither ultrasound nor F-DOPA-PET-CT.

IntroductionPentagastrin (Pg) stimulated calcitonin (sCT) was used to enhance accuracy in medullary thyroid cancer (MTC) diagnosis. As it is now unavailable, calcium (Ca) has been recommended as an alternative. The aim of this study was to define gender-specific cut-off values to predict MTC in patients with elevated basal CT (bCT) following Pg-sCT and Ca-sCT stimulation and to compare the time courses of CT release during stimulation.Materials and methodsThe stimulation tests were applied in 62 consecutive patients with thyroid nodules. Basal calcitonin was measured by chemiluminescent immunometric assay. All patients underwent thyroidectomy and bilateral central neck dissection. C-cell pathology was confirmed by histological and immunohistochemical evaluation.ResultsIn 39 (0.63) patients MTC was documented while isolated C-cell hyperplasia (CCH) was identified in 23 (0.37) patients. Medullary thyroid cancer was predicted in males with bCT values > 43 pg/mL or sCT concentrations > 470 pg/mL (Pg-sCT) or > 1500 pg/mL (Ca-sCT), and in females with bCT concentrations > 23 pg/mL or sCT concentrations > 200 pg/mL (Pg-sCT) or > 780 pg/mL (Ca-sCT), respectively. Pg-sCT correctly predicted MTC in 16 (0.66) compared to 13 (0.54) after Ca-sCT in males and in 12 (0.80) compared to 11 (0.73) in females; without statistical significance. In patients with CCH or low tumor burden, there was a tendency of faster CT release after Ca stimulation (CT peak after 3min in > 60%) compared to patients with advanced MTC (CT peak after 3min in < 10%).ConclusionsUsing gender-specific cut-off values, Ca could replace Pg to predict MTC with similar diagnostic power.

Background Medullary thyroid cancer can be subdivided during surgery into tumours with or without a desmoplastic stromal reaction (DSR). DSR positivity is regarded as a sign of disposition to metastasize. The aim of this study was to analyse whether lateral lymph node dissection can be omitted in patients with DSR-negative tumours. Methods This was a retrospective cohort study of a prospectively maintained database of patients with medullary thyroid cancer treated using a standardized protocol, and subdivided into DSR-negative and -positive groups based on the results of intraoperative frozen-section analysis. Patients in the DSR-negative group did not undergo lateral lymph node dissection. Long-term clinical and biochemical follow-up data were collected, and baseline parameters and histopathological characteristics were compared between groups. Results The study included 360 patients. In the DSR-negative group (17.8 per cent of all tumours) no patient had lateral lymph node or distant metastases at diagnosis or during follow-up, and all patients were biochemically cured. In the DSR-positive group (82.2 per cent of all tumours), lymph node and distant metastases were present in 31.4 and 6.4 per cent of patients respectively. DSR-negative tumours were more often stage pT1a and were significantly smaller. The median levels of basal calcitonin and carcinoembryonic antigen were significantly lower in the DSR-negative group, although when adjusted for T category both showed widely overlapping ranges. Conclusion Lymph node surgery may be individualized in medullary thyroid cancer based on intraoperative analysis of the DSR. Patients with DSR-negative tumours do not require lateral lymph node dissection.

Although the majority of neuroendocrine tumors of the small intestine (siNETs) classified as low-grade G1 or G2 show slow local growth, they are frequently diagnosed at an advanced stage of metastatic disease. The surgical treatment is curative in stages I-III or palliative in stage IV in an attempt to avoid local complications of bowel obstruction and ischemia of the small bowel by unremoved lymph node metastases. Individualized surgical procedures performed by experienced surgeons considering tumor multifocality and the primary extent of lymph node metastases along the mesenteric vessels are recommended to remove as much tumor volume as possible, while avoiding major complications intraoperatively and small bowel syndrome postoperatively.

Background Hemodynamic instability is frequently observed during adrenalectomy for pheochromocytoma (PCC). Guidelines recommend liberal preoperative volume administration. However, it is unclear whether fluid deficiency or vasoplegia causes shifting hemodynamics and whether minimally invasive hemodynamic monitoring with esophageal Doppler (EDM) can help visualize intraoperative changes avoiding volume overload and complications. Methods Ten patients with biochemically verified PCC and five patients with hormonally inactive adrenal tumors (HIAT; control group) were treated following a strict protocol. During laparoscopic adrenalectomy, goal-directed fluid therapy was performed using EDM. Hemodynamic and biochemical data were documented. The primary outcome variables were fluid requirement and hemodynamic parameters. Results Applying EDM, total intraoperative fluid administration was slightly higher in PCC patients than in patients with HIAT (2100 ± 516 vs. 1550 ± 622 ml, p = 0.097; 12.9 ± 4.8 vs. 8.3 ± 0.7 ml kg −1 h −1 , p = 0.014). Hemodynamics varied considerably within the PCC group and was associated with type and level of secreted catecholamines. Arterial blood pressure and systemic vascular resistance index reached their minimum in the 10-min period after resection of PCC. Without liberal fluid administration, an increase in cardiac index was observed in both groups comparing baseline measurements to end of surgery. This increase was statistically significant only in PCC patients (PCC: 2.31 vs. 3.15 l min −1 m −2 , p = 0.005; HIAT: 2.08 vs. 2.56 l min −1 m −2 , p = 0.225). Conclusions As vasoplegia, but not hypovolemia, was documented after tumor resection, there is no evidence that PCC patients profit from liberal fluid administration during laparoscopic adrenalectomy. To avoid volume overload, noninvasive techniques such as EDM should be routinely used to visualize the variable intraoperative course. Trial registration: ClinicalTrials.gov, Identifier: NCT01425710.