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 (GEP-NETs) 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 diagnosed by fine-needle aspiration of the primary or metastases, and 2.5% were diagnosed during autopsy; 44.5% of tumors were not suspected clinically and were diagnosed incidentally during various surgical procedures. Overall, 18.7% of tumors were removed endoscopically and 67.6% were removed surgically; 13.7% of patients were followed without interventional treatment. Endoscopic or surgical intervention was curative in 81.4% of patients and palliative in 18.6% of patients. At the time of diagnosis, information on metastasis was available in 83.7% of patients with malignant NETs. Lymph node or distant metastases were documented in 74.7% of patients. In 19.3% of patients, 41 secondary tumors were documented, with 78.0% classified histologically as adenocarcinomas. Conclusion. This investigation summarizes the clinical presentation and current practice of management of GEP-NETs and thereby extends the understanding and clinical experience.

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.

Calcium-stimulated calcitonin - The “new standard” in the diagnosis of thyroid C-cell disease - clinically relevant gender-specific cut-off levels

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⁻¹h⁻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⁻¹m⁻²,p = 0.005; HIAT: 2.08 vs. 2.56 l min⁻¹m⁻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.

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.