FDOPA-PET is superior to 123-I-MIBG scintigraphy in patients with extraadrenal, predominantly noradrenaline-secreting, and hereditary types of PHEO/PGL. The lack of VMAT-1 expression predicts negativity for MIBG-scintigraphy.

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The German NET registry provides valid multicentric data on NET in Germany. Surgical therapy is the most frequent and important therapy with good clinical outcome. In non-resectable, metastatic tumours, systemic therapies are common. Continuation and evaluation of the new WHO and TNM classifications for NET and their therapies will be a future focus of the registry.

Zusammenfassung Hintergrund Seit 2015 erfolgt in Europa mithilfe des EUROCRINE®-Registers eine systematische Dokumentation endokrin-chirurgischer Operationen. Ziel dieser ersten Auswertung war eine Darstellung der Versorgungsrealität für Nebenniereneingriffe in einem homogenen Versorgungsumfeld, entsprechend des deutschsprachigen Raums – bzw. des Präsenzgebiets der Chirurgischen Arbeitsgemeinschaft Endokrinologie (CAEK) der Deutschen Gesellschaft für Allgemein- und Viszeralchirurgie (DGAV) – einschließlich einer Analyse der Adhärenz zu geltenden Therapieempfehlungen. Methodik Es erfolgte eine deskriptive Analyse der präoperativen Diagnostik, der angewandten Operationstechniken sowie der zugrunde liegenden histologischen Entitäten der zwischen den Jahren 2015 und 2019 über EUROCRINE® in Deutschland, Österreich und der Schweiz dokumentierten Nebennierenoperationen. Ergebnisse In den insgesamt 21 teilnehmenden Kliniken des deutschsprachigen EUROCRINE®-Gebiets wurden 658 Operationen an Nebennieren durchgeführt. In 90 % erfolgten unilaterale, in 3 % bilaterale Adrenalektomien und in 7 % andere Resektionsverfahren. Die in 41 % der Operationen dokumentierte histologische Hauptdiagnose war das adrenokortikale Adenom. In 15 % lagen maligne Befunde zugrunde (einschließlich 6 % Nebennierenrindenkarzinome (ACC) und 8 % Nebennierenmetastasen). 23 % der Operationen erfolgten bei Phäochromozytomen. Diese wurden zu 82 % minimal-invasiv operiert, Nebennierenrindenkarzinome lediglich zu 28 % und Nebennierenmetastasen zu 66 %. Schlussfolgerung Überraschenderweise wurden nach Nebennierenadenomen und Phäochromozytomen an dritthäufigster Stelle Nebennierenmetastasen unterschiedlicher Primärtumoren reseziert. 28 % der ACC waren für minimal-invasive Techniken vorgesehen, wobei 20 % dieser Fälle eine Konversion zur offenen Operation erforderten. Die aktuelle Analyse deckte Diskrepanzen zwischen Versorgungsrealität und Leitlinienempfehlungen auf, aus denen sich zahlreiche Fragestellungen ergeben, welche nun in ein überarbeitetes EUROCRINE®-Modul zur Dokumentation von Nebennierenoperationen einfließen werden.

Thyroid nodules represent heterogeneous tumors with distinct molecular signatures. While benign thyroid nodules correspond to poly- or monoclonal tumors, thyroid carcinomas are monoclonal and thus "real" neoplasms. These are caused by somatic mutations that lead to the constitutive activation of specific signaling cascades and determine the corresponding histology and also partly the functional phenotype of the thyroid tumor. Dedifferentiation of thyroid carcinomas is accompanied by the occurrence of additional mutations in the tumors. The mutation load of thyroid carcinomas correlates with their biological behavior. In clinical practice, detection of somatic mutations can help in the cytological differential diagnosis. In the prognostic assessment of thyroid tumors, proof of classical oncogene mutations (BRAF, RAS) has little relevance. Other genetic alterations, especially TERT promoter mutations that occur with increasing frequency in advanced thyroid carcinomas, probably have a prognostic significance. The molecular signature, however, is of great relevance for the development and application of targeted therapies in advanced carcinomas (radioactive iodine-refractory DTC, PDTC and ATC, metastatic medullary carcinoma). For this, there is increasing evidence from clinical studies and case reports that underline the concept of "oncogene addiction" as a pathogenetically relevant mechanism of thyroid tumorigenesis and carcinogenesis.

Pheochromocytomas and paragangliomas are rare chromaffin tumours that represent an exceptional challenge for the surgeon because of the concomitant secretion of catecholamines. Recent findings on the genetic background of hereditary tumours have challenged the rule of the 10 % -tumour and significantly changed the requirements for preoperative work-up and surgical strategy. Early detection of malignant growth or multiple hereditary tumours is the goal of imaging techniques such as CT/MRI, (123)I-MIBG-(SPECT) or (18)F-DOPA-PET. However, in the absence of metastasis, reliable differentiation between -benign and malignant growth is preoperatively and even histopathologically rarely possible. An essential precondition for successful surgical therapy with low operative risks is an adequate pretreatment with alpha-adrenergic antagonists which should slowly be increased to 3-5 mg/kg BW/day prior to resection. Dopamine-secreting paragangliomas represent the sole exception. Minimally invasive techniques using a transabdominal or retroperitoneal approach have become the gold standard for the resection of unifocal benign pheochromocytomas. In addition, most paragangliomas located below the diaphragm can be resected with a minimally invasive approach which, however, demands exceptional expertise. Open transabdominal resections are an approved therapy for large or potentially -malignant tumours and for settings with multi-focal tumour sites. Even for advanced malignant tumours, surgical debulking may be reasonable to improve the patient's quality of life and prognosis.

Neuroendocrine tumours (NET) of the stomach have increasingly entered into the focus of attention recently by endocrinologists as well as by surgeons on account of new findings on their biological background and their clinical relevance. The discriminating pathogenesis and the basically different biological behaviours of neuroendocrine tumours of the stomach require a differentiated therapeutical treatment for the different sub-types. The differential diagnostic clarification of the basic pathomechanism is therefore of decisive importance. As a consequence of the rarity of this entity, any treatment should be performed in corresponding centres. An interdisciplinary therapy planning, e. g., in the context of specialised inter-disciplinary conferences, such as an interdisciplinary board for endocrine and neuroendocrine tumours, is essential for an optimised management of these tumours. Important aspects of future research should - besides the genetic and molecular biological backgrounds - be the additional and potentially apparent factors for hypergastrinaemia, which induce a dysplastic and perhaps malignant transformation of the hyperplastic changes. Neuroendocrine tumours of the stomach remain a special challenge within the clinical daily routine - not only in the correct classification but also in the optimal surgical therapy.

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