Neuroendokrine Neoplasien der Lunge

Abstract

The pulmonary neuroendocrine neoplasms originate from the enterochromaffin cells which are diffusely distributed in the body. The incidence of these tumors has increased significantly in recent decades due to the available diagnostics. They make up about 1-2% of all lung tumors and 20-30% of all neuroendocrine neoplasms. The current WHO classification from 2004 divides them into typical carcinoids (TC), atypical carcinoids (AC), large cell neuroendocrine carcinomas (LCNEC) and small cell carcinomas (SCLC). The major neuroendocrine biomarkers are chromogranin A, synaptophysin and CD56. TC have a low mitotic rate of <2 mitoses/2mm(2) (10 HPF), whereas the mitotic rate of the AC is 2-10 mitoses/2 mm(2) (10 HPF). The Ki-67 staining is helpful to distinguish typical and atypical carcinoids from the highly malignant LCNEC and SCLC. Clinically, the patient presents usually with cough, hemoptysis or bronchial obstruction. The occurrence of a carcinoid or Cushing's syndrome and a tumor-associated acromegaly are rare. Surgical resection with radical lymph node dissection is the treatment of choice for achieving long-term survival. Endoscopic resection of the endobronchial tumor growth is a good alternative for inoperable endobronchially localized tumors. Peptide receptor radionuclide therapy (PRRT) is a promising treatment option for patients with metastatic or unresectable pulmonary neuroendocrine tumors. New targeted therapies using angiogenesis inhibitors, mTOR inhibitors, and tyrosine kinase inhibitors are being tested for their effectiveness in many previous studies. Typical carcinoid tumors metastasize less frequently than AC, the 5-year survival rate of patients with TC being over 90%. Patients with AC have a 5-year survival rate between 35% and 87%. The highly malignant LCNEC and SCLC, on the other hand, have a 5-year survival rate between 15% and 57%, and <5% respectively. The increasing number of therapeutic options and diagnostic procedures requires a multidisciplinary approach and decision-making in multidisciplinary tumor conferences to ensure a personalized treatment approach. Therefore patients with a neuroendocrine neoplasm of the lung should be treated in specialized centers.

Comparative evaluation of three proliferation markers, Ki-67, TOP2A, and RacGAP1, in bronchopulmonary neuroendocrine neoplasms: Issues and prospects

“…There were also high and comparable expansions in the Ki-67 expression rates from AC to SCLC/LCNEC for each of the three methods. In the literature it is often described that Ki-67 mainly helps to distinguish low-grade from high-grade BP-NEN [12, 30], but our results also revealed that there was an increase in AC compared with TC. As expected, compared with the Ki-67-Average evaluation, the Ki-67 levels were higher in the Hotspot evaluation in cases of AC, SCLC, and LCNEC, but not TC.…”

“…The distinction between the four BP-NEN entities is clinically very important because the subsequent classification-based diagnostic and therapeutic approaches differ due to their differential aggressiveness [3, 5, 6, 29, 30]. Mitosis counting is part of the classification procedure (based on the WHO and IASLC criteria); it is a very time-consuming process, and has a relatively high inter-observer variability [6, 8, 9].…”

Diagnostic and Management Pathways for Pulmonary Carcinoid Tumours in the United Kingdom: Results from the National Lung Neuroendocrine Tumour Pathway Project

“…e increasing variety of diagnostic and therapeutic approaches for patients with TC and AC [31], along with challenges in achieving accurate diagnosis and long-term follow-up of patients, has led to recommendations for a multidisciplinary approach from the care teams, involving collaboration between medical staff with a set of distinct expertise, to ensure each individual patient receives an International Journal of Endocrinology optimal disease management plan [8]. It has been suggested that multidisciplinary care in specialised centres such as ENETS CoE [23], or the North American equivalent (multidisciplinary reference centres) [32], could improve clinical outcomes for patients with TC/AC.…”

18F-DOPA vs. 18F-FDG PET/CT in the ectopic ACTH syndrome due to pulmonary carcinoid tumor

“…The low prevalence of thyroid abscess is related to thyroid gland resistance to infection due to its fibrous capsule, high blood perfusion, high iodine contents, and significant lymphatic drainage, and to anatomical separation of the gland from the airway. 1 AST may arise from hematogenous or lymphatic dissemination, continuity from adjacent organs, or direct inoculation after fine needle aspiration (FNA). 2 The predisposing factors vary depending on age at presentation.…”

“…Their incidence has increased significantly in recent decades due to the available diagnostic resources; they represent about 1---2% of all lung tumors and 20---30% of all NET. 1 6-Fluoro-(18F)-l-3,4-dihydroxyphenylalanine (18F-DOPA) is an aminoacid-analog for positron emission-tomography (PET) imaging which has been registered since 2006 in several European Union countries and by several pharmaceutical firms. NET imaging is part of its registered indications.…”