A systematic review of the relevant literature was carried out, and this was followed by appraisal and expert review. At this stage, there has not been a move to change toward the WHO terminology for gastroenteropancreatic (GEP) NETs.
The terminology used reflects the current practice of the term ‘carcinoid’ in pulmonary disease, specifically typical (TC) and atypical (AC) carcinoids. All agree that the management of such patients should involve a multidisciplinary tumor board with a specific interest/expertise in NETs. There is a paucity of randomized studies and as the disease is relatively uncommon the level of evidence is limited compared with more common cancers. This ‘expert’ consensus opinion arises from two meetings of the ENETS Advisory Board held in 20. This expert consensus document represents an initiative by the European Neuroendocrine Tumor Society (ENETS) to provide guidance on the management of pulmonary carcinoid (PC)/well-differentiated neuroendocrine tumors (NETs) taking into account advances in the characterization of NETs as well as improvements in both diagnosis and treatment. Systemic chemotherapy is used for progressive PCs, although cytotoxic regimens have demonstrated limited effects with etoposide and platinum combination the most commonly used, however, temozolomide has shown most clinical benefit. Locoregional or radiotargeted therapies should be considered for metastatic disease. Somatostatin analogs are the first-line treatment of carcinoid syndrome and may be considered as first-line systemic antiproliferative treatment in unresectable PCs, particularly of low-grade TC and AC. Resection of metastases should be considered whenever possible with curative intent. Surgery is the treatment of choice for PCs with the aim of removing the tumor and preserving as much lung tissue as possible. Plasma chromogranin A can be increased in PCs. Somatostatin receptor imaging may visualize nearly 80% of the primary tumors and is most sensitive for metastatic disease.
Contrast CT scan is the diagnostic gold standard for PCs, but pathology examination is mandatory for their correct classification. typical (TC) and atypical carcinoid (AC), respectively. PCs are well-differentiated neuroendocrine tumors and include low- and intermediate-grade malignant tumors, i.e.