The incidence of neuroendocrine neoplasms (NEN) is continuously increasing with gastrointestinal tract and pancreas being the most common primary sites. Currently, the guidelines proposed by European Neuroendocrine Tumor Society (ENETS), National Comprehensive Cancer Network (NCCN), European Society for Medical Oncology (ESMO) and North American Neuroendocrine Tumor Society (NANETS) are being widely applied. Among these, ENETS and NANETS guidelines were proposed in 2017 while ESMO and NCCN recently updated their guidelines for gastroenteropancreatic NEN in 2020 and 2021, respectively. This article interprets the diagnosis and treatment of gastroenteropancreatic NEN based on the newly updated ESMO and NCCN guidelines. The diagnosis of gastroenteropancreatic NEN depends on histological assessment including morphological evaluation, grading and immunohistochemistry results. Combination of different imaging methods can help determine tumor staging and risk assessment. Decision-making of treatment and follow-up strategies is based on primary tumor site, tumor classification, tumor grade, tumor type, functional status etc.
Gastrointestinal Neoplasms/therapy* ; Humans ; Incidence ; Neoplasm Staging ; Neuroendocrine Tumors/therapy*

Humans ; Consensus ; Pancreatic Neoplasms/therapy* ; Neuroendocrine Tumors/therapy*

Lung and thymus neuroendocrine neoplasms (NENs) are rare tumors. According to the fifth edition of the World Health Organization classification of thoracic tumors published in 2021, lung and thymus NENs include typical carcinoids, atypical carcinoids, large cell neuroendocrine carcinomas, and small cell carcinomas. Although the incidence of lung and thymus NENs has gradually increased in recent years, there is a lack of randomized controlled clinical study results to guide clinical practice. The treatment of early-stage lung and thymus NENs is complete surgical resection, and the treatment methods for unresectable advanced diseases include different medical treatments, peptide receptor radionuclide therapy, and local therapy. To improve the standardization of diagnosis and treatment of lung and thymus NENs in China, the Expert Committee of Neuroendocrine Neoplasms, Chinese Society of Clinical Oncology developed the expert consensus after multidisciplinary expert discussions based on existing clinical study evidences and guidelines from different neuroendocrine tumor societies. The contents of the consensus cover the epidemiology, diagnosis, pathological classification, staging, treatment and follow-up of lung and thymus NENs (except small cell lung cancer).
Carcinoid Tumor ; China ; Consensus ; Humans ; Lung ; Neuroendocrine Tumors/therapy*

Pulmonary neuroendocrine tumors (PNETs) are a kind of epithelial tumors originating from pulmonary neuroendocrine cells, accounting for about 20% of primary lung tumors, including typical carcinoid, atypical carcinoid, small cell carcinoma, and large cell neuroendocrine carcinoma. The morphologic and clinical characteristics of these four types of PNETs are relatively highly heterogeneous. Immune checkpoint inhibitors (ICIs) have been shown robust antitumor activity in a variety of solid tumors. Treatment regimens of advanced PNETs have developed greatly in the past decade, but ICIs are still in their infancy in the field of PNETs. This review focuses on the landscape of current clinical trials and research as well as the situation of ICIs-related biomarkers in PNETs.
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Carcinoid Tumor ; Carcinoma, Neuroendocrine ; Humans ; Immune Checkpoint Inhibitors ; Lung Neoplasms/drug therapy* ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors/drug therapy*

Neuroendocrine carcinoma (NEC) has been reported to comprise 25% of lung cancer cases. NEC is classified as typical carcinoid, atypical carcinoid, large-cell neuroendocrine carcinoma, and small-cell lung cancer. Carcinoid tumors are less aggressive and surgery is the mainstay of treatment; however, patients with metastatic or inoperable disease need systemic therapy to control carcinoid symptoms and improve survival. Somatostain analogues, targeted agents including everolimus, sunitinib, bevacizumab, and conventional chemotherapy have emerged as treatment options for such patients. Large-cell neuroendocrine carcinoma exhibits more aggressive behavior and has a poorer prognosis than carcinoid tumors. No standard adjuvant and palliative chemotherapeutic agents have yet been established. This review sets out the treatment options for neuroendocrine tumors.
Adenocarcinoma* ; Carcinoid Tumor ; Carcinoma, Neuroendocrine* ; Drug Therapy* ; Humans ; Lung Neoplasms ; Lung* ; Neuroendocrine Tumors ; Prognosis ; Bevacizumab ; Everolimus

Pancreatic neuroendocrine tumors (pNETs) are diverse diseases with different prognosis. Among available various therapeutic options, curative resection should be considered for localized tumors and in some selected cases of metastatic disease. Somatostatin analogs are used to control hormonal symptoms and also effective to inhibit the tumor progression in specific settings. The molecular targeted agents such as sunitinib and everolimus are efficacious treatments for metastatic WHO grade 1/2 pNETs. Chemotherapy is generally used in highly symptomatic and rapidly growing pNETs such as WHO grade 3. In addition, local ablative therapy should be considered in patients with hepatic predominant unresectable metastatic pNETs, and peptide receptor radionucleotide therapy, which is unavailable in Korea, could be considered after failure of initial medical therapy. The most important of all is a multidisciplinary approach to pNETs. This is essential to optimal management of pNETs regarding the diverse disease nature
Drug Therapy ; Everolimus ; Humans ; Korea ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors* ; Prognosis ; Receptors, Peptide ; Somatostatin

The goals of medical treatment for advanced pancreatic neuroendocrine tumor (PNET) are to control hormone-related symptoms and to prolong overall survival. Somatostatin analogues have been used for decreasing the secretion of peptides and relieving symptoms. Recent studies showed that these agents also have an anti-tumor effect and could prolong the survival of patients with advanced PNET. For well-differentiated PNET, streptozocin-based combination chemotherapy has been used for a long time and recent several studies showed survival benefit of targeted agents including angiogenesis inhibitor and mTOR inhibitor. For poorly-differentiated tumors, the number of clinical studies is very limited and the combination of etoposide and cisplatin is widely used. Based on the recent progress in the understanding of tumor biology, newer targeted agents are tested and some of these agents showed promising activity.
Biology ; Cisplatin ; Consensus ; Drug Therapy, Combination ; Etoposide ; Humans ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors ; Pancreas ; Peptides ; Somatostatin

Neuroendocrine tumor in thymus is rare and has poor prognosis due to frequent recurrence and distant metastasis. Approximately half of thymic carcinoids are hormonally active and Cushing's syndrome is seen in 33% of affected patients. Treatment of choice is surgical excision of tumor and role of chemotherapy and radiotherapy is controversal. We report 2 cases of thymic neuroendocrine carcinoma associated with Cushing's syndrome.
Carcinoid Tumor ; Carcinoma, Neuroendocrine ; Cushing Syndrome* ; Drug Therapy ; Humans ; Neoplasm Metastasis ; Neuroendocrine Tumors* ; Prognosis ; Radiotherapy ; Recurrence ; Thymus Gland ; Thymus Neoplasms

A 57-year-old male with periampullary duodenal mass was diagnosed as grade 3 duodenal neuroendocrine carcinoma with multiple liver metastasis. After nine cycles of cisplatin and etoposide, abdominal computed tomography (CT) findings showed complete regression of primary duodenal mass with marked size reduction of liver metastasis. Positron emission tomography findings showed metabolic complete response in both duodenal and liver mass. Pylorus-preserving pancreaticoduodenectomy was done and pathologic finding showed 5 mm sized remnant neuroendocrine tumor. The patient has remained alive with no evidence of disease for 43 months after initial diagnosis. This case suggests the possibility of heterogeneous nature of grade 3 neuroendocrine carcinoma and selected population may have extreme sensitivity to cisplatin and etoposide chemotherapy leading to complete response.
Carcinoma, Neuroendocrine ; Cisplatin ; Diagnosis ; Drug Therapy ; Etoposide ; Humans ; Liver ; Male ; Middle Aged ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Pancreaticoduodenectomy ; Positron-Emission Tomography

Neuroendocrine neoplasms(NEN) is a rare group of tumors with gastrointestinal tract as one of the most common primary locations. The most commonly used guidelines are proposed by European Neuroendocrine Tumor Society (ENETS) and National Comprehensive Cancer Network(NCCN) respectively, while the management of gastrointestinal NEN is generally identical in these two guidelines. Surgery is still the sole curative method which should be considered as the first choice of locoregional NEN. Otherwise, 40% to 50% of patients manifests metastatic disease when diagnosed. Hence, somatostatin analogs, targeted drugs, chemotherapy, and peptide receptor radionuclide therapy should be used comprehensively, especially for unresectable lesions. Therapeutic decision making should be based on the tumor location, functional status, tumor grade, tumor stage, somatostatin receptor status and adverse effect of drugs, etc.
Gastrointestinal Neoplasms ; chemistry ; therapy ; Humans ; Neuroendocrine Tumors ; therapy ; Pancreatic Neoplasms ; Receptors, Somatostatin