Journal of Clinical Trials: Pancreatic, Gastrointestinal, Pulmonary, Bronchial, and Thymic Neuroendocrine Neoplasm


Journal of Clinical Trials is an open-access journal that publishes & accepts all types of articles that comes under the scope of the journal. JCTR published an article on the titled AMulticenter,ProspectiveRegistryofPatientswithPancreatic,Gastrointestinal,Pulmonary,BronchialandThymicNeuroendocrineNeoplasm.


Both the diagnosis and the treatment of patients with neuroendocrine neoplasm (NEN) have recently improved globally. Since little data has been presented on the current situation of NEN treatment in Japan, Japan Neuroendocrine Tumor Society (JNETS) established the Japan NEN Registry study and constructed a registry of Japanese NEN patients with a primary site of the pancreas gastrointestinal tract, lungs, bronchi, and thymus in order to clarify the current status of NEN treatment in Japan.

Neuroendocrine neoplasm (NEN) is a type of tumor arising from neuroendocrine cells, which produces various kinds of hormones, some of which cause severe clinical symptoms. It has become clear that NEN can occur in any organ throughout the body due to the diffuse neuroendocrine system.

In general, NEN is an uncommon disease. According to the Surveillance, Epidemiology and End Results (SEER) program in the United States, the number of patients has been increasing and the annual incidence was 5.25 per 100,000 population in 2004, approximately 5-times greater than 1.09 per 100,000 population reported in 1973 [1]. The reason for this increase has not yet been clarified, but the improvement of disease recognition and diagnostic techniques might be considered major reasons.

According to Ito et al., the annual incidence of pancreatic NEN (PanNEN) in Japan was estimated to be 1.01/100,000 population in 2005, and that of gastrointestinal NEN (GI-NEN) was 2.10/100,000. They conducted a second survey in 2010, and reported that each of PanNEN and GI-NEN incidences had increased. Interestingly, the incidence of midgut NEN was lower in Japan compared to the data reported from the SEER database or other European registries. Similar lower incidences of midgut NEN have been reported from other Asian countries. Achieving a more precise analysis of NEN patients in Japan thus seems likely to contribute to a more comprehensive understanding of NEN, at least for Asian NEN patients.

Recently, a new classification method based on the growth kinetics of tumor cells (number of cell nuclear fission images and Ki67 index [%]) was proposed in the 2010 World Health Organization (WHO) classification [7] for PanNEN and GI-NEN. NEN has been classified as NET G1/G2 (Grade 1/Grade 2) and neuroendocrine carcinoma (NEC). Several articles have discussed whether the present cutoff value for Ki67 index is optimal. We consider this to be an urgent problem concerning Asian NEN patients. In addition, although surgical treatment is the first choice for patients with resectable NEN, several arguments have remained regarding what type of operation is the best suitable for prolongation of patient survival and how the surgery should be performed for patients with distant metastases. More precise analysis with registry is necessary to solve these problems for gastroenteropancreatic NEN patients.

Pulmonary, bronchial, and thymic neuroendocrine tumors comprised of malignant carcinoma such as large-cell neuroendocrine carcinoma (LCNEC) and small cell lung cancer (SCLC), and typical and atypical carcinoid. Those tumors form a pathologically and clinically heterogeneous group. According to the SEER program in the United States, the total annual incidence of pulmonary and bronchial NEN since 2003 is 1.57/100,000. SCLC is the most common pulmonary and bronchial NEN, representing 15%–20% of invasive lung cancers, and LCNEC is said to make up 1.6%–3% of resectable lung cancers. Thymic NEN is a very rare cancer; with the incidence of 0.02/100,000 people annually in the SEER report. According to the Japan Surgical Society statistics in a study of surgical cases in Japan, carcinoid was seen in 198 patients (0.6%), LCNEC in 492 patients (1.4%), and SCLC in 581 patients (1.7%) among 34,228 primary lung cancer patients in 2011 [15]. Thymic NEN was seen in only 41 patients (0.9% of 4,463 thymic tumors). Surgery is the mainstay of the treatment of Typical Carcinoid (TC) and Atypical Carcinoid (AC) based on the general principle of complete resection with preservation of as much normal lung tissue as possible. LCNEC has conventionally been handled as a large-cell lung cancer with a pathology differing from that of small cell lung cancer. LCNEC is a challenging tumor, and its poor prognosis is also related to the difficulty of preoperative diagnosis.

Although treatment and diagnosis of NEN have changed globally in recent years, the situation of current clinical practice in Japan has not been clearly identified. To improve the outcome of NEN treatment in Japan, it is necessary to clarify pathology and prognosis of NEN as well as patient distribution.

Thus we established a registry system for NEN patients with the primary site of pancreas, gastrointestinal tract, lungs, bronchi, and thymus in Japan.

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Thanks & Regards,

Managing Editor
Journal of Clinical Trials