Gastroenteropancreatic neuroendocrine neoplasms are a large and very diverse group of neoplasms. They are becoming a burning clinical problem because of increasing frequency and diagnosis in the advanced state. The treatment landscape has been changed over the last years. Treatment choice depends on many factors such as the tumor’s type, location, aggressiveness, and hormone-producing capabilities. The main goals of treatment are long-term symptomatic control, antitumor effect, and improvement of the quality of life. The results of the PROMID and CLARINET trials have augmented fundamental position of somatostatin analogs. Our understanding of the biology, genetics of the neoplasms has improved considerably in the last several decades and the spectrum of available therapeutic options is rapidly expanded. The current evidence-based treatment options include everolimus, sunitinib, peptide receptor radionuclide therapy, and chemotherapy. Treatment practice changed as a result of high-quality phase 3 clinical trials which shaped current guidelines; multiple retrospective studies which raised new questions and attempted to fill some of the data gaps. Here we review the treatment options for gastroenteropancreatic neuroendocrine neoplasms, discussing important diagnosis and biomarker-related factors, safety of therapy with special insight into cardiac safety, as well we looked at promising investigative therapies.
Most patients with obstructive jaundice caused by an etiology other than choledocholithiasis are at risk of adenocarcinoma development. Other types of tumors are less common, although more benign. The presented study described management in case of liver hilar tumors with special regards to common hepatic duct carcinoids
Gastroenteropancreatic neuroendocrine tumors (GEP NET) represent heterogenous group of very rare neoplasms. Nevertheless, these tumors have been increasingly diagnosed recently. Authors present own experience with surgical treatment of gastrointestinal neuroendocrine tumors.The aim of the study was to analyse retrospectively own material of patients with gastrointestinal neuroendocrine tumors treated surgically in the Department of General and Transplant Surgery of Medical University in Łódź.Material and methods. The analysis included all patients with neuroendocrine tumors surgically treated from January 2007 to June 2009 in the Department of General and Transplant Surgery of Medical University in Łódź. The clinical patients data were obtained from medical histories, operative protocols and outcomes of final histopathological examinations. Analyzed data were as follows: age, gender, type and localization of tumor, clinical signs, results of preoperative tests and type of surgical procedure.Results. Analysis revealed that 17 patients were operated on for gastrointestinal neuroendocrine tumors between years 2007-2009 in the Department of General and Transplant Surgery. Foregut tumors (5 gastric neuroendocrine tumors and 4 pancreatic insulinomas), midgut tumors (1 neuroendocrine cancer of ascending colon, 3 hepatic neuroendocrine cancers metastases, 2 primary hepatic neuroendocrine cancers, 1 gall bladder neuroendocrine cancer) and hindgut tumors (neuroendocrine cancer of rectum) were diagnosed in nine cases (53%), in seven cases (41%) and in one case (5%), respectively. Wide range of surgeries were performed in the Department, as follows: in 2 cases right hemihepatectomy, in 3 cases extended right hemihepatectomy, in 1 case left hemihepatectomy, in 4 cases pancreatic tumor enucleation, in 2 cases gastric resection, in 3 cases gastrectomy, in 1 case right hemicolectomy and in 1 case anterior resection of the rectum. The vast majority (11/17 patients; 64%) of tumors were poorly-differentiated neuroencorine carcinomas with high grade of histological malignancy. In one case coincidence of insulinoma and nesidioblastosis was confirmed. One patient suffered from signs of neuroglycopenia with loss of consciousness and convulsion preoperatively, incorrectly diagnosed as epilepsy.Conclusions. Treatment of patients with gastrointestinal tumors is complex process and most commonly require close cooperation of various proffesional clinicians. Since asymptomatic course of disease and late stage of tumor advancement at diagnosis, technically difficult operations are often essential. Thus, surgeon who perform operations of patients with neuroendocrine tumors should be well experienced in carring out extensive surgical procedures.
The following changes were introduced in 2017 WHO and TnM classifications: 1. a new group of well-differentiated neuroendocrine tumors with the proliferation index of more than 20% and mitotic count of 20 per 10 hpf (NET G3), formerly classified under neuroendocrine carcinomas (NEC G3) 2. the division of poorly differentiated neuroendocrine tumors (PD NET) with the Ki-67 index of more than 20% into two groups in terms of the degree of differentiation and prognosis: NET G3 and NEC. 3. the replacement of MANEC with MINEN within the mixed group 4. the verification of histological grading (G) criteria 5. new TNM staging criteria, based on ENETS guidelines.
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