Update of the diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours) [Aktualizacja zaleceń ogólnych dotyczących postępowania diagnostyczno-terapeutycznego w nowotworach neuroendokrynnych układu pokarmowego (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)]
Beata Kos‐KudłaWanda FołtynAnna MalczewskaTomasz BednarczukMarek BolanowskiMałgorzata BorowskaEwa ChmielikJarosław B. ĆwikłaIwona GisterekDaria Handkiewicz-JunakAlicja Hubalewska‐DydejczykBarbara JarząbMichał JarząbRoman JunikDariusz KajdaniukGrzegorz KamińskiAgnieszka Kolasińska-ĆwikłaAldona KowalskaLeszek KrólickiMaciej KrzakowskiJolanta KunikowskaKatarzyna KuśnierzAndrzej LewińskiŁukasz LiszkaMagdalena Londzin-OlesikBogdan MarekAnna Nasierowska‐GuttmejerEwa Nowakowska-DuławaMarianne PavelJoanna Pilch-KowalczykJarosław RegułaVioletta RosiekMarek RuchałaGrażyna RydzewskaLucyna SiemińśkaAnna Sowa‐StaszczakTeresa StarzyńskaZoran StojčevJanusz StrzelczykMichał StudniarekAnhelli SyreniczMarek SzczepkowskiEwa WachułaWojciech ZajęckiAnna ZemczakWojciech ZgliczyńskiKrzysztof Zieniewicz
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Abstract:
Continuous progress in the diagnostics and treatment of neuroendocrine neoplasms (NENs), the emerging results of new clinical trials, and the new guidelines issued by medical societies have prompted experts from the Polish Network of Neuroendocrine Tumours to update the 2017 recommendations regarding the management of neuroendocrine neoplasms. This article presents the general recommendations for the management of NENs, resulting from the findings of the experts participating in the Fourth Round Table Conference, entitled "Polish Guidelines for the Diagnostics and Treatment of Neuroendocrine Neoplasms of the gastrointestinal tract, Żelechów, June 2021". Drawing from the extensive experience of centres treating these cancers, we hope that we have managed to formulate the optimal method of treating patients with NENs, applying the latest reports and achievements in the field of medicine, which can be effectively implemented in our country. The respective parts of this work present the approach to the management of: NENs of the stomach and duodenum (including gastrinoma), pancreas, small intestine, and appendix, as well as large intestine.Keywords:
Gastrinoma
Neuroendocrine tumour
Gastrinoma
Glucagonoma
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Gastrinoma
MEN1
Zollinger-Ellison syndrome
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This paper reviews data on benign and malignant neuroendocrine tumours of pancreas and gastrointestinal tract including insulinoma, glucagonoma, somatostatinoma, gastrinoma, carcinoid syndrome, and multiple endocrine neoplasms. Their origin, prevalence, clinical manifestations, laboratory and instrumental diagnosis, surgical and medicamentous treatment are discussed.
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Glucagonoma
Zollinger-Ellison syndrome
Carcinoid tumour
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We present our surgical experiences with functioning neuroendocrine neoplasms of the pancreas to define its natural history, and to suggest its proper management.From June 1990 to June 2005, patients with diagnosis of functioning neuroendocrine (islet cell) neoplasms of the pancreas were retrospectively reviewed.Fourteen patients (5 men and 9 women) with a median age of 49 years (range, 12 -68 years) were identified.Twelve patients (86%) had insulinoma, two (14%) had gastrinoma.One (7%) with pancreatic insulinoma was multiple endocrine neoplasia type 1. Intraoperative ultrasound scan (sensitivity, 83%) was the most powerful modality for tumor localization.Fifteen neoplasms with median tumor size 1 cm (range 0-3 cm) were resected.Four insulinomas (26.7%) were located in the head of the pancreas and 5 (36%), in the tail.Another 5 (36%) insulinomas and 1 (7%) gastrinoma were located around the neck area near the SMV or PV.Eleven patients (79%) underwent enucleation, and 2 patients (14%), distal pancreatectomy with splenectomy.100% of patients with functioning neuroendocrine neoplasms of the pancreas have survived.The overall disease free 10-year survival was found to be about 81%.Exact localization of tumor by intraoperative ultrasound and surgical removal are promising for good prognosis.
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Somatostatin-producing neuroendocrine tumors (SOM-NETs) of the duodenum and pancreas appear to be heterogeneous. To determine their clinicopathological profiles, respective data were analyzed on a series of 82 duodenal and 541 pancreatic NETs. In addition, the clinical records of 821 patients with duodenal or pancreatic NETs were reviewed for evidence of a somatostatinoma syndrome. Predominant or exclusive expression of somatostatin was found in 21 (26%) duodenal and 21 (4%) pancreatic NETs. They were classified as sporadic (n=31) or neurofibromatosis type 1 (NF1)-associated duodenal NETs (n=3), gangliocytic paragangliomas (GCPGs; n=6), or poorly differentiated neuroendocrine carcinomas (pdNECs; n=2). In addition, five duodenal and four pancreatic SOM-NETs were found in five patients with multiple endocrine neoplasia type 1 (MEN1). Metastases occurred in 13 (43%) patients with sporadic or NF1-associated SOM-NETs, but in none of the duodenal or pancreatic MEN1-associated SOM-NETs or GCPGs. Sporadic advanced (stage IV) SOM-NETs were more commonly detected in the pancreas than in the duodenum. None of the patients (including the 821 patients for whom only the clinical records were reviewed) fulfilled the criteria of a somatostatinoma syndrome. Our data show that somatostatin expression is not only seen in sporadic NETs but may also occur in GCPGs, pdNECs, and hereditary NETs. Surgical treatment is effective in most duodenal and many pancreatic SOM-NETs. MEN1-associated SOM-NETs and GCPGs follow a benign course, while somatostatin-producing pdNECs are aggressive neoplasms. The occurrence of the so-called somatostatinoma syndrome appears to be extremely uncommon.
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Gastrinoma
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Zollinger and Ellison first called attention to instances of recurrent peptic ulceration associated with islet cell tumors of the pancreas.1These were characterized by hyperacidity and hypersecretion of acid gastric juice and not controlled by vagotomy and extensive gastric resection. Previous experimental studies have also demonstrated an interrelationship between the pancreas and gastric secretion. Dragstedt reported in 1942 that approximately 100% of dogs with external total pancreatic fistulae developed duodenal ulcer, whereas dogs in whom the pancreatic ducts had been ligated had only a 29% incidence of duodenal ulcer. He further found that dogs upon whom a pancreatectomy had been performed developed ulcer in only 1%-3%.2In all three of these preparations the external secretion of the pancreas is absent from the duodenum. The internal drainage of bile and pancreatic juice to the terminal ileum in the Mann-Williamson dog also results in a high incidence of peptic ulceration.
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Multiple endocrine neoplasia type 1 (MEN-1) is a rare autosomal-dominant disease. It is associated with a broad range of endocrine tumours, most frequently arising in the parathyroid glands, the pituitary and the pancreas. Most neuroendocrine tumours will be diagnosed in the pancreas as non-functioning neuroendocrine tumours or insulinomas. Forty-two percent of the patients will develop a gastrin-secreting neuroendocrine tumour, a gastrinoma. Gastrinomas in MEN-1 tend to be small, multiple and preferentially located in the duodenum. This paper will focus on the specific characteristics of gastrinomas in the setting of MEN-1 compared to sporadic gastrinomas. The developments in understanding the tumorigenesis of these tumours and the consequences for diagnosis and therapy will be discussed.
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