Gastrinoma (Duodenal and Pancreatic)

Jensen, Robert T.; Niederle, Bruno; Mitry, Emmanuel; Ramage, John; Steinmüller, Thomas; Lewington, Val; Scarpa, Aldo; Sundín, Anders; Perren, Aurel; Gross, David J.; O’Connor, Juan Manuel; Pauwels, Stanislas; Klöppel, Günter

doi:10.1159/000098009

Cited by 260 publications

(539 citation statements)

References 139 publications

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“…In contrast, patients with unresectable disease are candidates for diverse medical treatments that range from use of somatostatin analogs, as most cases express somatostatin receptors, to variably aggressive chemotherapy regimens or peptide receptor radionuclide therapy. [6][7][8][9][10][11] The open question is how we can best identify patients to treat with somatostatin analogs and those who will benefit from more aggressive therapies as first-line treatment.…”

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confidence: 99%

Pancreatic endocrine tumors: improved TNM staging and histopathological grading permit a clinically efficient prognostic stratification of patients

et al. 2010

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Pancreatic endocrine tumors are rare diseases and devising a clinically effective prognostic stratification of patients is a major clinical challenge. This study aimed at assessing whether the tumor-node-metastasis (TNM)-based staging and proliferative activity-based grading recently proposed by the European NeuroEndocrine Tumors Society (ENETS) have clinical value. TNM was applied to 274 patients with histologically diagnosed pancreatic endocrine tumors operated from 1991 to 2005, with last follow-up at December 2007. According to World Health Organization (WHO) classification, 246 were well-differentiated neoplasms (51 benign, 56 uncertain behavior, 139 carcinomas) and 28 poorly differentiated carcinomas. Grading was based on Ki67 immunohistochemistry. Survival analysis not only ascertained the prognostic value of the TNM system but also highlighted that in the absence of nodal and distant metastasis, infiltration and tumor dimensions over 4 cm had prognostic significance. T parameters were then appropriately modified to reflect this weakness. The 5-year survival for modified TNM stages I, II, III and IV were 100, 93, 65 and 35%, respectively. Multivariate analysis identified TNM stages as independent predictors of death, in which stages II, III and IV showed a risk of death of 7, 29 and 58 times higher than stage I tumors (Po0.0001). Ki67-based grading resulted an independent predictor of survival with cut-offs at 5 and 20%. In conclusion, WHO classification assigns clinically significant diagnostic categories to pancreatic endocrine tumors that need prognostic stratification by applying a staging system. The ENETS-TNM provides the best option, but it requires some modifications to be fully functional. The modified TNM described in this study ameliorates the clinical applicability and prediction of outcome of the ENETS-TNM; it (i) assigns a risk of death proportional to the stage at the time of diagnosis, and (ii) allows a clinically based staging of patients, as the T parameters as modified permit their clinical-radiological recognition. Ki67-based grading discerns prognosis of patients with same stage diseases. Modern Pathology (2010) 23, 824-833; doi:10.1038/modpathol.2010 published online 19 March 2010 Keywords: pancreas; tumors; endocrine; staging; TNM; grading; prognosis Pancreatic endocrine tumors are rare and clinically taxing diseases, as their natural history is largely unknown. 1-4 Affected patients seek medical assistance due to symptoms resulting from either hormonal hypersecretion or mass. Thus, pancreatic endocrine tumors are clinically defined as functioning or nonfunctioning depending on the presence of a syndrome related to inappropriate hormone secretion. Such distinction, however, does not provide prognostic information. Pancreatic endocrine tumors are histologically divided into well-

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confidence: 99%

Pancreatic endocrine tumors: improved TNM staging and histopathological grading permit a clinically efficient prognostic stratification of patients

et al. 2010

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“…Hypergastrinaemia results in hypersecretion of gastric acid, and consequently in peptic ulcer disease and gastroesophageal reflux disease with a severe course [19,20]. Gastrinomas are well-differentiated neoplasms NEN G1/G2.…”

Section: Gastrinomamentioning

confidence: 99%

“…Depending on its location and the presence of MEN-1, a gastrinoma demonstrates the following characteristics [12,20,22,23]:…”

Section: Gastrinomamentioning

confidence: 99%

“…-in sporadic ZES gastrinomas may occur occasionally in the liver (< 1%) and liver/biliary ducts [20,25,26,27], which has recently been demonstrated also for patients with MEN-1/ZES [28]. Zollinger-Ellison Syndrome should be suspected in patients with ulcer disease with [20]: -multiple ulcers of the upper part of the gastrointestinal tract, with unusual location, -relapses after treatment, -concomitant severe oesophagitis, -negative H. pylori test results, -complications of the disease (gastrointestinal tract perforation, bleeding), -diarrhoea, -thickening of the gastric folds (present in 92% of ZES patients).…”

Section: Pancreatic Gastrinomasmentioning

confidence: 99%

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Nowotwory neuroendokrynne żołądka i dwunastnicy z uwzględnieniem gastrinoma (zasady postępowania rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

Lipiński¹,

Rydzewska²,

Fołtyn³

et al. 2017

Endokrynologia Polska

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“…Gastrinoma is a neuroendocrine tumor characterized by the secretion of gastrin, which causes hyperchlorhydria, thereby producing the Zollinger-Ellison syndrome (ZES) (1). This syndrome manifests as severe peptic ulcer disease in 70-90% of the cases (2, 3).…”

Section: Introductionmentioning

confidence: 99%

Sporadic duodenal macrogastrinoma: a rare case report

Šimčikas¹,

Poškus²,

Stanaitis³

et al. 2013

AML

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Gastrinomas are rare neuroendocrine tumors characterized by the secretion of gastrin, which causes hyperchlorhydria, thereby producing the Zollinger-Ellison syndrome. In most cases this syndrome manifests as severe peptic ulcer disease. We are presenting an extremely rare clinical case of sporadic duodenal macrogastrinoma. The patient underwent investigation due to six-year history of epigastric pain, heartburn and episodic diarrhea. Endoscopy, endosonoscopy and histologic examination of biopsy specimens indicated the presence of duodenal gastrinoma with no signs of peptic ulcers. Pyloroduodenal segment including 3.5 cm macrogastrinoma was resected. This case is unique as duodenal gastrinomas are usually very small, up to 1 cm. During the follow up period we observed slowly decreasing hypergastrinemia. Somatostatin receptor scintigraphy, CT and upper GI endoscopy were performed to reveal the reasons, though did not find any abnormalities. 8 months of follow-up did not reveal any progression of the disease. The etiology of slowly decreasing hypergastrinemia remains unclear, controversial and is under investigation.

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Gastrinoma (Duodenal and Pancreatic)

Cited by 260 publications

References 139 publications

Pancreatic endocrine tumors: improved TNM staging and histopathological grading permit a clinically efficient prognostic stratification of patients

Pancreatic endocrine tumors: improved TNM staging and histopathological grading permit a clinically efficient prognostic stratification of patients

Nowotwory neuroendokrynne żołądka i dwunastnicy z uwzględnieniem gastrinoma (zasady postępowania rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

Sporadic duodenal macrogastrinoma: a rare case report

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