scholarly journals Neuroendocrine Tumors — Laboratory Diagnosis

2010 ◽  
Vol 29 (4) ◽  
pp. 254-264 ◽  
Author(s):  
Anna Tzontcheva
Keyword(s):  
Gastrointestinal Tract ◽  
Tumor Markers ◽  
Neuroendocrine Tumors ◽  
Chromogranin A ◽  
Endocrine Cells ◽  
Neuron Specific Enolase ◽  
Laboratory Diagnosis ◽  
Pancreatic Tumors ◽  
Endocrine Pancreatic Tumors ◽  
Well Differentiated

Neuroendocrine Tumors — Laboratory DiagnosisNeuroendocrine tumors (NETs) are a heterogeneous group of neoplasms originating from endocrine cells, which are characterized by the presence of secretory granules as well as the ability to produce biogenic amines and polypeptide hormones. These tumors originate from endocrine glands such as the adrenal medulla, the pituitary, and the parathyroids, as well as endocrine islets within the thyroid or the pancreas, and dispersed endocrine cells in the respiratory and gastrointestinal tract. The clinical behavior of NETs is extremely variable; they may be functioning or not functioning, ranging from very slow-growing tumors (well-differentiated NETs), which are the majority, to highly aggressive and very malignant tumors (poorly differentiated NETs). Classically, NETs of the gastrointestinal tract are classified into 2 main groups: (1) carcinoids and (2) endocrine pancreatic tumors (EPTs). Most neuroendocrine tumors produce and secrete a multitude of peptide hormones and amines. Some of these substances cause a specific clinical syndrome: carcinoid, Zollinger-Ellison, hyperglycemic, glucagonoma and WDHA syndrome. Specific markers for these syndromes are basal and/or stimulated levels of urinary 5-HIAA, serum or plasma gastrin, insulin, glucagon and vasoactive intestinal polypeptide, respectively. Some carcinoid tumors and about one third of endocrine pancreatic tumors do not present any clinical symptoms and are called ‘nonfunctioning’ tumors. Therefore, general tumor markers such as chromogranin A, pancreatic polypeptide, serum neuron-specific enolase and subunits of glycoprotein hormones have been used for screening purposes in patients without distinct clinical hormone-related symptoms. Among these general tumor markers chromogranin A, although its precise function is not yet established, has been shown to be a very sensitive and specific serum marker for various types of neuroendocrine tumors. This is because it may also be elevated in many cases of less well-differentiated tumors of neuroendocrine origin that do not secrete known hormones. At the moment, chromogranin A is considered the best general neuroendocrine serum or plasma marker available both for diagnosis and therapeutic evaluation, and is increased in 50-100% of patients with various neuroendocrine tumors. Chromogranin A serum or plasma levels reflect tumor load, and it may be an independent marker of prognosis in patients with midgut carcinoids.

Current aspects of diagnosis and treatment of patients with neuroendocrine tumors

2021 ◽  
Vol 23 (3) ◽  
pp. 83-92
Author(s):  
Maxim V. Lysanyuk ◽  
Pavel N. Romashchenko ◽  
Nicolay A. Maistrenko
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Tumors ◽  
Survival Rates ◽  
Neuron Specific Enolase ◽  
Treatment Method ◽  
Pancreatic Tumors ◽  
Arterial Phase ◽  
Computed Tomographic ◽  
Positron Emission ◽  
Degree Of Malignancy

The article presents modern possibilities and existing problematic aspects of the choice of therapeutic and diagnostic tactics in patients with neuroendocrine tumors of the gastrointestinal tract and pancreas are presented. The asymptomatic course of neuroendocrine tumors of the gastrointestinal tract and pancreas was established in 18.5% and 24.6% of cases, respectively. Carcinoid syndrome was detected in 12.9%. The sensitivity rates of chromogranin A and neuron-specific enolase in the diagnosis of tumors were 54% and 13%, respectively. The levels of cancer-embryonic antigen in G-1/G-2 and G-3 tumors were 5 ng/ml and 8.9 ng/ml, respectively (p 0.001). A pathognomonic sign of neuroendocrine tumors of the small intestine is a mesentery tumor conglomerate, and the sensitivity rates of computed tomography and positron emission tomography with 68Ga to detect this sign were 92.3% and 92.9%, respectively (p 0.05). The computed tomographic density of neuroendocrine pancreatic tumors G-1/G-2 in the arterial phase was 112.1 40.2 HU and that of G-3 tumors was 54.0 10.4 HU (p = 0.025). Surgical treatment was performed in 259 (79.7%) patients. Postoperative complications that developed in localized and locally distributed neuroendocrine tumors of the gastrointestinal tract and of the pancreas were found in 3.5% and 8.8%, and in 58.1% and 40% of the cases, respectively, and those of generalized tumors were noted in 20%. The tumor-specific 5-year survival rates of patients with localized neuroendocrine tumors of the gastrointestinal tract and pancreas were 92.5% and 94.4%, those with locally distributed tumors had 66.8% and 77.8%, and those with generalized tumors had 51.8% and 47.1%, respectively. In patients with generalized tumors, the 5-year survival rates after cytoreduction and removal of the primary tumor were 88.2% and 64.6%, respectively (p = 0.097), and the rate after drug therapy was 28.8% (p 0.001). The prognosis of the 5-year survival of patients is determined by the degree of malignancy and tumor localization, treatment method, and patient age. In general, neuroendocrine tumors are a heterogeneous group of neoplasms that require a multidisciplinary approach to diagnosis and choice of therapeutic strategies.

IL-2 −330 T/G SNP and serum values—potential new tumor markers in neuroendocrine tumors of the gastrointestinal tract and pancreas (GEP-NETs)

2010 ◽  
Vol 88 (4) ◽  
pp. 423-429 ◽  
Author(s):  
Maja Cigrovski Berković ◽  
Mladen Jokić ◽  
Jasminka Marout ◽  
Senka Radošević ◽  
Vanja Zjačić-Rotkvić ◽  
...  
Keyword(s):  
Gastrointestinal Tract ◽  
Tumor Markers ◽  
Neuroendocrine Tumors

Chromogranin A, neuron specific enolase, carcinoembryonic antigen, and hydroxyindole acetic acid evaluation in patients with neuroendocrine tumors

Cancer ◽  
1999 ◽  
Vol 86 (5) ◽  
pp. 858-865 ◽  
Author(s):  
Emilio Bajetta ◽  
Leonardo Ferrari ◽  
Antonia Martinetti ◽  
Luigi Celio ◽  
Giuseppe Procopio ◽  
...  
Keyword(s):  
Acetic Acid ◽  
Carcinoembryonic Antigen ◽  
Neuroendocrine Tumors ◽  
Chromogranin A ◽  
Neuron Specific Enolase

scholarly journals Chromogranin A: From Laboratory to Clinical Aspects of Patients with Neuroendocrine Tumors

2018 ◽  
Vol 2018 ◽  
pp. 1-12 ◽  
Author(s):  
Paola Di Giacinto ◽  
Francesca Rota ◽  
Laura Rizza ◽  
Davide Campana ◽  
Andrea Isidori ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Phase Ii ◽  
Chromogranin A ◽  
Carcinoid Syndrome ◽  
Phase Ii Trial ◽  
Randomized Study ◽  
Clinical Aspects ◽  
Clinical Activity ◽  
Octreotide Lar ◽  
Well Differentiated

Background. Neuroendocrine tumors (NETs) are characterized by having behavior and prognosis that depend upon tumor histology, primary site, staging, and proliferative index. The symptoms associated with carcinoid syndrome and vasoactive intestinal peptide tumors are treated with octreotide acetate. The PROMID trial assesses the effect of octreotide LAR on the tumor growth in patients with well-differentiated metastatic midgut NETs. The CLARINET trial evaluates the effects of lanreotide in patients with nonfunctional, well-, or moderately differentiated metastatic enteropancreatic NETs. Everolimus has been approved for the treatment of advanced pancreatic NETs (pNETs) based on positive PFS effects, obtained in the treated group. Sunitinib is approved for the treatment of patients with progressive gastrointestinal stromal tumor or intolerance to imatinib, because a randomized study demonstrated that it improves PFS and overall survival in patients with advanced well-differentiated pNETs. In a phase II trial, pasireotide shows efficacy and tolerability in the treatment of patients with advanced NETs, whose symptoms of carcinoid syndrome were resistant to octreotide LAR. An open-label, phase II trial assesses the clinical activity of long-acting repeatable pasireotide in treatment-naive patients with metastatic grade 1 or 2 NETs. Even if the growth of the neoplasm was significantly inhibited, it is still unclear whether its antiproliferative action is greater than that of octreotide and lanreotide. Because new therapeutic options are needed to counter the natural behavior of neuroendocrine tumors, it would also be useful to have a biochemical marker that can be addressed better in the management of these patients. Chromogranin A is currently the most useful biomarker to establish diagnosis and has some utility in predicting disease recurrence, outcome, and efficacy of therapy.

scholarly journals Baseline plasma chromogranin A levels in patients with well-differentiated neuroendocrine tumors of the pancreas: A potential predictor of postoperative recurrence

Pancreatology ◽  
2017 ◽  
Vol 17 (2) ◽  
pp. 291-294 ◽  
Author(s):  
Yoshihide Nanno ◽  
Hirochika Toyama ◽  
Ippei Matsumoto ◽  
Kyoko Otani ◽  
Sadaki Asari ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Chromogranin A ◽  
Postoperative Recurrence ◽  
Potential Predictor ◽  
Well Differentiated

Chromogranin: A newly recognized marker for endocrine cells of the human gastrointestinal tract

1985 ◽  
Vol 89 (6) ◽  
pp. 1366-1373 ◽  
Author(s):  
P. Facer ◽  
A.E. Bishop ◽  
R.V. Lloyd ◽  
B.S. Wilson ◽  
R.J. Hennessy ◽  
...  
Keyword(s):  
Gastrointestinal Tract ◽  
Chromogranin A ◽  
Endocrine Cells ◽  
Human Gastrointestinal Tract

Everolimus in combination with octreotide LAR as the first-line treatment for advanced neuroendocrine tumors: A phase II trial of the I.T.M.O. (Italian Trials in Medical Oncology) group.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 4136-4136 ◽  
Author(s):  
Emilio Bajetta ◽  
Laura Catena ◽  
Nicola Fazio ◽  
Sara Pusceddu ◽  
Pamela Biondani ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Phase Ii ◽  
Medical Oncology ◽  
Objective Response ◽  
Complete Response ◽  
Multicenter Trial ◽  
Pancreatic Tumors ◽  
Primary Tumor Site ◽  
Octreotide Lar ◽  
Well Differentiated

4136^ Background: Everolimus has shown antitumor activity in patients (pts) with advanced pancreatic neuroendocrine tumors (NETs). We aimed to assess efficacy and safety of everolimus in combination with octreotide long-acting repeatable (LAR) in patients with well differentiated NETs of gastroenteropancreatic and of lung origin. Methods: We performed a phase II, multicenter trial using a Simon two-stage minmax design. Pts with advanced well differentiated, previously untreated NETs of the gastroenteropancreatic tract and of the lung received octreotide LAR 30 mg every 28 days in conjunction with everolimus 10 mg per day continuously. The primary endpoint was objective response rate (ORR). Results: A total of 50 pts (58% males) were enrolled. The median age was 60.5 years (range 25-76). Primary tumor site was pancreas in 14 (28%), unknown in 14 (28%), lung in 11 (22%), ileum in 9 (18%) and jejunum and duodenum in 2 (4%) of pts. 13 (26%) pts had carcinoid syndrome. The ORR, calculated on the ITT population, was 20.0% (95% CI 8.9-31.1): 2 patients (4%) had a complete response (CR), 8 (16%) a partial response (PR). Thirty-six patients (72%) achieved stable disease (SD). All CR and all PR as well as 91.7% of SD had a duration ≥ 6 months. Clinical benefit (CR+PR+SD) was 92%. At a median follow-up of 277 days, the median time to progression (TTP) was 16.3 months (95% CI 10.7-20.1). Overall survival could not be assessed. Treatment-related adverse events (AEs) were mostly of grade 1 or 2; the only grade 4 AE was mucositis in 1 patient, while grade 3 AEs included skin rash in 1 case, stomatitis in 4 cases (8%) and diarrhea in 11 cases (22%). Conclusions: Everolimus in combination with octreotide LAR has shown to be active and well tolerated in advanced NETs and, in this study, not only in primary pancreatic tumors. Compared to other clinical trials with everolimus in NETs, the observed ORR in this study was higher. Aknowledgements: The Authors thank the Italian Trials in Medical Oncology (I.T.M.O.) group and Novartis Pharma for the support provided. Clinical trial information: 2008-007153-13.

Somatostatin receptor scintigraphy and chromogranin A assay in staging and follow-up of patients with well-differentiated neuroendocrine tumors

2011 ◽  
Vol 32 (8) ◽  
pp. 731-737 ◽  
Author(s):  
Marcel P.M. Stokkel ◽  
Daphne D. Rietbergen ◽  
Catharina M. Korse ◽  
Babs G. Taal
Keyword(s):  
Neuroendocrine Tumors ◽  
Chromogranin A ◽  
Somatostatin Receptor ◽  
Somatostatin Receptor Scintigraphy ◽  
Receptor Scintigraphy ◽  
Well Differentiated

scholarly journals Significance of chromogranin A and synaptophysin in pancreatic neuroendocrine tumors

2020 ◽  
Author(s):  
Tatsuo Tomita
Keyword(s):  
Neuroendocrine Tumors ◽  
Chromogranin A ◽  
Endocrine Cells ◽  
Elevated Serum ◽  
Metastatic Tumor ◽  
Neuroendocrine Cells ◽  
Pancreatic Neuroendocrine Tumors ◽  
Pancreatic Hormones ◽  
Β Cell ◽  
Tumor Mass

The two most commonly used immunohistochemical markers for neuroendocrine cells and their tumors are chromogranin A (CgA) and synaptophysin (SPY). CgA is a marker for neuroendocrine secretory granules of four pancreatic hormones and gastrin while SPY is a marker for synaptic vesicles in neuroendocrine cells, which release classic neurotransmitters such as acetylcholine and others. CgA is involved in synthesis and secretion of peptide hormones through exocytosis while function of SPY is elusive. Thirty-five pancreatic neuroendocrine tumors (Pan-NETs) were studied, consisting of 14 insulinomas, 8 gastrinomas, 2 glucagonomas, 6 pancreatic polypeptidomas and 5 non-functioning tumors, and were immunostained for four pancreatic hormones, gastrin, CgA and SPY. Majority of Pan-NETs were less immunostained for the endocrine hormones and CgA than the normal pancreatic endocrine cells. CgA immunostaining mostly correlates with each hormone staining in non-β-cell tumors while SPY immunostaining recognizes endocrine cells diffusely in the cytoplasm. CgA immunostaining is less in insulinomas than in non-β-cell tumors, and CgA immunostaining may distinguish CgA-weaker insulinomas from CgA-stronger non-β-cell tumors. CgA immunostaining may be used as an independent marker for biological aggressiveness in non-β-cell Pan-NETs. The serum CgA levels are higher in subjects harboring non-β-cell tumors than those harboring insulinomas, and the serum CgA elevate in parallel to the increasing metastatic tumor mass. Thus, CgA positive immunostaining in Pan-NETs correlate with the elevated serum levels of CgA for diagnosing CgA-positive non-β-cell Pan-NETs and the increasing serum CgA levels indicate increasing metastatic tumor mass.

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