scholarly journals Neuroendocrine tumors of gastrointestinal tract: The paradigm that lasts

2013 ◽  
Vol 60 (1) ◽  
pp. 39-45
Author(s):  
Milos Bjelovic ◽  
Tamara Babic
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Tumors ◽  
Clinical Presentation ◽  
Treatment Options ◽  
Delayed Diagnosis ◽  
Neuroendocrine Cells ◽  
Biologically Active ◽  
Biological Behaviour ◽  
Adequate Treatment ◽  
Subcellular Level

Historically, the tumors that were morphologically different and clinically less agressive than the more common gastrointestinal adenocarcinomas were clasified under carcinoid tumors. However, the development of molecular biology tehniques revealed the heterogeneity of these tumors on cellular and subcellular level and ther different biological behaviour. Neuroendocrine tumors of gastrointestinal tract originated from neuroendocrine cells scaterred across the gastrointestinal mucosa. As a result these tumors were capable of secreting many different neurotransmiters, which may or may not be biologically active. The incidence of gastrointestinal NETs has been incresing over the last 2 to 3 decades. Patients often presented with vague, nonspecific symptoms which resulted in delayed diagnosis and adequate treatment. In this article, we discuss the nature of gastrointestinal NETs, clinical presentation, treatment options and prognosis.

scholarly journals Ca2+ Channel Toolkit in Neuroendocrine Tumors

2019 ◽  
Vol 110 (1-2) ◽  
pp. 147-154
Author(s):  
Alessandra Fiorio Pla ◽  
Dimitra Gkika
Keyword(s):  
Neuroendocrine Tumors ◽  
Transient Receptor Potential ◽  
Treatment Options ◽  
Unmet Need ◽  
Receptor Potential ◽  
Transient Receptor Potential Channels ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Neoplastic Progression ◽  
Ca2 Channels

Neuroendocrine tumors (NET) constitute a heterogeneous group of malignancies with various clinical presentations and growth rates but a common origin in neuroendocrine cells located all over the body. NET are a relatively low-frequency disease mostly represented by gastroenteropancreatic (GEP) and bronchopulmonary tumors (pNET); on the other hand, an increasing frequency and prevalence have been associated with NET. Despite great efforts in recent years, the management of NET is still a critical unmet need due to the lack of knowledge of the biology of the disease, the lack of adequate biomarkers, late presentation, the relative insensitivity of imaging modalities, and a paucity of predictably effective treatment options. In this context Ca2+ signals, being pivotal molecular devices in sensing and integrating signals from the microenvironment, are emerging to be particularly relevant in cancer, where they mediate interactions between tumor cells and the tumor microenvironment to drive different aspects of neoplastic progression (e.g., cell proliferation and survival, cell invasiveness, and proangiogenetic programs). Indeed, ion channels represent good potential pharmacological targets due to their location on the plasma membrane, where they can be easily accessed by drugs. The present review aims to provide a critical and up-to-date overview of NET development integrating Ca2+ signal involvement. In this perspective, we first give an introduction to NET and Ca2+ channels and then describe the different families of Ca2+ channels implicated in NET, i.e., ionotropic receptors, voltage-dependent Ca2+ channels, and transient receptor potential channels, as well as intracellular Ca2+ channels and their signaling molecules.

Primary Neuroendocrine Tumors of the Ureter: A Short Review

2016 ◽  
Vol 140 (7) ◽  
pp. 714-717 ◽  
Author(s):  
Andres M. Acosta ◽  
Andre Kajdacsy-Balla
Keyword(s):  
Neuroendocrine Tumors ◽  
Multimodal Treatment ◽  
Clinical Presentation ◽  
Treatment Approach ◽  
Short Review ◽  
Small Cell ◽  
Neuroendocrine Cells ◽  
Dense Granules ◽  
Multimodal Treatment Approach ◽  
Ureteral Neoplasms

Neuroendocrine tumors represent 0.05% of urinary tract malignancies, and most originate in the bladder. Their pathogenesis is debated, since ureters are thought to lack neuroendocrine cells. Morphologically, most ureteral neuroendocrine tumors demonstrate the typical small cell neuroendocrine appearance, are immunohistochemically positive for synaptophysin/chromogranin A/CD56, and show electron-dense granules by electron microscopy. Clinical presentation is similar to that of other more common ureteral neoplasms. Prognosis is usually dismal, but a multimodal treatment approach including platinum-based adjuvant or neoadjuvant chemotherapy seems to be beneficial.

Understanding the Management and Treatment of Well-Differentiated Pancreatic Neuroendocrine Tumors: A Clinician’s Guide to a Complex Illness

2020 ◽  
Vol 16 (11) ◽  
pp. 720-728
Author(s):  
Daneng Li ◽  
Adam Rock ◽  
Jonathan Kessler ◽  
Richard Ballena ◽  
Shadman Hyder ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Treatment Options ◽  
Arterial Embolization ◽  
Standard Of Care ◽  
Neuroendocrine Cells ◽  
Pancreatic Neuroendocrine Tumors ◽  
Current Standard ◽  
Systemic Treatments ◽  
Individualized Approach ◽  
Diagnostic Modalities

Pancreatic neuroendocrine tumors (PanNETs) are rare neoplasms that arise in the neuroendocrine cells of the pancreas. Although their clinical presentations differ depending on cell type, most are indolent, whereas others cause noteworthy hormone-related symptoms. The increasing incidence of PanNETs, attributed to improved diagnostic modalities, demonstrates advances in current standard of care. However, given the heterogeneity of these tumors, treatment decisions can become complex and an individualized approach is often required. Surgical intervention has remained the mainstay for localized tumors, whereas systemic therapies remain viable options for patients with unresectable or metastatic disease. Liver-directed therapies such as radiofrequency ablation and hepatic arterial embolization have also become available adjunct therapies for patients with liver-predominant metastases. Despite the increase in the armamentarium of treatment options for patients with PanNETs, data regarding the ideal sequence of treatment, especially systemic treatments, are currently lacking. Ongoing clinical trials are aimed at addressing this knowledge gap in addition to developing the next generation of novel therapeutics.

scholarly journals Endoscopic diagnosis and treatment of neuroendocrine tumors of the digestive system

Open Medicine ◽  
2016 ◽  
Vol 11 (1) ◽  
pp. 369-373
Author(s):  
Luigi Sivero ◽  
Donato Alessandro Telesca ◽  
Simona Ruggiero ◽  
Teresa Russo ◽  
Maurizio Amato ◽  
...  
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Tumors ◽  
Digestive System ◽  
Neuroendocrine Cells ◽  
Surgical Endoscopy ◽  
International Literature ◽  
Potential Treatment ◽  
Digestive Endoscopy ◽  
Endoscopic Techniques

AbstractThe authors evaluated the role of endoscopic techniques in the diagnosis and in the potential treatment of neuroendocrine tumors (NET) localized in the gastro-entero-pancreatic system, on the basis of their experience and of the international literature. NET are rare tumors that arise from neuroendocrine cells of the gastrointestinal tract and pancreas. It is a possibility that both the digestive endoscopy and EUS play an important role in the diagnosis, staging and surveillance of this disease. In some cases, especially in the early stages, surgical endoscopy allows the treatment of such tumors.

scholarly journals AAVP displaying octreotide for ligand-directed therapeutic transgene delivery in neuroendocrine tumors of the pancreas

2016 ◽  
Vol 113 (9) ◽  
pp. 2466-2471 ◽  
Author(s):  
Tracey L. Smith ◽  
Ziqiang Yuan ◽  
Marina Cardó-Vila ◽  
Carmen Sanchez Claros ◽  
Asha Adem ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Treatment Options ◽  
Somatostatin Receptors ◽  
Somatostatin Analogs ◽  
Suppressor Gene ◽  
Biologically Active ◽  
Cell Internalization ◽  
Pancreatic Net

Patients with inoperable or unresectable pancreatic neuroendocrine tumors (NETs) have limited treatment options. These rare human tumors often express somatostatin receptors (SSTRs) and thus are clinically responsive to certain relatively stable somatostatin analogs, such as octreotide. Unfortunately, however, this tumor response is generally short-lived. Here we designed a hybrid adeno-associated virus and phage (AAVP) vector displaying biologically active octreotide on the viral surface for ligand-directed delivery, cell internalization, and transduction of an apoptosis-promoting tumor necrosis factor (TNF) transgene specifically to NETs. These functional attributes of AAVP-TNF particles displaying the octreotide peptide motif (termed Oct-AAVP-TNF) were confirmed in vitro, in SSTR type 2-expressing NET cells, and in vivo using cohorts of pancreatic NET-bearing Men1 tumor-suppressor gene KO mice, a transgenic model of functioning (i.e., insulin-secreting) tumors that genetically and clinically recapitulates the human disease. Finally, preclinical imaging and therapeutic experiments with pancreatic NET-bearing mice demonstrated that Oct-AAVP-TNF lowered tumor metabolism and insulin secretion, reduced tumor size, and improved mouse survival. Taken together, these proof-of-concept results establish Oct-AAVP-TNF as a strong therapeutic candidate for patients with NETs of the pancreas. More broadly, the demonstration that a known, short, biologically active motif can direct tumor targeting and receptor-mediated internalization of AAVP particles may streamline the potential utility of myriad other short peptide motifs and provide a blueprint for therapeutic applications in a variety of cancers and perhaps many nonmalignant diseases as well.

scholarly journals Carcinoid syndrome in neuroendocrine tumors - not easy to recognize

2013 ◽  
Vol 60 (3) ◽  
pp. 13-16
Author(s):  
Milos Bjelovic
Keyword(s):  
Neuroendocrine Tumors ◽  
Carcinoid Syndrome ◽  
Clinical Presentation ◽  
Malignant Tumors ◽  
Treatment Options ◽  
Medical Doctor ◽  
Clinical Syndromes ◽  
Potentially Malignant ◽  
The Right ◽  
Symptoms And Signs

Neuroendocrine tumors (NET) are solid potentially malignant tumors originated from the diffuse neuroendocrine system. They could origin in many organs, with highest prevalence in lungs, small intestine and rectum. Characteristics of NET are slow growth, non-specific clinical presentation causing diagnostic problems. Thus, in majority of patients diagnosis is established in the metastatic phase of the disease. Hopefully, there are new and very potent treatment options capable to successfully control the disease. Clinical presentation cause local tumor growth or para-neoplastic syndrome. Secretory active tumors produce peptides or hormones causing different clinical syndromes. In most cases NET cause carcinoid syndrome. It is often misinterpreted, because similar symptoms are present in more prevalent disorders. Symptoms are not specific and include flashing, diarrhea, abdominal pain, right heart disease, bronchoconstriction... and to establish the right diagnosis medical doctor have to think about NET as a possibility. Thus, it is very important to recognize symptoms and signs of the carcinoid syndrome, and distinguish them from other gastrointestinal disorders. Early diagnosis and treatment have significant impact in control of the disease, and overall treatment results.

scholarly journals On the history and some characteristics of neuroendocrine tumors of the gastrointestinal tract. Review. Part 1

2021 ◽  
pp. 60-72
Author(s):  
S. Rybakov
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Tumors ◽  
Endocrine System ◽  
Secretory Activity ◽  
Clinical Syndrome ◽  
Biologically Active ◽  
Clinical Aspects ◽  
Clinical Syndromes ◽  
Diffuse Endocrine System ◽  
Active Substances

The literature review is devoted to the analysis of numerous studies that have formed the basis for creating ideas about the diffuse endocrine system. In addition to the main endocrine glands, also there are cellular accumulations and individual cells that produce various hormones and biologically active substances. It is established that, in particular, in the organs of the gastrointestinal tract, starting from the esophagus and to the terminal parts of the large intestine, there are several types of endocrine cells with these functions. Most of them are in the pancreas, antrum of the stomach, duodenum, the initial part of the intestine, in the liver. Excessive secretory activity of these cells can cause the development of pathological clinical syndromes, characterized by the effect of the corresponding hormones. The morphological substrate of such syndromes may be tumors or diffuse hyperplasia of the corresponding cells. The author considers the pancreas as an integral part of the diffuse endocrine system, as specific endocrine functions in it perform numerous accumulations (islets of Langerhans) or individual cells. The review of the numerous neuroendocrine tumors of the gastrointestinal tract examines in detail the characteristics of some of them in historical and clinical aspects, in particular insulinoma as the most common and well-studied tumor. Glucagonoma is also considered as a neuroendocrine tumor derived from α-cells of the pancreas, is a kind of insulinoma antagonist, as it secretes an excess of the hormone glucagon, causing the development of hyperglycemic clinical syndrome in contrast to hypoglycemic, caused by insulin. It is a rare tumor and its consideration after insulinoma can be explained by the antagonistic nature of the action of hormones produced by these tumors. In the group of neuroendocrine tumors of the pancreas, glucagonoma follows the frequency of insulinoma and gastrinoma.

scholarly journals On the history and some characteristics of neuroendocrine tumors of the gastrointestinal tract. Neuroendocrine tumors of the pancreas. Part 2

2021 ◽  
pp. 70-81
Author(s):  
S. I. Rybakov
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Tumors ◽  
Endocrine System ◽  
Secretory Activity ◽  
Clinical Syndrome ◽  
Biologically Active ◽  
Clinical Aspects ◽  
Clinical Syndromes ◽  
Diffuse Endocrine System ◽  
Active Substances

The literature review presents the analysis of numerous studies that have formed the basis for creating ideas about the diffuse endocrine system,namely existence of the cellular accumulations and individual cells that produce various hormones and biologically active substances in addition to the main endocrine glands. Several types of endocrine cells with these functionshave been established, in particular, in the organs of the gastrointestinal tract, starting from the esophagus and up to the terminal parts of the large intestine. Most of them are contained in the pancreas, antrum of the stomach, duodenum, the initial part of the intestine, in the liver. Excessive secretory activity of these cells can cause the development of pathological clinical syndromes, characterized by the effects of the corresponding hormones. The tumors or diffuse hyperplasia of the corresponding cellsmay present morphological substrate of such syndromes. The author considers the pancreas as an integral part of the diffuse endocrine system, as specific endocrine functions in it perform numerous accumulations (islets of Langerhans) or individual cells. The review of the numerous neuroendocrine tumors of the gastrointestinal tract examines in detail the characteristics of some of them in historical and clinical aspects, in particular Zollinger—Ellison syndrome as the most common and well-studied tumor. Glucagonoma is also considered as a neuroendocrine tumorderived from α-cells of the pancreas, is a kind of insulinoma antagonist, as it secretes an excess of the hormone glucagon, causing the development of hyperglycemic clinical syndrome in contrast to hypoglycemic, caused by insulin. It is a rare tumor and its consideration after insulinoma can be explained by the antagonistic nature of the action of hormones produced by these tumors. In the group of neuroendocrine tumors of the pancreas, glucagonoma follows the frequency of insulinoma and gastrinoma.

scholarly journals EPIDEMIOLOGIChESKIE ASPEKTY PERVIChNOGOGIPERPARATIREOZA V ROSSII

2010 ◽  
Vol 13 (3) ◽  
pp. 13-18
Author(s):  
L. Ya. Rozhinskaya ◽  
L. G. Rostomyan ◽  
N. G. Mokrysheva ◽  
S. S. Mirnaya ◽  
N. O. Kirdyankina
Keyword(s):  
Clinical Presentation ◽  
Treatment Options ◽  
Delayed Diagnosis ◽  
Clinical Profile ◽  
Ulcer Disease ◽  
Predominant Form ◽  
And Gender ◽  
Calcium Measurement ◽  
Variable Clinical Presentation ◽  
Regions Of Russia

Primary hyperparathyroidism (PHPT) is the third most frequent endocrine disorder and has a variable clinical presentation. Asymptomatic PHPT became the predominant form of the disease with increase of its incidence after the introduction of automated serum calcium measurement in North America and Europe. Data from Russia is lacking. Aim: To present the clinical profile of PHPT in Russia. Materials and Methods: This retrospective study was conducted at endocrinology centers in 8 regions of Russia. We analyzed the clinical presentation, and treatment options in patients with confirmed PHPT (1995-2010). Results: 738 patients (F: M-8:1) with age ranging from 13 to 83,4 years (mean 54,3) were analyzed. 54% was from Moscow (n=397), 11% - from Moscow region (n=79) and 35% - from 53 regions of Russia (n=262). Symptomatic PHPT was the most common form (74%) and was revealed with osteoporosis in 56%, nephrolithiasis - in 45% and ulcer disease - in 18%. Our data showed an increase in the incidence of PHPT (especially mild PHPT) after 2005 compared with earlier period. 64,9% of patients were treated surgically, 17 patients undergone repeated parathyroid surgery. 28,3% received bisphosphonates, calcitonin and/or cinacalcet . 11,5% was observed without treatment. Conclusions: This data analyzes some causes of delayed diagnosis of PHPT in Russia, characterized age and gender distribution of patients with PHPT and demonstrates the changes in clinical profile of disease from 1995 to 2010. PHPT still remains symptomatic disorder in our country most frequently with skeletal and renal manifestations.

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