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Reza Kianmanesh - One of the best experts on this subject based on the ideXlab platform.
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enets consensus guidelines for the management of patients with digestive neuroEndocrine neoplasms functional pancreatic Endocrine Tumor Syndromes
Neuroendocrinology, 2012Co-Authors: Guillaume Cadiot, Wouter W De Herder, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Reza KianmaneshAbstract:Pancreatic Endocrine Tumors (p-NETs) include both pancreatic neuroEndocrine Tumors (p-NETs) associated with a functional syndrome (functional p-NETs) or those associated with no distinct clinical syndrome (non-functional p-NETs) [1,2,3,4]. Non-functional p-NETs frequently secrete pancreatic polypeptide, chromogranin A, neuron-specific enolase, human chorionic gonadotrophin subunits, calcitonin, neurotensin or other peptides, but they do not usually produce specific symptoms and thus are considered clinically to be non-functional Tumors [2,3,5,6,7]. Only the functional p-NETs will be considered in this section. The two most common functional p-NETs (gastrinomas, insulinomas) are considered separately, whereas the other well-described and possible rare functional p-NETs are considered together as a group called rare functional p-NETs (RFTs) (table (table1)1) [1,2,3,4]. Table 1 Functional pancreatic Endocrine Tumor (PET) Syndromes Gastrinomas are neuroEndocrine neoplasms, usually located in the duodenum or pancreas, that secrete gastrin and cause a clinical syndrome known as Zollinger-Ellison syndrome (ZES). ZES is characterized by gastric acid hypersecretion resulting in severe peptic disease (peptic ulcer disease (PUD), gastroesophageal reflux disease (GERD)) [8,9,10]. In this section, ZES due to both duodenal and pancreatic gastrinomas will be covered together because clinically they are similar [8,10]. Specific points related to gastrinomas associated with the genetic syndrome of Multiple Endocrine Neoplasia type 1 (MEN1) (25% of cases) will also be mentioned [11,12]. Insulinomas are neuroEndocrine neoplasms located in the pancreas that secrete insulin, which causes a distinct syndrome characterized by symptoms due to hypoglycemia [2,13,14,15]. The symptoms are typically associated with fasting and the majority of patients have symptoms secondary to hypoglycemic central nervous system (CNS) effects (headaches, confusion, visual disturbances, etc.) or due to catecholamine excess secondary to hypoglycemia (sweating, tremor, palpitations, etc.) [2,3,13,14,15]. RFTs can occur in the pancreas or in other locations (VIPomas, somatostatinomas, GRHomas, ACTHomas, p-NETs causing carcinoid syndrome or hypercalcemia (PTHrp-omas)) (table (table1)1) [1,2,3,4,5,7]. Each of the established RFT Syndromes is associated with a distinct clinical syndrome reflecting the actions of the ectopically secreted hormone. Other RFTs are listed as causing a possible specific syndrome either because there are too few cases or there is disagreement about whether the described features are actually a distinct syndrome (table (table1)1) [1,2,3,4,5,7].
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ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes.
'S. Karger AG', 2012Co-Authors: R. T. Jensen, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Guillaume Cadiot, R. Salazar, Reza KianmaneshAbstract:ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes
Maria Luisa Brandi - One of the best experts on this subject based on the ideXlab platform.
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enets consensus guidelines for the management of patients with digestive neuroEndocrine neoplasms functional pancreatic Endocrine Tumor Syndromes
Neuroendocrinology, 2012Co-Authors: Guillaume Cadiot, Wouter W De Herder, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Reza KianmaneshAbstract:Pancreatic Endocrine Tumors (p-NETs) include both pancreatic neuroEndocrine Tumors (p-NETs) associated with a functional syndrome (functional p-NETs) or those associated with no distinct clinical syndrome (non-functional p-NETs) [1,2,3,4]. Non-functional p-NETs frequently secrete pancreatic polypeptide, chromogranin A, neuron-specific enolase, human chorionic gonadotrophin subunits, calcitonin, neurotensin or other peptides, but they do not usually produce specific symptoms and thus are considered clinically to be non-functional Tumors [2,3,5,6,7]. Only the functional p-NETs will be considered in this section. The two most common functional p-NETs (gastrinomas, insulinomas) are considered separately, whereas the other well-described and possible rare functional p-NETs are considered together as a group called rare functional p-NETs (RFTs) (table (table1)1) [1,2,3,4]. Table 1 Functional pancreatic Endocrine Tumor (PET) Syndromes Gastrinomas are neuroEndocrine neoplasms, usually located in the duodenum or pancreas, that secrete gastrin and cause a clinical syndrome known as Zollinger-Ellison syndrome (ZES). ZES is characterized by gastric acid hypersecretion resulting in severe peptic disease (peptic ulcer disease (PUD), gastroesophageal reflux disease (GERD)) [8,9,10]. In this section, ZES due to both duodenal and pancreatic gastrinomas will be covered together because clinically they are similar [8,10]. Specific points related to gastrinomas associated with the genetic syndrome of Multiple Endocrine Neoplasia type 1 (MEN1) (25% of cases) will also be mentioned [11,12]. Insulinomas are neuroEndocrine neoplasms located in the pancreas that secrete insulin, which causes a distinct syndrome characterized by symptoms due to hypoglycemia [2,13,14,15]. The symptoms are typically associated with fasting and the majority of patients have symptoms secondary to hypoglycemic central nervous system (CNS) effects (headaches, confusion, visual disturbances, etc.) or due to catecholamine excess secondary to hypoglycemia (sweating, tremor, palpitations, etc.) [2,3,13,14,15]. RFTs can occur in the pancreas or in other locations (VIPomas, somatostatinomas, GRHomas, ACTHomas, p-NETs causing carcinoid syndrome or hypercalcemia (PTHrp-omas)) (table (table1)1) [1,2,3,4,5,7]. Each of the established RFT Syndromes is associated with a distinct clinical syndrome reflecting the actions of the ectopically secreted hormone. Other RFTs are listed as causing a possible specific syndrome either because there are too few cases or there is disagreement about whether the described features are actually a distinct syndrome (table (table1)1) [1,2,3,4,5,7].
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ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes.
'S. Karger AG', 2012Co-Authors: R. T. Jensen, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Guillaume Cadiot, R. Salazar, Reza KianmaneshAbstract:ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes
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skin lesions in hereditary Endocrine Tumor Syndromes
Endocrine Practice, 2011Co-Authors: A Saggini, Maria Luisa BrandiAbstract:OBJECTIVE: To review the main cutaneous manifestations of hereditary Endocrine Tumor Syndromes and discuss currently known molecular mechanisms involved in their pathogenesis. METHODS: On the basis of our collective experience and a comprehensive MEDLINE literature search of the English-language literature published between January 1957 and September 2010 using the search terms "skin," "cutaneous," "multiple Endocrine neoplasia," "Carney complex," and "McCune-Albright syndrome," we reviewed the dermatologic findings in multiple Endocrine neoplasia type 1 and type 2, Carney complex, and McCune-Albright syndrome. RESULTS: Although the category of hereditary Endocrine Tumor Syndromes consists of a broad spectrum of conditions, only the aforementioned few are prominently associated with cutaneous features. Because the cutaneous findings associated with these diseases are generally benign, they are often ignored or dismissed as ancillary findings in the context of severe systemic involvement. Accordingly, the pertinent literature is relatively scarce and often fails to provide a comprehensive insight about this issue. Nevertheless, timely recognition of such dermatologic manifestations may have a critical role in the early diagnosis and appropriate management of the related Syndromes. Moreover, specific genotype-phenotype correlations may convey important prognostic implications. CONCLUSION: Many physicians are unfamiliar with the cutaneous findings in the hereditary Endocrine Tumor Syndromes described in this review. Nonetheless, knowledge of their existence can have a major role in establishing an early diagnosis of these Syndromes and determining the patient's prognosis.
Guillaume Cadiot - One of the best experts on this subject based on the ideXlab platform.
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enets consensus guidelines for the management of patients with digestive neuroEndocrine neoplasms functional pancreatic Endocrine Tumor Syndromes
Neuroendocrinology, 2012Co-Authors: Guillaume Cadiot, Wouter W De Herder, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Reza KianmaneshAbstract:Pancreatic Endocrine Tumors (p-NETs) include both pancreatic neuroEndocrine Tumors (p-NETs) associated with a functional syndrome (functional p-NETs) or those associated with no distinct clinical syndrome (non-functional p-NETs) [1,2,3,4]. Non-functional p-NETs frequently secrete pancreatic polypeptide, chromogranin A, neuron-specific enolase, human chorionic gonadotrophin subunits, calcitonin, neurotensin or other peptides, but they do not usually produce specific symptoms and thus are considered clinically to be non-functional Tumors [2,3,5,6,7]. Only the functional p-NETs will be considered in this section. The two most common functional p-NETs (gastrinomas, insulinomas) are considered separately, whereas the other well-described and possible rare functional p-NETs are considered together as a group called rare functional p-NETs (RFTs) (table (table1)1) [1,2,3,4]. Table 1 Functional pancreatic Endocrine Tumor (PET) Syndromes Gastrinomas are neuroEndocrine neoplasms, usually located in the duodenum or pancreas, that secrete gastrin and cause a clinical syndrome known as Zollinger-Ellison syndrome (ZES). ZES is characterized by gastric acid hypersecretion resulting in severe peptic disease (peptic ulcer disease (PUD), gastroesophageal reflux disease (GERD)) [8,9,10]. In this section, ZES due to both duodenal and pancreatic gastrinomas will be covered together because clinically they are similar [8,10]. Specific points related to gastrinomas associated with the genetic syndrome of Multiple Endocrine Neoplasia type 1 (MEN1) (25% of cases) will also be mentioned [11,12]. Insulinomas are neuroEndocrine neoplasms located in the pancreas that secrete insulin, which causes a distinct syndrome characterized by symptoms due to hypoglycemia [2,13,14,15]. The symptoms are typically associated with fasting and the majority of patients have symptoms secondary to hypoglycemic central nervous system (CNS) effects (headaches, confusion, visual disturbances, etc.) or due to catecholamine excess secondary to hypoglycemia (sweating, tremor, palpitations, etc.) [2,3,13,14,15]. RFTs can occur in the pancreas or in other locations (VIPomas, somatostatinomas, GRHomas, ACTHomas, p-NETs causing carcinoid syndrome or hypercalcemia (PTHrp-omas)) (table (table1)1) [1,2,3,4,5,7]. Each of the established RFT Syndromes is associated with a distinct clinical syndrome reflecting the actions of the ectopically secreted hormone. Other RFTs are listed as causing a possible specific syndrome either because there are too few cases or there is disagreement about whether the described features are actually a distinct syndrome (table (table1)1) [1,2,3,4,5,7].
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ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes.
'S. Karger AG', 2012Co-Authors: R. T. Jensen, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Guillaume Cadiot, R. Salazar, Reza KianmaneshAbstract:ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes
Jean-yves Scoazec - One of the best experts on this subject based on the ideXlab platform.
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enets consensus guidelines for the management of patients with digestive neuroEndocrine neoplasms functional pancreatic Endocrine Tumor Syndromes
Neuroendocrinology, 2012Co-Authors: Guillaume Cadiot, Wouter W De Herder, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Reza KianmaneshAbstract:Pancreatic Endocrine Tumors (p-NETs) include both pancreatic neuroEndocrine Tumors (p-NETs) associated with a functional syndrome (functional p-NETs) or those associated with no distinct clinical syndrome (non-functional p-NETs) [1,2,3,4]. Non-functional p-NETs frequently secrete pancreatic polypeptide, chromogranin A, neuron-specific enolase, human chorionic gonadotrophin subunits, calcitonin, neurotensin or other peptides, but they do not usually produce specific symptoms and thus are considered clinically to be non-functional Tumors [2,3,5,6,7]. Only the functional p-NETs will be considered in this section. The two most common functional p-NETs (gastrinomas, insulinomas) are considered separately, whereas the other well-described and possible rare functional p-NETs are considered together as a group called rare functional p-NETs (RFTs) (table (table1)1) [1,2,3,4]. Table 1 Functional pancreatic Endocrine Tumor (PET) Syndromes Gastrinomas are neuroEndocrine neoplasms, usually located in the duodenum or pancreas, that secrete gastrin and cause a clinical syndrome known as Zollinger-Ellison syndrome (ZES). ZES is characterized by gastric acid hypersecretion resulting in severe peptic disease (peptic ulcer disease (PUD), gastroesophageal reflux disease (GERD)) [8,9,10]. In this section, ZES due to both duodenal and pancreatic gastrinomas will be covered together because clinically they are similar [8,10]. Specific points related to gastrinomas associated with the genetic syndrome of Multiple Endocrine Neoplasia type 1 (MEN1) (25% of cases) will also be mentioned [11,12]. Insulinomas are neuroEndocrine neoplasms located in the pancreas that secrete insulin, which causes a distinct syndrome characterized by symptoms due to hypoglycemia [2,13,14,15]. The symptoms are typically associated with fasting and the majority of patients have symptoms secondary to hypoglycemic central nervous system (CNS) effects (headaches, confusion, visual disturbances, etc.) or due to catecholamine excess secondary to hypoglycemia (sweating, tremor, palpitations, etc.) [2,3,13,14,15]. RFTs can occur in the pancreas or in other locations (VIPomas, somatostatinomas, GRHomas, ACTHomas, p-NETs causing carcinoid syndrome or hypercalcemia (PTHrp-omas)) (table (table1)1) [1,2,3,4,5,7]. Each of the established RFT Syndromes is associated with a distinct clinical syndrome reflecting the actions of the ectopically secreted hormone. Other RFTs are listed as causing a possible specific syndrome either because there are too few cases or there is disagreement about whether the described features are actually a distinct syndrome (table (table1)1) [1,2,3,4,5,7].
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ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes.
'S. Karger AG', 2012Co-Authors: R. T. Jensen, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Guillaume Cadiot, R. Salazar, Reza KianmaneshAbstract:ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes
Paul Komminoth - One of the best experts on this subject based on the ideXlab platform.
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enets consensus guidelines for the management of patients with digestive neuroEndocrine neoplasms functional pancreatic Endocrine Tumor Syndromes
Neuroendocrinology, 2012Co-Authors: Guillaume Cadiot, Wouter W De Herder, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Reza KianmaneshAbstract:Pancreatic Endocrine Tumors (p-NETs) include both pancreatic neuroEndocrine Tumors (p-NETs) associated with a functional syndrome (functional p-NETs) or those associated with no distinct clinical syndrome (non-functional p-NETs) [1,2,3,4]. Non-functional p-NETs frequently secrete pancreatic polypeptide, chromogranin A, neuron-specific enolase, human chorionic gonadotrophin subunits, calcitonin, neurotensin or other peptides, but they do not usually produce specific symptoms and thus are considered clinically to be non-functional Tumors [2,3,5,6,7]. Only the functional p-NETs will be considered in this section. The two most common functional p-NETs (gastrinomas, insulinomas) are considered separately, whereas the other well-described and possible rare functional p-NETs are considered together as a group called rare functional p-NETs (RFTs) (table (table1)1) [1,2,3,4]. Table 1 Functional pancreatic Endocrine Tumor (PET) Syndromes Gastrinomas are neuroEndocrine neoplasms, usually located in the duodenum or pancreas, that secrete gastrin and cause a clinical syndrome known as Zollinger-Ellison syndrome (ZES). ZES is characterized by gastric acid hypersecretion resulting in severe peptic disease (peptic ulcer disease (PUD), gastroesophageal reflux disease (GERD)) [8,9,10]. In this section, ZES due to both duodenal and pancreatic gastrinomas will be covered together because clinically they are similar [8,10]. Specific points related to gastrinomas associated with the genetic syndrome of Multiple Endocrine Neoplasia type 1 (MEN1) (25% of cases) will also be mentioned [11,12]. Insulinomas are neuroEndocrine neoplasms located in the pancreas that secrete insulin, which causes a distinct syndrome characterized by symptoms due to hypoglycemia [2,13,14,15]. The symptoms are typically associated with fasting and the majority of patients have symptoms secondary to hypoglycemic central nervous system (CNS) effects (headaches, confusion, visual disturbances, etc.) or due to catecholamine excess secondary to hypoglycemia (sweating, tremor, palpitations, etc.) [2,3,13,14,15]. RFTs can occur in the pancreas or in other locations (VIPomas, somatostatinomas, GRHomas, ACTHomas, p-NETs causing carcinoid syndrome or hypercalcemia (PTHrp-omas)) (table (table1)1) [1,2,3,4,5,7]. Each of the established RFT Syndromes is associated with a distinct clinical syndrome reflecting the actions of the ectopically secreted hormone. Other RFTs are listed as causing a possible specific syndrome either because there are too few cases or there is disagreement about whether the described features are actually a distinct syndrome (table (table1)1) [1,2,3,4,5,7].
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ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes.
'S. Karger AG', 2012Co-Authors: R. T. Jensen, Jean-yves Scoazec, Paul Komminoth, Gregory Kaltsas, Alain Sauvanet, Maria Luisa Brandi, Guillaume Cadiot, R. Salazar, Reza KianmaneshAbstract:ENETS Consensus Guidelines for the management of patients with digestive neuroEndocrine neoplasms: functional pancreatic Endocrine Tumor Syndromes