Gastrinomas are neuroendocrine tumors defined by excessive gastric acid production, which leads to severe peptic ulcer disease and diarrhea, a combination commonly known as Zollinger-Ellison syndrome.
The most prevalent functional and malignant pancreatic endocrine tumors are gastrinomas. They are often diagnosed between the ages of 20 and 50, and their incidence is slightly higher in men.
At the time of diagnosis, at least 60% of gastrinoma cases have already metastasized.
Epidemiology
The annual incidence of gastrinomas is between 0.5 and 3 per million people. As a result of improvements in tumor detection procedures, the incidence rates are growing annually.
80-90% of these tumors grow in the so-called “gastrinoma triangle,” an anatomical region in the abdomen whose limits are created by the confluence of the cystic and common bile ducts, the second and third parts of the duodenum, and the neck of the pancreas.
Gastrinomas can also arise infrequently in the stomach, peri-pancreatic lymph nodes, liver, bile duct, ovary, and heart, as well as in conjunction with small cell lung cancer.
Duodenal gastrinomas are typically 1 cm in size, numerous, and primarily located in the first portion of the duodenum. They account for roughly 50 to 88 percent of gastrinomas associated with sporadic ZES and 70-100% of gastrinomas associated with MEN 1. 25% of these tumors are pancreatic gastrinomas, which are larger than duodenal cancers, and they can arise in any area of the pancreas.
Gastrinomas are the most prevalent malignant and functional pancreatic endocrine tumors. They are often diagnosed in individuals aged between 20-50, and their incidence is slightly higher in men.
In patients with MEN1, gastrinomas typically manifest sooner, between the ages of 10 and 30. Gastrinomas are slow-growing tumors, yet approximately 60% of them are malignant and have spread by the time of diagnosis
Anatomy
Pathophysiology
The gastrinoma tumor cells secrete an excessive amount of gastrin, resulting in hyperplasia of the fundic parietal cells and a rise in the basal stomach acid output.
The increased stomach acid output compromises the mucosal defences of the gastric and duodenal walls, produces ulceration, and deactivates pancreatic digesting enzymes, leading to fat malabsorption and diarrhea.
Small intestinal salt and water absorption inhibition leads in a secretory component of diarrhea. A well-differentiated neuroendocrine tumor exhibits an organoid cellular organisation with nesting, trabecular, or gyriform patterns.
The tumour cells are spherical with uniformly bland nuclei and produce copious amounts of secretory granules that display diffuse immunoexpression of neuroendocrine markers. Poorly differentiated NET, on the other hand, exhibits unusual, sheet-like, dispersed and irregular nuclei, fewer cytoplasmic secretory granules, and low biomarker immunoexpression.
Immunostaining for chromogranin A and synaptophysin is required for the accurate diagnosis of neuroendocrine tumors. Gastrin immunostaining can help differentiate neuroendocrine tumors from other types of tumors.
Due to the fact that gastrinomas express a high concentration of somatostatin receptors, somatostatin scintigraphy is a useful localization method.
In 2010, the WHO classified all neuroendocrine tumors into 3 grades. This classification was done on the basis of the mitotic rate, or the Ki-67 index.
The characteristics of these 3 grades are:
Grade I- Mitotic rate below 2 and Ki 97 index between 3%
Grade II- Mitotic rate between 2-20 and Ki index between 3%-20%
Grade III- Mitotic rate above 20 and Ki index above 20%
Around 50%-80% of neuroendocrine tumors are Grade II, and 10-30% are Grade I. At 1%-3%, Grade III neuroendocrine tumors are rare.
Etiology
In addition to other pancreatic endocrine cancers, these tumors appear to arise from endodermal pluripotent cells. 75%-80% of gastrinomas are either spontaneous or related with an autosomal dominant inherited disorder called multiple endocrine neoplasia type 1 or MEN1 syndrome.
MEN1 is caused by germline mutations in the chromosome 11q13 tumor suppressor gene MEN1. Multiple separate second-hit mutations in the MEN1 gene are theorized to be responsible for the development of tumors in vulnerable patients.
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References
https://www.ncbi.nlm.nih.gov/books/NBK441842/
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Gastrinomas are neuroendocrine tumors defined by excessive gastric acid production, which leads to severe peptic ulcer disease and diarrhea, a combination commonly known as Zollinger-Ellison syndrome.
The most prevalent functional and malignant pancreatic endocrine tumors are gastrinomas. They are often diagnosed between the ages of 20 and 50, and their incidence is slightly higher in men.
At the time of diagnosis, at least 60% of gastrinoma cases have already metastasized.
The annual incidence of gastrinomas is between 0.5 and 3 per million people. As a result of improvements in tumor detection procedures, the incidence rates are growing annually.
80-90% of these tumors grow in the so-called “gastrinoma triangle,” an anatomical region in the abdomen whose limits are created by the confluence of the cystic and common bile ducts, the second and third parts of the duodenum, and the neck of the pancreas.
Gastrinomas can also arise infrequently in the stomach, peri-pancreatic lymph nodes, liver, bile duct, ovary, and heart, as well as in conjunction with small cell lung cancer.
Duodenal gastrinomas are typically 1 cm in size, numerous, and primarily located in the first portion of the duodenum. They account for roughly 50 to 88 percent of gastrinomas associated with sporadic ZES and 70-100% of gastrinomas associated with MEN 1. 25% of these tumors are pancreatic gastrinomas, which are larger than duodenal cancers, and they can arise in any area of the pancreas.
Gastrinomas are the most prevalent malignant and functional pancreatic endocrine tumors. They are often diagnosed in individuals aged between 20-50, and their incidence is slightly higher in men.
In patients with MEN1, gastrinomas typically manifest sooner, between the ages of 10 and 30. Gastrinomas are slow-growing tumors, yet approximately 60% of them are malignant and have spread by the time of diagnosis
The gastrinoma tumor cells secrete an excessive amount of gastrin, resulting in hyperplasia of the fundic parietal cells and a rise in the basal stomach acid output.
The increased stomach acid output compromises the mucosal defences of the gastric and duodenal walls, produces ulceration, and deactivates pancreatic digesting enzymes, leading to fat malabsorption and diarrhea.
Small intestinal salt and water absorption inhibition leads in a secretory component of diarrhea. A well-differentiated neuroendocrine tumor exhibits an organoid cellular organisation with nesting, trabecular, or gyriform patterns.
The tumour cells are spherical with uniformly bland nuclei and produce copious amounts of secretory granules that display diffuse immunoexpression of neuroendocrine markers. Poorly differentiated NET, on the other hand, exhibits unusual, sheet-like, dispersed and irregular nuclei, fewer cytoplasmic secretory granules, and low biomarker immunoexpression.
Immunostaining for chromogranin A and synaptophysin is required for the accurate diagnosis of neuroendocrine tumors. Gastrin immunostaining can help differentiate neuroendocrine tumors from other types of tumors.
Due to the fact that gastrinomas express a high concentration of somatostatin receptors, somatostatin scintigraphy is a useful localization method.
In 2010, the WHO classified all neuroendocrine tumors into 3 grades. This classification was done on the basis of the mitotic rate, or the Ki-67 index.
The characteristics of these 3 grades are:
Grade I- Mitotic rate below 2 and Ki 97 index between 3%
Grade II- Mitotic rate between 2-20 and Ki index between 3%-20%
Grade III- Mitotic rate above 20 and Ki index above 20%
Around 50%-80% of neuroendocrine tumors are Grade II, and 10-30% are Grade I. At 1%-3%, Grade III neuroendocrine tumors are rare.
In addition to other pancreatic endocrine cancers, these tumors appear to arise from endodermal pluripotent cells. 75%-80% of gastrinomas are either spontaneous or related with an autosomal dominant inherited disorder called multiple endocrine neoplasia type 1 or MEN1 syndrome.
MEN1 is caused by germline mutations in the chromosome 11q13 tumor suppressor gene MEN1. Multiple separate second-hit mutations in the MEN1 gene are theorized to be responsible for the development of tumors in vulnerable patients.
https://www.ncbi.nlm.nih.gov/books/NBK441842/
medtigo
Gastrinoma
Updated :
September 5, 2023
Gastrinomas are neuroendocrine tumors defined by excessive gastric acid production, which leads to severe peptic ulcer disease and diarrhea, a combination commonly known as Zollinger-Ellison syndrome.
The most prevalent functional and malignant pancreatic endocrine tumors are gastrinomas. They are often diagnosed between the ages of 20 and 50, and their incidence is slightly higher in men.
At the time of diagnosis, at least 60% of gastrinoma cases have already metastasized.
The annual incidence of gastrinomas is between 0.5 and 3 per million people. As a result of improvements in tumor detection procedures, the incidence rates are growing annually.
80-90% of these tumors grow in the so-called “gastrinoma triangle,” an anatomical region in the abdomen whose limits are created by the confluence of the cystic and common bile ducts, the second and third parts of the duodenum, and the neck of the pancreas.
Gastrinomas can also arise infrequently in the stomach, peri-pancreatic lymph nodes, liver, bile duct, ovary, and heart, as well as in conjunction with small cell lung cancer.
Duodenal gastrinomas are typically 1 cm in size, numerous, and primarily located in the first portion of the duodenum. They account for roughly 50 to 88 percent of gastrinomas associated with sporadic ZES and 70-100% of gastrinomas associated with MEN 1. 25% of these tumors are pancreatic gastrinomas, which are larger than duodenal cancers, and they can arise in any area of the pancreas.
Gastrinomas are the most prevalent malignant and functional pancreatic endocrine tumors. They are often diagnosed in individuals aged between 20-50, and their incidence is slightly higher in men.
In patients with MEN1, gastrinomas typically manifest sooner, between the ages of 10 and 30. Gastrinomas are slow-growing tumors, yet approximately 60% of them are malignant and have spread by the time of diagnosis
The gastrinoma tumor cells secrete an excessive amount of gastrin, resulting in hyperplasia of the fundic parietal cells and a rise in the basal stomach acid output.
The increased stomach acid output compromises the mucosal defences of the gastric and duodenal walls, produces ulceration, and deactivates pancreatic digesting enzymes, leading to fat malabsorption and diarrhea.
Small intestinal salt and water absorption inhibition leads in a secretory component of diarrhea. A well-differentiated neuroendocrine tumor exhibits an organoid cellular organisation with nesting, trabecular, or gyriform patterns.
The tumour cells are spherical with uniformly bland nuclei and produce copious amounts of secretory granules that display diffuse immunoexpression of neuroendocrine markers. Poorly differentiated NET, on the other hand, exhibits unusual, sheet-like, dispersed and irregular nuclei, fewer cytoplasmic secretory granules, and low biomarker immunoexpression.
Immunostaining for chromogranin A and synaptophysin is required for the accurate diagnosis of neuroendocrine tumors. Gastrin immunostaining can help differentiate neuroendocrine tumors from other types of tumors.
Due to the fact that gastrinomas express a high concentration of somatostatin receptors, somatostatin scintigraphy is a useful localization method.
In 2010, the WHO classified all neuroendocrine tumors into 3 grades. This classification was done on the basis of the mitotic rate, or the Ki-67 index.
The characteristics of these 3 grades are:
Grade I- Mitotic rate below 2 and Ki 97 index between 3%
Grade II- Mitotic rate between 2-20 and Ki index between 3%-20%
Grade III- Mitotic rate above 20 and Ki index above 20%
Around 50%-80% of neuroendocrine tumors are Grade II, and 10-30% are Grade I. At 1%-3%, Grade III neuroendocrine tumors are rare.
In addition to other pancreatic endocrine cancers, these tumors appear to arise from endodermal pluripotent cells. 75%-80% of gastrinomas are either spontaneous or related with an autosomal dominant inherited disorder called multiple endocrine neoplasia type 1 or MEN1 syndrome.
MEN1 is caused by germline mutations in the chromosome 11q13 tumor suppressor gene MEN1. Multiple separate second-hit mutations in the MEN1 gene are theorized to be responsible for the development of tumors in vulnerable patients.
https://www.ncbi.nlm.nih.gov/books/NBK441842/
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