Pheochromocytoma (Chromaffin Cell Cancer) is a tumor that can produce hormones and develop in the adrenal glands. Mr. Pick coined the word “pheochromocytoma” in 1912. Chromaffin cells found in the adrenal medulla and paraganglion cells can develop into a tumor called a pheochromocytoma.
It was given this name because these tissues respond differently when chromium salt is applied to them. In 1937, Dr. Charles Mayo published the first pheochromocytoma case, which included a medical assessment and a surgical excision for therapeutic purposes.
Epidemiology
A very uncommon neuroendocrine condition is pheochromocytoma. It affects between 0.05 percent and 0.2 percent of people with hypertension. It is detected in roughly two to eight people out of 1 million people in the US, with a yearly incidence of 0.8 every 100,000 individuals’-years.
Both genders are equally affected by it. Pheochromocytoma is often diagnosed between the ages of 30 and 50, but it might manifest sooner due to hereditary susceptibility.
Anatomy
Pathophysiology
A neuroendocrine neoplasm that secretes catecholamines is called a pheochromocytoma. It belongs to one of the three catecholamine-secreting kinds.
Tumor that secretes epinephrine and norepinephrine
Tumors produce just norepinephrine and are typically associated with persistent hypertension. Paroxysmal hypertension is a presenting symptom of tumors that secrete norepinephrine and epinephrine. Hypotension, as opposed to hypertension, can only be brought on by epinephrine.
The inotropic impact, systemic vascular resistance, heart rate, and decrease in venous compliance all rise as a result of catecholamine secretion. Postural hypotension occurs in pheochromocytoma because it is a volume-depletion form of hypertension.
Etiology
90 percent of the time, pheochromocytoma is sporadic; however, in 10 percent of cases, it is hereditary and linked to disorders including Von Hippel-Lindau syndrome, multiple endocrine neoplasm syndromes type IIA and IIB, and neurofibromatosis type 1 with an autosomal dominant transmission mechanism.
Genetics
Prognostic Factors
Surgery typically cures non-metastatic cancers or benign ones. 50 percent of metastatic cancers survive for five years.
Starting dose of 250 mg taken orally every six hours and increase by 250 to 500 mg/day, but not exceeding a maximum of 4 g/day
Maintenance dose of usually 2 to 3 g/day, divided into four equal doses every six hours Dosing Considerations
Not advised in hypertension
Gamma Scintigraphy
Administer dose of 10 millicurie intravenously
Begin whole body planar scintigraphy imaging 24 hours (plus or minus 6 hours) after administration
For <12 years: Safety and efficacy not determined
For ≥12 years
Starting dose of 250 mg taken orally every six hours and increase by 250 to 500 mg/day, but not exceeding a maximum of 4 g/day
Maintenance dose of usually 2 to 3 g/day, divided into four equal doses every six hours Dosing Considerations
Not advised in hypertension
Pheochromocytoma (Chromaffin Cell Cancer) is a tumor that can produce hormones and develop in the adrenal glands. Mr. Pick coined the word “pheochromocytoma” in 1912. Chromaffin cells found in the adrenal medulla and paraganglion cells can develop into a tumor called a pheochromocytoma.
It was given this name because these tissues respond differently when chromium salt is applied to them. In 1937, Dr. Charles Mayo published the first pheochromocytoma case, which included a medical assessment and a surgical excision for therapeutic purposes.
A very uncommon neuroendocrine condition is pheochromocytoma. It affects between 0.05 percent and 0.2 percent of people with hypertension. It is detected in roughly two to eight people out of 1 million people in the US, with a yearly incidence of 0.8 every 100,000 individuals’-years.
Both genders are equally affected by it. Pheochromocytoma is often diagnosed between the ages of 30 and 50, but it might manifest sooner due to hereditary susceptibility.
A neuroendocrine neoplasm that secretes catecholamines is called a pheochromocytoma. It belongs to one of the three catecholamine-secreting kinds.
Tumor that secretes epinephrine and norepinephrine
Tumors produce just norepinephrine and are typically associated with persistent hypertension. Paroxysmal hypertension is a presenting symptom of tumors that secrete norepinephrine and epinephrine. Hypotension, as opposed to hypertension, can only be brought on by epinephrine.
The inotropic impact, systemic vascular resistance, heart rate, and decrease in venous compliance all rise as a result of catecholamine secretion. Postural hypotension occurs in pheochromocytoma because it is a volume-depletion form of hypertension.
90 percent of the time, pheochromocytoma is sporadic; however, in 10 percent of cases, it is hereditary and linked to disorders including Von Hippel-Lindau syndrome, multiple endocrine neoplasm syndromes type IIA and IIB, and neurofibromatosis type 1 with an autosomal dominant transmission mechanism.
Surgery typically cures non-metastatic cancers or benign ones. 50 percent of metastatic cancers survive for five years.
Starting dose of 250 mg taken orally every six hours and increase by 250 to 500 mg/day, but not exceeding a maximum of 4 g/day
Maintenance dose of usually 2 to 3 g/day, divided into four equal doses every six hours Dosing Considerations
Not advised in hypertension
Gamma Scintigraphy
Administer dose of 10 millicurie intravenously
Begin whole body planar scintigraphy imaging 24 hours (plus or minus 6 hours) after administration
For <12 years: Safety and efficacy not determined
For ≥12 years
Starting dose of 250 mg taken orally every six hours and increase by 250 to 500 mg/day, but not exceeding a maximum of 4 g/day
Maintenance dose of usually 2 to 3 g/day, divided into four equal doses every six hours Dosing Considerations
Not advised in hypertension
Gamma Scintigraphy
Start full body planar scintigraphy imaging 24 hours (plus or minus 6 hours) after administration
https://www.ncbi.nlm.nih.gov/books/NBK535360/
medtigo
Pheochromocytoma
Updated :
September 6, 2023
Pheochromocytoma (Chromaffin Cell Cancer) is a tumor that can produce hormones and develop in the adrenal glands. Mr. Pick coined the word “pheochromocytoma” in 1912. Chromaffin cells found in the adrenal medulla and paraganglion cells can develop into a tumor called a pheochromocytoma.
It was given this name because these tissues respond differently when chromium salt is applied to them. In 1937, Dr. Charles Mayo published the first pheochromocytoma case, which included a medical assessment and a surgical excision for therapeutic purposes.
A very uncommon neuroendocrine condition is pheochromocytoma. It affects between 0.05 percent and 0.2 percent of people with hypertension. It is detected in roughly two to eight people out of 1 million people in the US, with a yearly incidence of 0.8 every 100,000 individuals’-years.
Both genders are equally affected by it. Pheochromocytoma is often diagnosed between the ages of 30 and 50, but it might manifest sooner due to hereditary susceptibility.
A neuroendocrine neoplasm that secretes catecholamines is called a pheochromocytoma. It belongs to one of the three catecholamine-secreting kinds.
Tumor that secretes epinephrine and norepinephrine
Tumors produce just norepinephrine and are typically associated with persistent hypertension. Paroxysmal hypertension is a presenting symptom of tumors that secrete norepinephrine and epinephrine. Hypotension, as opposed to hypertension, can only be brought on by epinephrine.
The inotropic impact, systemic vascular resistance, heart rate, and decrease in venous compliance all rise as a result of catecholamine secretion. Postural hypotension occurs in pheochromocytoma because it is a volume-depletion form of hypertension.
90 percent of the time, pheochromocytoma is sporadic; however, in 10 percent of cases, it is hereditary and linked to disorders including Von Hippel-Lindau syndrome, multiple endocrine neoplasm syndromes type IIA and IIB, and neurofibromatosis type 1 with an autosomal dominant transmission mechanism.
Surgery typically cures non-metastatic cancers or benign ones. 50 percent of metastatic cancers survive for five years.
https://www.ncbi.nlm.nih.gov/books/NBK535360/
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