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
(Off-Label)
After clonidine suppression testing, if pheochromocytoma diagnosis happens, then oral administration of 0.3 mg for 60 to 80 kg weight of the patient where the blood sample is collected after 3 hours of administration to the supine patient
:
Dosage Modifications
Myelosuppression
Do not give the first therapeutic dosage if your platelet count is below 80,000/mcL or your ANC count is below 1,200/mcL
Pneumonitis
If pneumonitis is diagnosed following the initial therapeutic dosage, do not give the second therapeutic dose:
Dosimetric dose
Patients weighing more than 50 kg: The recommended dosimetric dosage as an intravenous injection is 185-222 MBq
Patients weight less than 50 kg: The recommended dosimetric dosage as an intravenous injection is (0.1 mCi/kg) 3.7 MBq/k
Therapeutic dose
Patients weighing more than 62.5 kg: 18,500 MBq
Patients weighing less than 62.5 kg: dose of 296 MBq/kg
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
Dosage Modifications
Myelosuppression
Do not give the first therapeutic dosage if your platelet count is below 80,000/mcL or your ANC count is below 1,200/mcL
Pneumonitis
If pneumonitis is diagnosed following the initial therapeutic dosage, do not give the second therapeutic dose:
Dosimetric dose
Patients weighing more than 50 kg: The recommended dosimetric dosage as an intravenous injection is 185-222 MBq
Patients weighing less than 50 kg: The recommended dosimetric dosage as an intravenous injection is 3.7 MBq/kg
Therapeutic dose
Patients weighing more than 62.5 kg: 18,500 MBq
Patients weighing less than 62.5 kg: 296 MBq/kg (8 mCi/kg)
Thyroid blockade
Inorganic iodine should be administered at least 24 hours before and for 10 days after each iobenguane I 131 treatments
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/
medtigo
Pheochromocytoma
Updated :
February 20, 2024
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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