What is the rule of 10 in pheochromocytoma?
According to the “rule of 10”, in 1/10 patients with pheochromocytoma it is malignant, in 1/10 of cases the tumor is bilateral, in 1/10 extra-adrenal and in 1/10 familial. Surgical resection, the only curative treatment, carries a high risk of hypertensive crises due to massive catecholamine release.
Which is the most common surgical treatment option for a person with pheochromocytoma?
Complete surgical excision is the definitive treatment for benign and malignant pheochromocytoma with low morbidity and mortality. Laparoscopic adrenalectomy is not recommended for malignant pheochromocytoma and tumors larger than 8 cm.
What is MIBG scan pheochromocytoma?
MIBG is a nuclear scan test that uses injected radioactive material (radioisotope) and a special scanner to locate or confirm the presence of pheochromocytoma and neuroblastoma, which are tumors of specific types of nervous tissue. Alternative Names: Adrenal medullary imaging.
Why is pheochromocytoma known as 10% tumor?
They are often called the “10% tumor” because it was commonly thought that 10% of pheochromocytomas are: Malignant – 10% behave like cancer and can spread. Bilateral – 10% are found in both adrenal glands. Pediatric – 10% are found in children.
How do alpha blockers treat pheochromocytoma?
During resection of the tumor, the source of excess hormone secretion is abruptly removed, which can lead to life-threatening blood pressure fluctuations during surgery. Alpha blockers are a class of medication that blocks the alpha receptor on blood vessels.
What is the removal of pheochromocytoma?
Surgery to remove pheochromocytoma is usually an adrenalectomy (removal of one or both adrenal glands). During this surgery, the tissues and lymph nodes inside the abdomen will be checked and if the tumor has spread, these tissues may also be removed.
How fast do pheochromocytomas grow?
Most people develop a pheochromocytoma in 1 adrenal gland. Some people develop a tumor in both glands. There can also be multiple tumors in a gland. Pheochromocytomas usually grow slowly.
How is the diagnosis and treatment of pheochromocytoma done?
Pheochromocytoma is diagnosed by measuring elevated levels of metanephrines (catecholamine metabolites) in blood or urine. CT scanning or MRI is the preferred technique for localizing pheochromocytomas (see the image below). Surgical resection of the tumor is the treatment of choice and usually cures the hypertension (see Workup and Treatment).
What can MIBG uptake scanning be used for?
Some authors prefer to use MIBG uptake scanning as the initial screening modality because it enables whole-body imaging, making it useful for the detection of extra-adrenal tumors and metastatic deposits. [ 1, 4, 5, 6, 2, 7, 8, 9, 10, 11]
Which is better pet or CT for pheochromocytoma?
Saad et al found in a study of 23 patients with a history of pheochromocytoma/paraganglioma that (18)F-FDG PET/CT was a superior tool for the localization of recurrent tumors. [ 15]
Is there a 10% false positive rate for MIBG?
False positives/negatives. As a result of the 10% false-negative rate with MIBG scanning, some authors recommend abdominal CT scanning or MRI if a high clinical suspicion of pheochromocytoma exists but a causative tumor is not identified by assessing MIBG uptake.