Editing Adrenal: Pheochromocytoma (section)

=== Special Scenarios ===

==== Treatment of Hereditary Pheochromocytoma ====
* Given that for patients with MEN-2 and VHL, the risk of malignancy is low whereas the risk of bilateral disease is significant, '''partial cortical-sparing adrenalectomy''' has been advocated. This strategy is used to avoid lifelong hormonal replacement, with its associated morbidity

==== Treatment of Malignant Pheochromocytoma ====
* '''Currently, malignant pheochromocytoma can only be defined by the presence of clinical metastases'''.
** A number of pathologic criteria to differentiate benign from malignant disease have been proposed, but to date there is no criterion agreed on.
*Currently therapy for metastatic pheochromocytoma is largely '''palliative'''.
* '''Surgical metastasectomy of resectable disease is the standard of care'''
** Little evidence exists to demonstrate that it prolongs patient survival or is more effective for symptomatic relief than medical treatment with α/β-blockade and α-methyl-p-tyrosine
* Chemotherapy is primarily used in patients in whom MIBG therapy has failed or in those whose tumors do not demonstrate MIBG uptake on initial MIBG imaging studies.

==== Treatment of Pheochromocytoma in Pregnancy ====
* In a late term pregnancy, the patient should be treated with alpha-adrenergic blockade with phenoxybenzamine until the fetus has reached maturity to manage the hypertension. At this point, she should undergo caesarean section and tumor resection in one operation. The patient should not undergo the stress of vaginal delivery.