Current concepts of pheochromocytoma

Abstract

Pheochromocytoma (PCC), a rare neuroendocrine tumor, shows a prevalence ranging between 0.1% and 0.6% in individuals suffering from hypertension. To date, an increasing number of patients with hereditary forms or subclinical PCCs have been diagnosed. We reviewed the main controversies and the most recent updates, especially inheritance genetics and surgical management. 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. Alpha 1 blocker therapy, alone or in combination with beta blockers, calcium antagonists, and plasma volume expansion, is the most commonly used preoperative treatment protocol. Minimally invasive adrenalectomy (laparoscopic and retro-peritoneoscopic) allows earlier mobilization and recovery, reducing the risk of pulmonary infections and thromb-oembolic complications, and is associated with lower morbidity and mortality rates than traditional surgery; it is currently considered the gold standard for the treatment of adrenal tumors ≤6 cm in diameter and weighing < 100 g. Genetic testing will increasingly be the key factor in estimating the life-long risk for development of recurrent disease, contralateral disease or malignant dedifferentiation, thus influencing follow-up protocols.

Perioperative Management of Adrenalectomy and Inferior Vena Cava Reconstruction in a Patient With a Large, Malignant Pheochromocytoma With Vena Caval Extension

“…The annual incidence of these tumors is approximately 2-to-8 cases per 1 million, with 80% to 85% being pheochromocytomas and the rest paragangliomas. 2,63 Pheochromocytomas can metastasize in about 10% of patients, invading the liver and beyond. 2,63 Factors associated with tumor malignancy in this setting include large tumor size and genetic susceptibilities.…”

“…2,63 Pheochromocytomas can metastasize in about 10% of patients, invading the liver and beyond. 2,63 Factors associated with tumor malignancy in this setting include large tumor size and genetic susceptibilities. 2,62,63 Although the diagnosis of pheochromocytoma typically is based on classic clinical signs and symptoms that develop secondary to catecholamine release, there is a wide range of clinical presentations because this catecholamine production is highly variable.…”

“…2,63 Factors associated with tumor malignancy in this setting include large tumor size and genetic susceptibilities. 2,62,63 Although the diagnosis of pheochromocytoma typically is based on classic clinical signs and symptoms that develop secondary to catecholamine release, there is a wide range of clinical presentations because this catecholamine production is highly variable. 2,62 In fact, the initial clinical presentation of pheochromocytoma can be as a perioperative hypertensive crisis.…”

“…2,62,63 Although the diagnosis of pheochromocytoma typically is based on classic clinical signs and symptoms that develop secondary to catecholamine release, there is a wide range of clinical presentations because this catecholamine production is highly variable. 2,62 In fact, the initial clinical presentation of pheochromocytoma can be as a perioperative hypertensive crisis. 64 Suspicion of a pheochromocytoma should prompt measurement of catecholamine levels, such as total metanephrines in the urine, as was done in this case conference (Fig 4).…”

“…1 The only cure for pheochromocytoma is surgical resection, with most recent studies demonstrating minimal or zero perioperative mortality, which commonly is attributed to improved preoperative preparation. 2,3 There are case reports demonstrating that large pheochromocytomas have the potential to invade into the IVC and result in significant IVC thrombus formation. 4,5 To date, there exists minimal literature documenting the anesthetic planning and management of these complicated patients.…”