title: 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.
authors.affiliation: Department of Anaesthesiologic, Surgical and Emergency Science, VII Division of General and Endocrine Surgery, Second University of Naples, Via Gen. Giordano Orsini 42, 80132 Naples, Italy. Electronic address: giovanni.conzo@unina2.it.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Conzo
authors.affiliation: Department of Cardio-Thoracic and Respiratory Science, Endocrinology Unit, Second University of Naples, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Pasquali
authors.affiliation: Department of Biological, Geological and Environmental Sciences, University of Sannio, Benevento, Italy.
authors.forename: Vittorio
authors.initials: V
authors.lastname: Colantuoni
authors.affiliation: National Cancer Institute "G. Pascale", Naples, Italy.
authors.forename: Luisa
authors.initials: L
authors.lastname: Circelli
authors.affiliation: Department of Anaesthesiologic, Surgical and Emergency Science, VII Division of General and Endocrine Surgery, Second University of Naples, Via Gen. Giordano Orsini 42, 80132 Naples, Italy.
authors.forename: Ernesto
authors.initials: E
authors.lastname: Tartaglia
authors.affiliation: Department of Anaesthesiologic, Surgical and Emergency Science, VII Division of General and Endocrine Surgery, Second University of Naples, Via Gen. Giordano Orsini 42, 80132 Naples, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Gambardella
authors.affiliation: Department of Anaesthesiologic, Surgical and Emergency Science, VII Division of General and Endocrine Surgery, Second University of Naples, Via Gen. Giordano Orsini 42, 80132 Naples, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Napolitano
authors.affiliation: Department of Anaesthesiologic, Surgical and Emergency Science, VII Division of General and Endocrine Surgery, Second University of Naples, Via Gen. Giordano Orsini 42, 80132 Naples, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Mauriello
authors.affiliation: Department of General Surgery, S. Maria Hospital, University of Perugia, Terni, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Avenia
authors.affiliation: Department of Anaesthesiologic, Surgical and Emergency Science, VII Division of General and Endocrine Surgery, Second University of Naples, Via Gen. Giordano Orsini 42, 80132 Naples, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Santini
authors.affiliation: Department of Cardio-Thoracic and Respiratory Science, Endocrinology Unit, Second University of Naples, Italy.
authors.forename: Antonio Agostino
authors.initials: AA
authors.lastname: Sinisi
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D010673:Pheochromocytoma / Q000473:pathology* / Q000628:therapy*
keywords: Adrenergic blocking drugs; Laparoscopic adrenalectomy; Pheochromocytoma