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Extra-adrenal pheochromocytomas (EAPs) may arise in any portion of the paraganglion system, though they most commonly occur below the diaphragm, frequently in the organ of Zuckerkandl. EAPs probably represent at least 15% of adult and 30% of childhood pheochromocytomas, as opposed to the traditional teaching that 10% of all pheochromocytomas are at extra-adrenal sites. They may be malignant in up to 40% of the cases, though conflicting data add to the uncertainty of this point. Patients with EAPs may present with headache, palpitations, sweating, or hypertension. A small percent of patients may also be asymptomatic at presentation due to nonfunctional tumors. The diagnosis is confirmed by demonstrating elevated blood and urine levels of catecholamines and their metabolites. Imaging studies to evaluate for EAPs include CT, MRI, and (131)I-labelled metaiodobenzylguanidine scintigraphy. Preoperative pharmacologic preparation, attentive intraoperative monitoring, and aggressive surgical therapy have important roles in achieving successful outcomes. Recent reports suggest that a laparoscopic approach, along with intraoperative ultrasound, can safely remove these tumors. EAPs recur and metastasize more often than their adrenal counterparts, making lifelong follow-up essential.

Adrenal tumors in children may be benign or malignant. In addition, both benign and malignant tumors may be hormonally active, leading to hypertension, metabolic crises, and endocrinopathies. These tumors may be found incidentally or secondary to signs and symptoms of the aforementioned disorders. Both a metabolic and a radiographic work-up are required before treatment of an adrenal tumor. The primary therapy for most adrenal lesions is surgical, though some are treated medically or require chemotherapy before excision. Laparoscopy has become the surgical approach of choice in both adult and pediatric patients with localized disease. Open surgical approaches remain necessary in patients with extensive locally invasive or metastatic disease.

Evaluation of a functional adrenal mass may be initiated based on symptomatic presentation or the detection of an incidental adrenal mass. Recent literature suggests that 10% to 20% of adrenal incidentalomas demonstrate subclinical hormonal dysfunction, which may place patients at a higher risk for metabolic or cardiovascular disorders. Many diagnostic algorithms have been proposed for the evaluation of pheochromocytoma, Cushing's adenoma, aldosteronoma, and hormonally active adrenal cortical carcinoma. In this article, the available literature on functional adrenal masses is reviewed and up-to-date methods of efficient diagnosis are proposed.