Current Approaches and Recommended Algorithm for the Diagnostic Localization of Pheochromocytoma

Abstract

Pheochromocytomas (PHEO) (1-3) are catecholamineproducing tumors that arise from chromaffin cells. PHEO are mostly situated within the adrenal medulla, although in about 9 -23% of cases, tumors develop from extraadrenal chromaffin tissue (adjacent to sympathetic ganglia of the neck, mediastinum, abdomen, and pelvis) and are often referred to as paragangliomas (4 -6).PHEO situated in the adrenal gland are identified more commonly than those in extraadrenal tissues, because clinicians usually focus on the adrenal gland as a main source of catecholamine production. Although they usually choose a proper imaging technique to attempt the localization of PHEO in the adrenal gland, they are often confused as to which algorithm to follow and what technique to choose for the detection of extraadrenal PHEO. Furthermore, physicians often neglect the facts that up to approximately 25% of patients with apparent sporadic PHEO may, in fact, be carriers of germline mutations, indicating hereditary disease with a predisposition for extraadrenal, often multifocal, PHEO (7); that in children, multifocal and extraadrenal PHEO are found in up to 30 -43% of cases (6, 8 -12); that malignant PHEO account for up to 26 -35% of cases (13)(14)(15)(16)(17)(18); that the prevalence of malignancy in sporadic adrenal PHEO is 9% (5); and that about 10% of patients with PHEO present with metastatic disease at the time of their initial work-up (19). After initial failed surgery, patients with metastatic PHEO are commonly reevaluated using metaiodobenzylguanidine (MIBG) scintigraphy, a modality that should actually be performed before surgery to confirm that a tumor was indeed a PHEO (5-9% of the population harbor an adrenal tumor, most commonly a benign adenoma) (20 -22) or to rule out metastatic disease. These patients are then reoperated upon, suffering additional surgery-related complications and substantial financial expenses. Some clinicians consider the presence of sporadic unilateral small PHEO as indicative of benign disease. This was concluded in older studies of series of patients with . However, more recent works from large series of patients with benign and malignant disease in the United States and Europe do not support this view; rather, they support the opinion that there are no absolute clinical, imaging, or laboratory criteria to predict malignancy and clinical course of PHEO (5, 16, 19,[26][27][28][29][30][31][32]. Nevertheless, PHEO tumors with a diameter larger than 5 cm have a higher potential to metastasize, and such patients should be followed more frequently (33). Consequently, it seems that ruling out metastatic PHEO before initial surgery would be useful, because the detection of other lesions may dramatically affect treatment and follow-up.Localization of PHEO should be attempted using at least two imaging modalities. Anatomical imaging studies [computed tomography (CT) and magnetic resonance imaging (MRI)] should be combined with functional (nuclear medicine) imaging studies for optimal results to locate primary, recurr...

Evaluation of the clonidine suppression test in the diagnosis of phaeochromocytoma

“…Most are situated within the adrenal medulla; however, between 9 and 23% develop from extraadrenal chromograffin tissue and are referred to as paragangliomas. 1 Diagnosis is important as, if untreated, they can lead to fatal cardiovascular complications. 2 They should be considered in patients with sustained or paroxysmal hypertension especially if accompanied by headache, palpitations (with or without tachycardia), sweating, pallor, tremor, extreme anxiety and hyperglycaemia.…”

Personalized diagnostics of chromaffin tumors (pheochromocytoma, paraganglioma) in oncoendocrinology

“…Хотя КТ и МРТ имеют практически одина-ковую диагностическую ценность [6,60,61], КТ рекомендована в качестве метода первой линии [43]. МРТ предпочтительна для топи-ческой диагностики ПГ головы и шеи, мета-стазов, при аллергии на контрастное веще-ство и пациентам c абсолютными и относи-тельными противопоказаниями к лучевой нагрузке (детский возраст, беременные, не-давняя лучевая терапия) [43,60].…”

“…МРТ предпочтительна для топи-ческой диагностики ПГ головы и шеи, мета-стазов, при аллергии на контрастное веще-ство и пациентам c абсолютными и относи-тельными противопоказаниями к лучевой нагрузке (детский возраст, беременные, не-давняя лучевая терапия) [43,60].…”

“…В первичной диагностике ПГ/ФХ, а также при рецидиве и метастатической диссеми-нации опухоли чувствительность и специ-фичность КТ невысоки, ниже 60% (57 и 50% соответственно) [60,67].…”

“…Согласно клиническим рекомендациям, оптимальные результаты достигаются путем совмещения двух методов лучевой визуали-зации: анатомической (КТ, МРТ) и функцио-нальной (ОФЭКТ, ПЭТ) [60,71].…”