Pheochromocytoma and paraganglioma during childhood and adolescence

Abstract

Pheochromocytoma and paraganglioma belong to the group of rare catecholamine-producing tumours during childhood and adolescence. They occur most frequently in patients with tumour predisposition syndromes. Imaging is essential to assess tumour stage and to plan therapy initiation. The aim of this review is asummary of the most important characteristics of the aforementioned tumour entities with aspecial focus on imaging. In particular, magnetic resonance imaging (MRI) as well as nuclear medicine techniques are presented. Diagnostic methods in patients with pheochromocytoma and paraganglioma are presented based on the literature and own case reports. The radiologic modality of choice for the staging of catecholamine-producing tumours during childhood and adolescence is MRI, due to the lack of ionizing radiation and high soft tissue contrast. In addition, 123-I-meta-iodo-benzyl-guanidine (MIBG) scintigraphy and positron emission tomography/computer tomography (PET/CT) are performed. Whole-body MRI is particularly important as ascreening tool in patients with atumour predisposition syndrome. Radiologic imaging and nuclear medicine techniques are important for the assessment of disease stage and therapy planning in patients with catecholamine-producing tumours. Detection of metastatic disease is essential, as there are no known histopathologic markers, which can predict the metastatic potential of the tumours.