Pediatric pheochromocytoma: current status of diagnostic imaging and treatment procedures.

PURPOSE OF REVIEW

To provide an overview of relevant data available and updated recommendations for management of pediatric patients with pheochromocytoma (PCC).

RECENT FINDINGS

Much of the available data surrounding pediatric PCC is in the form of case reports and case series. With the accumulation of data over time, pediatric PCC does in fact differ significantly from not only what is known in the adult population, but also from classic teaching. Pediatric patients are much more likely to have a hereditary predisposition as well as aggressive and malignant disease. Much of the recent literature focuses on defining these genetic syndromes in order to provide recommendations for screening and genetic counseling. Other recent advances center around developing treatments for metastatic disease. Timely diagnosis with plasma metanephrines and cross-sectional imaging, and appropriate preoperative medical optimization followed by surgical resection remain the center of treatment.

SUMMARY

Although rare and adult principles are applied to pediatric PCC, genetic testing plays a pivotal role in management of children, adolescents and young adults with PCC.