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Pheochromocytoma and paraganglioma in children

Author
William F Young, Jr, MD, MSc
Section Editors
Alberto S Pappo, MD
Mitchell E Geffner, MD
Deputy Editor
Alison G Hoppin, MD

INTRODUCTION

Pheochromocytomas and paragangliomas are rare tumors in children. Tumors that arise from the adrenal medulla are termed pheochromocytomas, and secrete catecholamines. Tumors with extra-adrenal origins are called paragangliomas, and either secrete catecholamines or are nonfunctional.

This topic review will discuss the clinical presentation, diagnosis, and management of pheochromocytomas and catecholamine-secreting paragangliomas in children. Given the limited experience with pheochromocytomas in children and the similarity in clinical presentation, diagnosis, and treatment among children and adults, the following discussion includes some data derived from the adult literature. Detailed reviews of these tumors in adults are presented separately. (See "Clinical presentation and diagnosis of pheochromocytoma" and "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology".)

DEFINITIONS

Pheochromocytoma – Pheochromocytomas are catecholamine-secreting tumors that arise from the chromaffin cells of the adrenal medulla.

Paraganglioma – Paragangliomas arise from the paraganglia of the autonomic nervous system outside of the adrenal medulla. Those that involve the sympathetic nervous system usually secrete catecholamines and typically are located in the lower mediastinum, abdomen, and pelvis. Those that involve the parasympathetic nervous system are usually nonfunctional and often located in the skull base, neck, and upper mediastinum. This topic review will focus only on catecholamine-secreting tumors, and not nonfunctional paragangliomas. (See "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology".)

Because pheochromocytomas and catecholamine-secreting paragangliomas have similar clinical presentations and are treated with similar approaches, many clinicians use the term "pheochromocytoma" to refer to both types of tumors. However, the distinction between pheochromocytoma and paraganglioma is an important one because of the implications for associated neoplasms, risk for malignancy, and implications for genetic testing.  

                                      
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Literature review current through: Nov 2017. | This topic last updated: Nov 08, 2017.
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