Childhood Pheochromocytoma

Definition

Childhood pheochromocytoma and paraganglioma are rare tumors that arise from nerve tissue in children. Pheochromocytomas form in the adrenal glands, while paragangliomas develop outside the adrenal glands along nerve pathways.

Characteristics

These tumors often secrete excess hormones, leading to symptoms like high blood pressure, headaches, sweating, rapid heartbeat, anxiety, and weight loss. Some tumors are nonfunctional and may cause symptoms by pressing on nearby organs.

Diagnosis and Prognosis

Diagnosis involves blood and urine tests to detect elevated hormone levels, imaging studies such as MRI, CT, or MIBG scans, and genetic testing due to the frequent association with inherited syndromes. Prognosis is good when tumors are detected early and completely removed, but long-term follow-up is necessary because of the risk of recurrence or metastasis.

Treatment

Treatment typically involves surgery to remove the tumor. Pre-surgical medications are used to control blood pressure and heart rate. In cases of metastasis or recurrence, additional treatments like targeted therapy, radiation, or chemotherapy may be needed.

Last Revised: 12 May , 2025