This page covers the clinical approach for children and adolescents diagnosed with a localized, resectable sympathetic paraganglioma — adrenal or extra-adrenal — where there is no evidence of metastatic or unresectable disease.

Clinical Scenario

A child or adolescent presents with localized sympathetic paraganglioma confined to an adrenal or extra-adrenal site and confirmed as surgically resectable. There is no metastatic involvement. Surgical resection is the mainstay of curative treatment for localized pediatric disease.

Preoperative Management

The preparatory approach before surgery centers on establishing alpha-adrenergic blockade in advance of the procedure, alongside measures to restore adequate volume status. The clinical goal is mild orthostatic hypotension — a controlled reduction in blood pressure on standing — while maintaining safe resting pressures. The complete preoperative regimen, including sequencing and all supportive interventions, is detailed in the full protocol.

References

DOI: 10.1093/ejendo/lvaf239

Surgical resection is the mainstay of curative treatment for localized pediatric PGL.
Alpha-adrenergic blockade is the cornerstone of preoperative management and should be initiated at least 7-14 days prior to surgery.
Volume expansion is vital to reverse chronic vasoconstriction and restore plasma volume.
The dosing goal is to achieve mild orthostatic hypotension, typically a fall in systolic blood pressure of up to ∼20 mmHg or a diastolic of ∼10 mmHg on standing, while maintaining tolerable supine pressures.

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Treatment of Sympathetic Paraganglioma in Children and Adolescents with Localized, Resectable Disease

Clinical Scenario

Preoperative Management

References