Sympathetic paraganglioma
ICD-10 D44.7 · ICD-11 2F9A&XH4G21

Treatment of Metastatic or Unresectable Sympathetic Paraganglioma in Children and Adolescents

This protocol covers the clinical management of metastatic or unresectable adrenal or extra-adrenal sympathetic paraganglioma occurring in a child or adolescent — a population with distinct therapeutic considerations.

Clinical Scenario

Metastatic and unresectable pediatric sympathetic paraganglioma requires individualized, multidisciplinary care aimed at balancing disease control, symptom palliation, and preservation of quality of life over what is often a prolonged disease course.

Metastatic disease is present in approximately 10%–20% of pediatric patients at diagnosis, with higher frequencies reported in cohorts enriched for hereditary conditions.

Treatment Approach

Targeted systemic therapy — with the specific agent determined by the patient's age and disease course — forms a central part of management in this setting. The complete regimen, eligibility criteria, and sequencing are in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1093/ejendo/lvaf239

Metastatic and unresectable pediatric PGL requires individualized, multidisciplinary care aimed at balancing disease control, symptom palliation, and preservation of quality of life over what is often a prolonged disease course (Figure 2).

Metastatic disease is present in approximately 10%-20% of pediatric patients at diagnosis, with higher frequencies—up to 30%-50%—reported in cohorts enriched for hereditary conditions, particularly SDHB PVs.

Tyrosine kinase inhibitors (eg, sunitinib) may be considered in refractory progressive disease, though monitoring for toxicity (eg, hypertension, fatigue) is essential.

However, as of May 14, 2025, the U.S. Food and Drug Administration has approved belzutifan for adult and pediatric patients aged 12 years and older with locally advanced, unresectable, or metastatic PGL.

View source ↗