This protocol addresses metastatic or unresectable adrenal or extra-adrenal sympathetic paraganglioma arising in a child or adolescent. Management in this setting demands an individualized, multidisciplinary approach oriented toward disease control, symptom palliation, and preservation of quality of life over a potentially prolonged course.
Metastatic disease is identified in approximately 10%–20% of pediatric patients at diagnosis; rates as high as 30%–50% have been reported in cohorts enriched for hereditary conditions. Age-specific considerations shape every aspect of treatment selection and sequencing.
For aggressive or rapidly progressing disease, systemic chemotherapy represents one considered option — the specific agents chosen and the overall treatment strategy depend on individual clinical factors. The complete structured regimen and decision algorithm are available via the full protocol.
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.
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.
Cyclophosphamide, vincristine, dacarbazine (CVD) chemotherapy is reserved for aggressive or rapidly progressing disease due to its toxicity profile.
Temozolomide is generally well tolerated and has demonstrated activity in adult and limited pediatric series.
View source ↗