Treatment of Metastatic or Unresectable Sympathetic Paraganglioma in Children and Adolescents
Clinical Scenario
This protocol applies to metastatic or unresectable adrenal or extra-adrenal sympathetic paraganglioma arising in children and adolescents. The goals are disease control, symptom palliation, and preservation of quality of life over what is often a prolonged course.
Pediatric Context
Metastatic disease is present in approximately 10%–20% of pediatric patients at diagnosis. In cohorts enriched for hereditary conditions, frequencies of 30%–50% have been reported. Patient age is a central factor shaping how therapy is selected and sequenced in this setting.
Treatment Approach (Partial Overview)
Management centers on radionuclide and focal therapies, with modality selection driven by tumor imaging characteristics and clinical burden. The full eligibility criteria, sequencing, and alternative options are in the protocol below.
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.
[131I]I-MIBG therapy is recommended for MIBG-avid metastatic PGL requiring tumor control or symptomatic relief.
Peptide receptor radionuclide therapy (PRRT) using radioactive STTR ligands such as [177Lu]Lu-DOTATATE may be considered in DOTATATE-avid tumors unsuitable for MIBG therapy.
Surgical debulking may be appropriate in oligometastatic cases to relieve symptoms or reduce catecholamine output.
Radiotherapy (conventional or stereotactic) may be used for unresectable or symptomatic lesions such as bone metastases.
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