OnCo

Pheochromocytoma and paraganglioma (PPGL): lines of therapy

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3 standard-of-care settings across 3 lines and 1 biomarker subgroup. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

LineAll comers
Early / localised1
Advanced, first line1
Other settings1

Early / localised

SubgroupSettingApproachProducts and trialsEvidence
All comersLocalised, secretingAlpha-blockade (phenoxybenzamine or doxazosin) for 7 to 14 days, volume expansion, then laparoscopic or open adrenalectomy; cortical-sparing surgery in hereditary bilateral disease.94

Advanced, first line

SubgroupSettingApproachProducts and trialsEvidence
All comersMetastatic or unresectableBelzutifan (FDA May 2025, LITESPARK-015); 177Lu-DOTATATE for SSTR-positive disease; 131I-MIBG where available; sunitinib (FIRSTMAPPP); CVD or temozolomide chemotherapy for rapidly progressive or SDHB-mutant disease; alpha-blockade throughout.94

Other settings

SubgroupSettingApproachProducts and trialsEvidence
All comersAll patientsGermline genetic testing and, for carriers, lifelong biochemical and imaging surveillance; cascade testing of relatives.94

Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.