Metastatic pheochromocytoma and paraganglioma: lines of therapy

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6 standard-of-care settings across 4 lines and 1 biomarker subgroup. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

Line	All comers
Screening, prevention and diagnosis	1
Early / localised	1
Second line	2
Other settings	2

Screening, prevention and diagnosis

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	Staging	68Ga-DOTATATE PET, 123I-MIBG scintigraphy where 131I-MIBG is available, CT or MRI, FDG-PET for SDHB-related disease; germline testing.	Somatostatin receptor PET (68Ga/64Cu-DOTATATE)MIBG imaging and 131I-MIBG therapyFDG PETMRIGermline (hereditary) testing	Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors	94

Early / localised

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	Indolent disease	Active surveillance; resection, radiotherapy, thermal ablation or embolisation of dominant or symptomatic lesions.	Active surveillanceSBRT / SABR (stereotactic radiotherapy)Thermal ablation (RFA, microwave, cryo)IMRT / IGRT (modern external beam)	Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors	93

Second line

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	Progressive disease, radionuclide therapy	Lutetium-177 dotatate for somatostatin-receptor-positive disease; 131I-MIBG for MIBG-avid disease where still available (approved 2018, withdrawn from market 2024).	Lutetium-177 dotatatePeptide receptor radionuclide therapy (PRRT)Radioligand therapy (beta emitters)131I-MIBG (iobenguane I-131) therapyMIBG imaging and 131I-MIBG therapy	Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors	96
All comers	Progressive disease, systemic drugs	Belzutifan (approved 2025); sunitinib (FIRSTMAPPP); cabozantinib; cyclophosphamide, vincristine and dacarbazine or temozolomide for rapidly progressive or SDHB-related disease.	BelzutifanSunitinibCabozantinibCyclophosphamideVincristineTemozolomideSmall-molecule kinase inhibitorsCytotoxic chemotherapy	Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors	97

Other settings

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	All patients	Alpha-blockade with beta-blockade added second; metyrosine for refractory symptoms; blockade before every procedure; bone-protective agents for skeletal metastases.	Adrenalectomy	Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors	-
All comers	Trials	ONC206, radioligand combinations and next-generation HIF-2 alpha inhibitors.	Study of ONC206 (JZP3507) in Advanced Pheochromocytoma and ParagangliomaBelzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL	Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors	33

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.