Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127): lines of therapy
6 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.
Screening, prevention and diagnosis
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Genetic diagnosis | Germline panel testing offered to every patient; SDHB immunohistochemistry on tumour tissue; cascade testing of relatives with genetic counselling. | Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors | 94 | |
| All comers | Biochemical and imaging work-up | Plasma or urinary metanephrines; CT or MRI; 68Ga-DOTATATE PET as the preferred functional scan for SDHx and other cluster 1 disease. | Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors | 89 | |
| All comers | Surveillance of carriers | Annual metanephrines and clinical review from childhood, with whole-body MRI every two to three years in SDHB and SDHD carriers; screening for associated tumours (GIST, renal cell carcinoma, pituitary). | Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors | 94 |
Advanced, first line
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Advanced disease in carriers | Belzutifan (approved for VHL-associated tumours 2021 and for advanced pheochromocytoma and paraganglioma 2025); lutetium-177 dotatate for somatostatin-receptor-positive disease; see the metastatic record. | Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors | 92 |
Other settings
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Adrenal tumours in VHL and MEN2 | Alpha-blockade then cortical-sparing (partial) adrenalectomy to preserve adrenal function given the risk of bilateral disease. | Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors | 40 | |
| All comers | Head and neck paragangliomas | Observation for small asymptomatic tumours; surgery or fractionated or stereotactic radiotherapy when growing or symptomatic, weighing cranial nerve risk. | Endocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors | 93 |
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.