Plasma free metanephrines are the gold-standard biochemical test for pheochromocytoma and...
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| ID | BMA-METANEPHRINES-PHEO-VHL-MEN2-SURVEILLANCE |
|---|---|
| Type | Pertinence thérapeutique |
| État | reviewed 2026-05-18 | actionability review required |
| Maladies | DIS-PHEOCHROMOCYTOMA |
| Sources | SRC-ENDOCRINE-SOCIETY-MEN-2023 SRC-NCCN-KIDNEY-2025 SRC-NCCN-NET-2025 |
Faits relatifs à l ' action en justice
| Biomarqueur | BIO-METANEPHRINES-PLASMA-PHEO |
|---|---|
| Variante | Plasma free metanephrines — pheochromocytoma/paraganglioma surveillance in VHL / MEN2 / SDH carriers |
| Maladie | DIS-PHEOCHROMOCYTOMA |
| Niveau ESCAT | IIIA |
| Combinaisons recommandées | Élévation confirmée du porteur syndromique : IRM abdomen + 68Ga-DOTATATE PET → adrénalectomie (épargnant cortical si risque bilatéral) ou résection paragangliomale, Equivocal : test de suppression de la clonidine OU 24 h de métanephrines fractionnées urinaires confirment |
| Contre-indiqué en monothérapie | Do not initiate beta-blockade for PPGL hypertension before adequate alpha-blockade (phenoxybenzamine 10-14 days) — risk of hypertensive crisis from unopposed alpha stimulation |
| Résumé des faits | Plasma free metanephrines are the gold-standard biochemical test for pheochromocytoma and paraganglioma (PPGL) — sensitivity ~96-99%, specificity ~85-95% (lower with sympathomimetic / tricyclic / acetaminophen interferents and improper supine sampling). Used for annual lifelong surveillance in confirmed carriers of VHL (Type 2A/2B/2C), MEN2A/MEN2B (RET-codon-stratified onset), and SDHB/SDHC/SDHD/SDHA per Endocrine Society MEN 2023 and NCCN Kidney 2025 (VHL component). Elevation >3-4× upper limit highly suggestive of PPGL; intermediate elevation prompts clonidine-suppression test or 24-hour urinary fractionated metanephrines. Positive biochemistry triggers MRI abdomen + 68Ga-DOTATATE PET (or I-123 MIBG). Biochemistry directs adrenalectomy / paraganglioma resection planning. ESCAT IIIA — biomarker directs curative-intent surgery scheduling in syndromic carriers. |
Notes
STUB pending two-Co-Lead signoff. SDH (B/C/D/A) carrier surveillance Indication entities are pending authoring (closest existing: none yet for SDH; future IND-SDH-CARRIER-SURVEILLANCE). VHL classification (Type 1 vs Type 2A/2B/2C) determines pheo surveillance need — Type 1 lacks pheochromocytoma, Type 2 has it as defining feature. Render layer should flag concurrent sympathomimetic / tricyclic / acetaminophen use, supine-sample status, and renal function — all affect interpretation.
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