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Plasmafreie Metanephrine (Phäochromozytom/Paragangliom-Überwachung — VHL/MEN2/SDH)

Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.

IDBIO-METANEPHRINES-PLASMA-PHEO
ArtBiomarker
Aliase
Plasmafreie Metanephrinefraktionierte PlasmametanephrinePlasmametanephrinPlasma-NormetanephrinМетанефрини плазми вільні (нагляд феохромоцитоми/парагангліоми — VHL/MEN2/SDH)
Statusreviewed 2026-05-18 | pending_clinical_signoff
ErkrankungenDIS-PHEOCHROMOCYTOMA
QuellenSRC-ENDOCRINE-SOCIETY-MEN-2023 SRC-NCCN-KIDNEY-2025 SRC-NCCN-NET-2025

Biomarker-Fakten

Biomarker-Typserum_marker
Messung
VerfahrenPlasma LC-MS/MS (vorzugsweise) oder Immunoassay; Patienten-Supin ≥ 30 min vor dem Ziehen
Einheitenpg/ml oder nmol/L (assayabhängige Bezugsbereiche; Metanephrin und Normetanephran, getrennt gemeldet)
Verwandte BiomarkerNone declared

Anmerkungen

Catecholamine-metabolite biomarker — gold-standard biochemical test for pheochromocytoma and paraganglioma (PPGL). Preferred for asymptomatic surveillance in syndromic carriers: VHL (annual, lifelong), MEN2A/MEN2B (annual after age 5-8 per RET codon stratification), SDHB/SDHC/SDHD/SDHA (annual, starting age 6-10 per Endocrine Society 2023). Sensitivity ~96-99% for PPGL when properly drawn; specificity ~85-95% (lower with drug/dietary interferents). VHL Type 1 lacks pheo; Type 2A/2B/2C have pheo as defining feature — surveillance schedule keyed to VHL phenotype classification (see BIO-VHL-CLASSIFICATION-PHENOTYPE). Elevation >3-4x upper limit highly suggestive; intermediate elevation prompts clonidine suppression test or 24-h urinary fractionated metanephrines as confirm. Imaging (MRI abdomen + I-123 MIBG or 68Ga-DOTATATE PET) when biochemistry positive. STUB pending two-Co-Lead signoff.

Verwendet von

Therapeutische Relevanz

Biomarker