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Medullary Schilddrüsenkarzinom (MTC)

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

IDDIS-MTC
ArtErkrankung
Aliase
MTCSchilddrüsenkrebsSchilddrüsenkarzinomМедулярна карцинома щитовидної залози
Statusreviewed 2026-04-27 | pending_clinical_signoff
ErkrankungenNone declared
QuellenSRC-NCCN-CNS-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Quelle: YAML

icd_o_3_morphology8345/3
icd_o_3_topography["C73"]
icd_10C73
who_classificationWHO Endokrine Tumore 5 ed (2022): Medullary Schilddrüsenkarzinom (parafollikulärer/C-Zell-Ursprung)

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Anmerkungen

Parafollicular C-cell origin — secretes calcitonin (diagnostic + monitoring biomarker) and CEA (parallel monitoring). RAI is NOT effective (C-cells do not concentrate iodide). Germline RET testing is mandatory at diagnosis — drives: - Prophylactic thyroidectomy timing in kindred (MEN2B: <1 yr; MEN2A C634: <5 yr per ATA risk classification). - Pheochromocytoma screening (plasma metanephrines / urinary catechols annually) — RET drives both MTC and adrenal medullary tumors. - Hyperparathyroidism screening in MEN2A. Therapy: - Localized: total thyroidectomy + central compartment LN dissection. - Advanced / unresectable (calcitonin doubling time <1 yr or structural progression): selpercatinib (LIBRETTO-001 / -531) or pralsetinib (ARROW) for RET-mutant MTC — superior to multikinase TKIs (vandetanib ZETA / cabozantinib EXAM remain alternatives, significant toxicity). - RET-WT (RAS-mutant or other): no targeted option; cabozantinib / vandetanib by default. Calcitonin doubling time <6 months portends very poor survival; <2 yr drives systemic-therapy decisions even without RECIST progression.

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