Medullary thyroid carcinoma (MTC)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-MTC |
|---|---|
| Type | Disease |
| Aliases | MTCMedullary thyroid cancerMedullary thyroid carcinomaМедулярна карцинома щитовидної залози |
| Status | reviewed 2026-04-27 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-NCCN-CNS-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8345/3 |
|---|---|
| icd_o_3_topography | ["C73"] |
| icd_10 | C73 |
| who_classification | WHO Endocrine Tumours 5th ed (2022): Medullary thyroid carcinoma (parafollicular / C-cell origin) |
Notes
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.
Used By
Actionability
BMA-CALCITONIN-MTC-MEN2-SURVEILLANCE- Serum calcitonin is a highly specific biomarker for medullary thyroid carcinoma (MTC) — p...BMA-RET-C634R-MTC- RET C634R is the most common MEN2A germline variant. In advanced / metastatic MEN2A-drive...BMA-RET-M918T-MTC- RET M918T in medullary thyroid carcinoma (MTC): selpercatinib superior to cabozantinib/va...
Algorithms
ALGO-MEN2-CARRIER-PROPHYLACTIC-THYROIDECTOMY-TIMING- ALGO-MEN2-CARRIER-PROPHYLACTIC-THYROIDECTOMY-TIMINGALGO-MTC-1L- ALGO-MTC-1L
Disease
DIS-PARATHYROID-CARCINOMA- Parathyroid carcinomaDIS-PITUITARY-ADENOMA- Pituitary adenomaDIS-THYROID-PAPILLARY- Papillary thyroid carcinoma (PTC)
Indication
IND-MEN2-CARRIER-INTENSIFIED- IND-MEN2-CARRIER-INTENSIFIEDIND-MEN2-CARRIER-SURVEILLANCE- IND-MEN2-CARRIER-SURVEILLANCEIND-MEN2-SUSPICION-PREVENTION-ENHANCED-SURVEILLANCE- IND-MEN2-SUSPICION-PREVENTION-ENHANCED-SURVEILLANCEIND-MEN2-SUSPICION-PREVENTION-GENETIC-COUNSELING- IND-MEN2-SUSPICION-PREVENTION-GENETIC-COUNSELINGIND-MTC-ADVANCED-1L-CABOZANTINIB-RET-WT- IND-MTC-ADVANCED-1L-CABOZANTINIB-RET-WTIND-MTC-ADVANCED-1L-SELPERCATINIB- IND-MTC-ADVANCED-1L-SELPERCATINIB
Procedures
PROC-PROPHYLACTIC-THYROIDECTOMY-MEN2- Prophylactic total thyroidectomy in MEN2 / RET-mutation carriers
Questionnaires
QUEST-MTC-1L-STUB- Medullary thyroid carcinoma — first line
Red flag
RF-MEN2-CONFIRMED-CARRIER- Confirmed germline pathogenic / likely-pathogenic variant in the RET proto-oncogene — MEN...RF-MEN2-FAMILY-HISTORY-SUSPICION- Family pedigree suggestive of Multiple Endocrine Neoplasia type 2 (MEN2A or MEN2B, RET-dr...RF-MTC-FRAILTY-AGE- Age ≥75 with ECOG ≥2 or ≥2 comorbidities — vandetanib / cabozantinib multikinase inhibito...RF-MTC-HIGH-RISK-BIOLOGY- Medullary thyroid carcinoma with actionable RET mutation: germline RET M918T (MEN2B — mos...RF-MTC-INFECTION-SCREENING- Active or latent infection requiring resolution / prophylaxis before initiating long-dura...RF-MTC-ORGAN-DYSFUNCTION- Baseline organ dysfunction precluding standard vandetanib / cabozantinib multikinase inhi...RF-MTC-RET-MUTANT- RET proto-oncogene activating mutation (somatic or germline) in advanced or metastatic me...RF-MTC-TRANSFORMATION-PROGRESSION- MTC progression on first-line RET-selective inhibitor (acquired RET solvent-front mutatio...
Sources
SRC-NCCN-THYROID-2025- NCCN Clinical Practice Guidelines — Thyroid Carcinoma