Parathyroid carcinoma
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-PARATHYROID-CARCINOMA |
|---|---|
| Type | Disease |
| Aliases | CDC73-associated parathyroid carcinomaHRPT2-associated parathyroid carcinomaMEN1-associated parathyroid carcinoma (rare)Parathyroid cancerКарцинома прищитоподібної залози |
| Status | reviewed 2026-05-18 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-NCCN-NET-2025 SRC-NCCN-THYROID-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 | 8140/3 |
|---|---|
| icd_o_3_topography | ["C75.0"] |
| icd_10 | C75.0 |
| who_classification | WHO Endocrine and Neuroendocrine Tumours 5th ed (2022): Parathyroid carcinoma — diagnostic criteria require evidence of invasion (capsular / vascular / soft-tissue / lymph node / metastasis) and not just atypical histology |
Notes
Diagnosis is histologic-plus-invasive (capsular / vascular / soft- tissue invasion or metastasis) — atypical adenoma alone is not parathyroid carcinoma. Germline CDC73 / HRPT2 testing recommended at diagnosis for any parathyroid carcinoma (HPT-JT cascade). Treatment: en-bloc resection of parathyroid + ipsilateral thyroid lobe + central lymph nodes (R0 is the strongest prognostic factor; do NOT shell out the tumor at index surgery). Recurrent / metastatic disease: re- resection where feasible; calcimimetic (cinacalcet) for hypercalcemia control; bisphosphonates / denosumab for skeletal disease; pembrolizumab in MSI-H / TMB-high subset. No standard cytotoxic regimen with consistent activity.
Used By
Actionability
BMA-CDC73-GERMLINE-HPT-JT- CDC73 (HRPT2) germline pathogenic variants drive Hyperparathyroidism–Jaw Tumor (HPT-JT) s...
Algorithms
ALGO-MEN2-CARRIER-PROPHYLACTIC-THYROIDECTOMY-TIMING- ALGO-MEN2-CARRIER-PROPHYLACTIC-THYROIDECTOMY-TIMINGALGO-PARATHYROID-CARCINOMA-1L- ALGO-PARATHYROID-CARCINOMA-1L
Disease
DIS-PITUITARY-ADENOMA- Pituitary adenoma
Drug
DRUG-CINACALCET- Cinacalcet
Indication
IND-PARATHYROID-CARCINOMA-1L-RESECTION- IND-PARATHYROID-CARCINOMA-1L-RESECTIONIND-PARATHYROID-CARCINOMA-2L-CINACALCET- IND-PARATHYROID-CARCINOMA-2L-CINACALCETIND-PARATHYROID-CARCINOMA-2L-PEMBROLIZUMAB-MSI- IND-PARATHYROID-CARCINOMA-2L-PEMBROLIZUMAB-MSI
Red flag
RF-PARATHYROID-CARCINOMA-MSI-TMB-HIGH-ACTIONABILITY- MSI-high / dMMR OR TMB-high (≥10 mut/Mb) recurrent, persistent, or metastatic parathyroid...
Regimen
REG-CINACALCET-HYPERCALCEMIA- Cinacalcet for hypercalcemia of parathyroid carcinoma