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Mutations in papillary thyroid carcinoma


Category

Subtype/Details

Genetic Alterations

Notes / Associations

Histologic Subtype Associations

Tall Cell PTC

- BRAF p.V600E (80%)


- TERT promoter mutations

High prevalence of BRAF p.V600E; some cases show TERT mutations


Follicular Variant PTC

- RAS mutations


- RET, NTRK, ALK fusions

RAS mutations common; presence of various gene fusions


Oncocytic PTC

- BRAF mutations


- GRIM-19


- RET rearrangements

Diverse genetic profile including BRAF and RET rearrangements


Warthin-like PTC

- BRAF p.V600E

Predominantly associated with BRAF p.V600E

Radiation-Associated PTC

Solid/Trabecular PTC

- NCOA4::RET rearrangements

Common in radiation-associated cases


Non-Radiation-Associated Cases

- CCDC6::RET


- NCOA4::RET


- ETV6::NTRK3 fusions

Diverse fusions; RET fusions linked to radiation exposure




RET fusions strongly associated with radiation exposure (e.g., Chernobyl)

Other Genetic Alterations


- CTNNB1 mutations


- p53 mutations


- Chromosome 1 LOH

CTNNB1 in fibromatosis-like variants; p53 and LOH in Tall Cell PTC

TERT Promoter Mutations


- C228T (NC_000005.9:g.1295228G>A)


- C250T (NC_000005.9:g.1295250G>A)

Found in ~10% of PTC; associated with poor prognosis and often coexists with BRAF p.V600E

Less Common Mutations & Fusions


- Mutations: HRAS, KRAS, PPM1D, CHEK2, MEK1


- Fusions: BRAF, PPARG, THADA, LTK, MET, FGFR2


- NTRK1, NTRK3 fusions (3-5%)

NTRK fusions are rare but actionable targets for therapy

RET Fusions


- CCDC6::RET (60-70% in sporadic PTC)


- NCOA4::RET (20-30%)

Common in sporadic and radiation-induced PTC; more prevalent in pediatric cases

Copy Number, Gene Expression, & microRNA


- Various alterations reported

Contribute to PTC tumorigenesis but are less well understood

Familial PTC

Syndromic

- APC mutations (e.g., Familial Adenomatous Polyposis - FAP)

Associated with syndromic forms of PTC


Non-Syndromic

- Genetic basis poorly understood

Genetic predisposition remains an area of ongoing research



  • RAS mutations & rare BRAF p.K601E seen in follicular patterned tumors | | Histologic Subtype Associations | - Tall cell PTC: High BRAF p.V600E (80%), some cases show TERT promoter mutations

  • Follicular variant PTC: RAS mutations, some show RET, NTRK, ALK fusions

  • Oncocytic PTC: Can have BRAF mutations, GRIM-19, and RET rearrangements

  • Warthin-like PTC: BRAF p.V600E | Different mutations correlate with histologic subtypes and aggressiveness | | Radiation-Associated PTC | - Solid/trabecular PTC: Commonly harbors NCOA4::RET rearrangements

  • Non-radiation-associated cases: CCDC6::RET, NCOA4::RET, ETV6::NTRK3 fusions | RET fusions are strongly associated with radiation exposure (e.g., Chernobyl-associated PTC) | | Other Genetic Alterations | - CTNNB1 mutations (spindle cells in PTC with fibromatosis-like stroma)

  • p53 mutations, Chromosome 1 LOH (Tall Cell PTC) | CTNNB1 mutations seen in rare fibromatosis-like variants | | TERT Promoter Mutations | - C228T (NC_000005.9:g.1295228G>A)

  • C250T (NC_000005.9:g.1295250G>A) | Found in ~10% of PTC

  • Associated with poor prognosis and aggressive behavior

  • Often coexists with BRAF p.V600E | | Less Common Mutations & Fusions | - Mutations in HRAS, KRAS, PPM1D, CHEK2, MEK1

  • Fusions in BRAF, PPARG, THADA, LTK, MET, FGFR2

  • NTRK fusions (NTRK1, NTRK3) (3-5%) | NTRK fusions are rare but actionable targets for therapy | | RET Fusions | - CCDC6::RET (most common in sporadic PTC, 60-70%)

  • NCOA4::RET (20-30%)

  • Common in radiation-induced PTC | RET rearrangements are more common in pediatric and radiation-associated cases | | Copy Number, Gene Expression, & microRNA | Alterations reported | Contribute to PTC tumorigenesis but less well understood | | Familial PTC | - Syndromic: APC mutations (e.g., FAP)

  • Non-syndromic: Poorly understood genetic basis | Genetic predisposition remains an area of ongoing research |

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