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MSH6 germline loss-of-function in crc produces dMMR/MSI-H phenotype. Pan-tumor MSI-H ICI...

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDBMA-MSH6-GERMLINE-CRC
TypeActionability
Statusreviewed 2026-04-27 | pending_clinical_signoff | actionability review required
DiseasesDIS-CRC
SourcesSRC-CIVIC SRC-ESMO-COLON-2024 SRC-NCCN-COLON-2025

Actionability Facts

BiomarkerBIO-DMMR-IHC
VariantMSH6 germline loss-of-function (dMMR / MSI-H)
DiseaseDIS-CRC
ESCAT tierIA
Recommended combinationspembrolizumab monotherapy (1L mCRC dMMR, KEYNOTE-177), dostarlimab (neoadjuvant rectal dMMR, off-label), nivolumab + ipilimumab (2L+ mCRC dMMR, CheckMate-142)
Contraindicated monotherapyFOLFOX/FOLFIRI 1L in dMMR mCRC (inferior to ICI), adjuvant 5-FU monotherapy in stage II dMMR (no benefit, possibly harmful)
Evidence summaryMSH6 germline loss-of-function in crc produces dMMR/MSI-H phenotype. Pan-tumor MSI-H ICI eligibility (pembrolizumab per KEYNOTE-158, dostarlimab per GARNET) supersedes tumor-specific lines via tissue-agnostic FDA approval. 1L mCRC: pembrolizumab monotherapy (KEYNOTE-177) — mPFS 16.5 vs 8.2 mo vs chemo. Adjuvant ICI in stage III dMMR CRC under investigation; deferral of adjuvant 5-FU appropriate in stage II dMMR (chemoinsensitive). Neoadjuvant dostarlimab in dMMR rectal cancer → 100% cCR (Cercek 2022).

Notes

Germline MSH6 loss → cascade testing mandatory (Lynch syndrome). Family meets Amsterdam/Bethesda criteria. Pan-tumor MSI-H ICI eligibility supersedes tumor- specific lines.

Used By

No reverse references found in the YAML corpus.