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Drivers, passengers & the two-hit model

Of the thousands of mutations in a tumour, only a handful (typically 2-8) actually drive it. Drivers either jam an accelerator on (oncogenes, one hit is enough) or remove a brake (tumour suppressors, both copies must go). Everything else is a passenger along for the ride.

Vogelstein's landscape: ~140 driver genes, each tumour carrying 2-8 driver events in ~12 pathways, on a background of tens to thousands of passengers. Oncogene activation is dominant and recurrent at hotspots: point mutation (KRAS G12, BRAF V600E, PIK3CA H1047R), amplification (HER2, MYC, MDM2), fusion (BCR-ABL, ALK, RET, NTRK, EWSR1-FLI1), promoter mutation (TERT) or ecDNA. Tumour suppressor loss follows Knudson's two hits, one inherited in hereditary syndromes (RB1, BRCA1/2, APC, MLH1, TP53), the second by deletion, LOH, mutation or methylation; some are haploinsufficient (PTEN). Gatekeepers (APC, RB1) control proliferation directly; caretakers (BRCA, MMR) guard the genome so their loss accelerates all other hits. Oncogene addiction, the dependence of a tumour on its driver, is why kinase inhibitors work; passenger load creates neoantigens and, occasionally, collateral vulnerabilities (MTAP deletion next to CDKN2A → PRMT5 dependence).

In one picture

A car with a stuck accelerator (oncogene: one fault is enough) and cut brake lines (tumour suppressor: both lines must fail because they are duplicated). The scratches on the paintwork (passengers) did not cause the crash but they tell you what road it drove on.

Diagram

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Light up a product:
Proto-oncogeneMutation, amp, fusionOncogene ON (1 hit)Tumour suppressor1st hit (germline/somatic)2nd hit: LOH, methylationBrake lost (2 hits)Passengers, neoantigensClonal expansionactivatesinhibitsdruggable target (click)hit by selected productescape route

How drugs attack it

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  • Oncogene addiction is the basis of every targeted kinase inhibitor and of HER2 antibodies
  • Suppressor loss cannot be 'inhibited', so it is exploited indirectly: synthetic lethality (BRCA-PARP, MTAP-PRMT5), CDK4/6 for RB-intact, MDM2 for TP53-wild-type
  • Germline first hits drive surveillance and risk-reducing surgery in hereditary syndromes
  • Comprehensive genomic profiling separates drivers from passengers at diagnosis

Connected

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