Pathology · Female Genital and Breast Pathology

Endometrial carcinoma is now classified by The Cancer Genome Atlas (TCGA) into four molecular subgroups. Which molecular subgroup has the BEST prognosis despite often showing high-grade morphology?

  • A MSI-high (microsatellite instability-high) hypermutated group
  • B POLE (polymerase epsilon) ultramutated group
  • C Copy number-low (CN-low), TP53 wildtype group
  • D Copy number-high (CN-high), TP53 mutated group (serous-like)
Correct answer: B. POLE (polymerase epsilon) ultramutated group

Explanation

The TCGA/ProMisE molecular classification of endometrial carcinoma identifies four prognostic groups: (1) POLE ultramutated (POLE exonuclease domain mutations, extremely high mutation burden >100 mut/Mb) — paradoxically best prognosis despite grade 3 morphology, due to high neoantigen load generating strong anti-tumor immunity; (2) MSI-H hypermutated (MLH1 promoter methylation or Lynch syndrome MMR deficiency) — intermediate-good prognosis, benefits from pembrolizumab; (3) CN-low/no specific molecular profile — intermediate prognosis, largely endometrioid; (4) CN-high/TP53 mutant (serous-like) — worst prognosis (equivalent to papillary serous carcinoma). Importantly, a POLE-ultramutated tumor classified as grade 3 endometrioid still has excellent prognosis and may not need adjuvant radiotherapy.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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