urachal carcinoma


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  • Criteria

    • location in dome or anterior wall
    • primary in the bladder wall
    • lack cystitis cystica et glandularis beyond dome or anterior wall
    • rule out metastasis
  • Histology

    • adenocarcinoma (vast majority)
      • mucinous
      • enteric
      • signet ring cell
      • mixed
      • NOS
    • other urachal tumors
      • adenoma: villous adenoma, mucinous cystadenoma
      • urothelial, squamous, neuroendocrine, mixed
    • association with urachal remnant (not required for dx)
    • may extend to umbilicus
  • Sheldon staging

    • I: mucosa confined
    • II: invasive, but confined to urachus
    • III: local extension
      • IIIA: bladder muscle
      • IIIB: abdominal wall
      • IIIC: peritoneum
      • IIID: other viscera
    • IV: metastasis
        • IVA: regional lymph nodes
        • IVB: distant sites
  • Molecular

    • mutually exclusive: microsatellite instability (40%), KRAS (40%)