Urachal Adenocarcinoma



Urachal Adenocarcinoma


Jesse K. McKenney, MD










This specimen includes a partial cystectomy with ulcerative tumor image, the urachal tract image, and umbilectomy image. This is the typical surgery required for urachal carcinomas.






Mucinous adenocarcinoma may arise within urachal remnants at the dome of the bladder. Correlation with clinical/imaging evaluation is critical to establish a urachal origin.


TERMINOLOGY


Definitions



  • Primary carcinoma of any morphologic subtype originating from urachus and fulfilling following criteria



    • Tumor primarily located in dome of bladder


    • Epicenter of mass is in wall (muscularis propria) of bladder


    • Absence of surface intestinal metaplasia or precursor lesions


    • Absence of adenocarcinoma elsewhere


ETIOLOGY/PATHOGENESIS


Developmental Anomaly



  • Urachal remnants



    • May undergo malignant transformation


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare


  • Age



    • 5th and 6th decades


  • Gender



    • More common in men (M:F = 2:1)


Site



  • Dome of bladder



    • Anatomic location of urachus



      • Refining feature of urachal origin


  • Intramural bladder


Presentation



  • Hematuria is most common symptom


  • Irritative bladder symptoms, such as voiding difficulties


  • Mass lesion


  • Mucusuria seen rarely


Treatment



  • Partial or radical cystectomy, usually with umbilectomy, is treatment of choice


  • Adjuvant therapy depends on stage



    • Chemotherapy


    • Radiotherapy


Prognosis



  • Poor prognosis



    • Usually diagnosed at advanced stage


    • 5-year survival rate reported from 25-61%


IMAGE FINDINGS


CT Findings



  • Thickened bladder dome



    • May extend along urachus to umbilicus


MACROSCOPIC FEATURES


General Features



  • Mucosa is intact in early stages



    • Becomes ulcerated as tumor grows endophytically


  • Mass localized to dome of bladder


MICROSCOPIC PATHOLOGY

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Urachal Adenocarcinoma

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