Cystitis Cystica and Glandularis

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Brief review about Cystitis Cystica and Glandularis. Is it a benign of a malignant condition?

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  • To understand this disorder well, we need to know where it comes from
  • One of the accepted theory is the metaplastic theory. It starts with formation of Von Brunn’s nests which are proliferation of transitional cell foci
  • Central degeneration of these foci leads to formation of what is called cystitis cystica
  • More degeneration in the centerwith metaplasia of the epithelial cells into a cuboidal/columnar phenotype produces cystitis glandularis
  • Presence of goblet cells is consistent with intestinal metaplasiaAnother theory says that cystitis glandularis originate in the fetal life from displaced intestinal epithelium during separation of the rectum fromthe urogenital sinus, some note that it is inadequate because it’s present in renal pelvis.
  • Why I’m saying symptomatic cystitis glandularis.
  • Long-term indwelling catheter especially in patients with spinal injury.
  • The most common presentation:What are the symptoms
  • This is an IVP of an AIDS patient with severe cystitis cystica and glandularis with bilateral obstruction.
  • The big question here is it a benign lesion or a precursor to bladder tumor should we follow it up?
  • .
  • Which might be linked to his history of upper tract disease rather than cystitis glandularis
  • In my view, Larger series of ….
  • Cystitis Cystica and Glandularis

    1. 1. Mahmoud Alameddine, MBBS Urology Associate Consultant International Medical Center
    2. 2.  Cystitis cystica and glandularis is a rare proliferative disease of mucus-producing glands within the mucosa and submucosa of the bladder epithelium.  It is characterized by foci of transitional cells that underwent glandular metaplasia.
    3. 3. Von Brunn’s Nests Submucocal formation and proliferation of transitional cells foci
    4. 4. Cystitis Cystica: Central degeneration of the epithelial foci
    5. 5. Cystitis Glandularis: More degeneration in the center with metaplasia of the epithelial cells into a cuboidal/columnar cells Cuboidal/ Columnar Cells
    6. 6. Intestinal Metaplasia Goblet cells
    7. 7.  The prevalence of symptomatic cystitis glandularis with a gross lesion in the US is 0.9–1.9%.¹  Autopsy series that looked for von Brunn nests, cystitis cystica and cystitis glandularis in asymptomatic people, found that 50–100% of samples have these histologic changes with no gross lesion²  Which led to the belief that cystitis cystica and glandularis may be an incidental histologic finding rather than a precursor to bladder cancer. 1. Lin JI et al. (1980) Diffuse cystitis glandularis. Associated with adeno- carcinomatous change. Urology 15: 411–415 2. Andersen JA and Hansen BF (1972) The incidence of cell nests, cystitis cystica and cystitis glandularis in the lower urinary tract revealed by autopsies. J Urol :–
    8. 8.  Chronic bladder inflammation caused by:  Stones  Bladder outflow obstruction  Long-term indwelling catheter i.e spinal injury  Pelvic Lipomatosis:  Rare proliferative disorder that causes increase deposition of fat around the bladder, rectum and prostate.  Cystitis glandularis is found in 75% of those patients¹  No clear risk factors. 1. Heyns CF et al. (1991) Pelvic lipomatosis associated with cystitis glandularis and adenocarcinoma of the bladder. J Urol 145: 364–366
    9. 9. CT scan view of Pelvic Lipomatosis: Pear shape bladder and deposition of fat around the bladder and rectum.
    10. 10.  Venous and lymphatic stasis due to perivesical fat compression and chronic inflammation.  Leads to mucosal edema and proliferation of blood vessels within the stroma.  The damaged epithelium sloughs off and regenerates with hyperplasia and subsequent glandular metaplasia. Tong RSK et al. (2002) Pelvic lipomatosis associated with proliferative cystitis occurring in two brothers. Urology 59 (Suppl): 602
    11. 11.  Irritative voiding symptoms:  Frequency  Dysuria  Urgency  Bacteruria  Gross hematuria  Chronic UTI  They may complain of voiding mucus  Less frequently: ureteral obstruction and subsequently hydronephrosis.
    12. 12. Coelho RF et al Cystoprostatectomy with ileal neobladder for treatment of severe cystitis glandularis in an AIDS patient. Clinics (Sao Paulo). 2008 Oct;63(5):713-6.
    13. 13.  A gross appearance looks like cobblestone pattern.  The bladder neck and trigone are the areas most frequently involved.  Followed by the lateral walls and the dome of the bladder.
    14. 14. It shows a cobblestone appearance of the mucosa with a focal polypoid mass cystitis glandularis forming a mass with blueberry spots in the urinary bladder.¹ 1. Shigehara K et al. Cystitis glandularis forming a tumorous lesion in the urinary bladder: A rare appearance of disease. Indian J Urol (2008)
    15. 15.  The first step is to treat the source of chronic bladder irritation if it is present i.e. treat UTI and stones, replace indwelling catheters with CIC.  Transurethral resection of bladder lesions.  Intravesical steroids injection.  Nephrostomy tubes may be initially necessary for severe ureteral obstruction before definitive therapy.  Bladder augmentation and cystectomy have been described in severe intractable cases who fail initial therapy and progressed into bladder contracture and renal failure.
    16. 16.  Squamous metaplasia  Fibroepithelial Polyps  GU Tuberculousis  Transitional cell carcinoma  Squamous cell carcinoma  Adenocarcinoma  Metastatic disease
    17. 17.  The first reported study implicating Cystitis Cystica and Glandularis in the development of adenocarcinoma was published in 1950.¹  In 1980, first report of bladder adenocarcinoma in a patient with Cystitis Cystica and Glandularis associated with pelvic lipomatosis.² 1. Immergut S, and Cottler ZR: Mucin producing adenocarcinoma of the bladder associated with cystitis follicularis and glandularis. Urol Cutaneous Rev 54: 531–534, 1950. 2. O’Brien AM, and Urbanski SJ: Papillary adenocarcinoma in situ of bladder. J Urol 134: 544–546, 1985.
    18. 18.  Since then, sporadic case reports have associated Cystitis Cystica and Glandularis with bladder cancer.  Around 16 case reports were published.  10 of them, the conclusions were based only on the synchronous presence of cancer with glandular metaplasia in the specimen. Smith et al. Role of Cystitis Cystica et Glandularis and Intestinal Metaplasia in Development of Bladder Carcinoma. UROLOGY :–
    19. 19.  In 2008 a study was published from Cleveland Clinic. They studied 88 patients with cystitis cystica and glandilaris and 15 patients with intestinal metaplasia who underwent interval follow up cystoscopies for a mean period of 4.4 years.  1 patient of the cystitis group developed bladder TCC, and this occurred 3 months after the initial biopsy. To be noted that this patient had a past history of upper tract TCC.  None of the intestinal metaplasia group had subsequent bladder cancer. Smith et al. Role of Cystitis Cystica et Glandularis and Intestinal Metaplasia in Development of Bladder Carcinoma. UROLOGY :–
    20. 20.  This case reveals the continued challenge to understand this rare disease and its clinical importance as a precursor to cancer.  It is clearly indicated that cystitis glandularis and intestinal metaplasia can be identified coincidentally with bladder cancer.  However, the long-term outcomes have not supported the role of these lesions as preneoplastic.  Larger series of patients with cystitis glandularis and intestinal metaplasia are needed to delineate the association between these lesions and bladder cancer.  Until then, interval cystoscopy might be of value and for an indefinite time.¹ 1. Lin JI et al. (1980) Diffuse cystitis glandularis. Associated with adenocarcinomatous change. Urology 15: 411–415

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