|Year : 2012 | Volume
| Issue : 2 | Page : 297-299
Primary amyloidosis with high grade transitional cell carcinoma of bladder: A rare case report
Prashant Gupta, Satish Hanamshetti, Jagdeesh N Kulkarni
Department of Urology, Bombay Hospital Institute of Medical Sciences, Mumbai, India
|Date of Web Publication||26-Jul-2012|
Room No. 717, 7th Floor, MRC Building, Bombay Hospital, 12 New Marine lines, Mumbai 400020
Primary amyloidosis of bladder is a rare disease that closely resembles bladder cancer on clinical presentation with painless gross hematuria. Pathologically this is a totally benign non-neoplastic lesion and its association with urothelial carcinoma of the bladder is rare. We herein report a 64-year-old diabetic male who has been treated for primary amyloidosis of bladder for the last 26 years presented recently with high-grade solid urothelial cancer with osseous metaplasia and sarcomatoid change of bladder with area of amyloid deposition.
Keywords: Amyloidosis, painless hematuria, urothelial cancer
|How to cite this article:|
Gupta P, Hanamshetti S, Kulkarni JN. Primary amyloidosis with high grade transitional cell carcinoma of bladder: A rare case report. J Can Res Ther 2012;8:297-9
|How to cite this URL:|
Gupta P, Hanamshetti S, Kulkarni JN. Primary amyloidosis with high grade transitional cell carcinoma of bladder: A rare case report. J Can Res Ther [serial online] 2012 [cited 2013 May 20];8:297-9. Available from: http://www.cancerjournal.net/text.asp?2012/8/2/297/98994
| > Introduction|| |
Amyloidosis is a heterogenous group of disorder which is benign, non-neoplastic characterized by extracellular deposition of fibrillar protein in tissue.  Clinically it can present as a primary or secondary disorder with systemic or localized manifestation. Primary localized amyloidosis of the urinary bladder presents with lower urinary tract symptoms (LUTS) and painless gross hematuria and mimics carcinoma of the bladder on imaging and cystoscopy. Generally the diagnosis is made on histological examination after specific staining with Congo red. Further it may coexist with malignancy.
| > Case Report|| |
Recently in April 2011, we saw a 64-year-old male patient with painless intermittent gross hematuria. For the last 26 years, he was treated at our institution for chronic primary amyloidosis of bladder with repeated trans urethral resections (TURBT) (six times, last in 2004) and each time the biopsy proved amyloidosis.
During the latest episode, complete evaluation showed normal hematocrit and biochemistry. Sonography showed multiple solid lesions on right lateral and posterior wall of bladder with right mild hydro-uretero nephrosis. On cystoscopy, there were multiple yellowish fat like deposits in the membranous urethra and right lateral wall of the bladder. Additionally multiple solid nodular lesions were seen in the bladder. Histology of the TURBT with deep muscle biopsy reported high-grade solid TCC [Figure 1]a with WHO grade III [Figure 1]b muscle invasion present, and [Figure 1]c focal osseous metaplasia with amyloidosis. CT scan showed multiple nodules with thick bladder with multiple focal calcification area and right side mild hydroureteronephrosis [Figure 1]d. In view of the above findings, he underwent radical cystectomy with ileal conduit. Cystectomy specimen showed multiple nodular areas of tumor with ulceration with whole thickened bladder [Figure 2]a. Microscopy confirmed sarcomatoid differentiation TCC [Figure 2]b with amyloidosis by the presence of apple green birefringence [Figure 2]c in the section stained with alkaline Congo red under a polarized light [Figure 2]d. Bilateral ureteric margins were positive for amyloid but negative for malignancy TCC. Final pathological staging was pT3b N1M0. At latest follow up, he is alive and well. He has been referred for adjuvant chemotherapy.
| > Discussion|| |
Historically amyloid term was coined by Virchow in 1854. Solonis first described bladder amyloidosis in 1897 at autopsy. Further amyloidosis of bladder with high-grade TCC is very rare  and review of literature shows only two previous reports till date. Amyloidosis of the bladder primarily affects posterior and posterior lateral walls with unknown etiology. It is mainly AL type Ig light chain. It is mainly present with gross painless hematuria and has clinical resemblance with bladder cancer.  Urinary dysfunction is found in 50% cases in patients due to amyloidotic neuropathy. It has very high rate of local recurrence. Cystoscopic appearance varies from solid circumscribed elevated sessile lesion to a grossly congested and ulcerated mucosa with petechial haemorrhage involving the whole bladder. Management of primary amyloidosis is mainly TURBT with symptomatic therapy if it is localized.  Adjuvant intravesical dimethyl sulfoxide therapy has been useful in selected patients with intractable hematuria.  However, follow up periodic cystoscopy is mandatory. Cystectomy is the option in some patients when the symptoms are life threatening.
Our case is unique because it exhibits long benign nature of amyloidosis which could be managed by repeated transurethral resections and hence cystectomy could be avoided for over two and a half decades. At each time, we tried to rule out coexisting urothelial malignancy to avoid pathological miss. However after long time (26 years), he did develop urothelial malignancy indicating the need for life long vigilant follow up, including meticulous biopsy of the lesions. It is safe to summarize that amyloidosis does not exhibit causal relationship with bladder cancer which may develop as an independent disease during the follow up. Further treatment of such a bladder cancer is not different from the other bladder cancers.
| > Acknowledgment|| |
We are highly thankful to Dr. D. D. Gaur for allowing us to operate on his patient and also to Dr. Mazumdar and Dr K. Patel of pathology department of our hospital for providing histopathology slides.
| > References|| |
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[Figure 1], [Figure 2]