Small cell carcinoma of the bladder. Report of five cases with immunohistochemistry and review of the literature with evaluation of prognosis according to stage

Abstract

Five cases of small cell carcinoma (SCC) of the bladder are reported with pathologic and immunohistochemical findings and clinical follow-up. Sixteen additional cases reported in the literature are studied and staged according to depth of tumor infiltration of the bladder wall. In our series of five cases and those reviewed from the literature, survival appeared to be dependent on stage of the tumor, analogous to the more common transitional cell carcinoma (TCC). We report two of five patients with the longest recorded follow-up times in the literature who are alive with no evidence of disease at 4 and 6 years. Contrary to the belief that SCC of the bladder is an aggressive, rapidly disseminating tumor similar to SCC of the lung, our findings support the notion that this is a potentially curable neoplasm with a prognosis that appears to be dependent on stage and surgical resectability.

Molecular Genetic Evidence for a Common Clonal Origin of Urinary Bladder Small Cell Carcinoma and Coexisting Urothelial Carcinoma

“…The first theory is that small-cell carcinomas originate from multipotential, undifferentiated cells or stem cells in the urothelium. 5,8,12,13 The frequent association of this tumor with coexisting urothelial carcinoma supports this theory. The second theory is that these tumors arise from neuroendocrine cells within normal or metaplastic urothelium.…”

“…Concordant genetic alterations and X chromosome inactivation between small-cell carcinoma and coexisting urothelial carcinoma suggest that both tumor compo- Small-cell carcinoma of the urinary bladder histologically resembles that occurring in the lung and has been reported with an increasing frequency in recent years. [1][2][3][4][5][6][7][8][9][10] It has been estimated to represent 0.5% of bladder malignancies and develops more frequently in older men, with hematuria as the most common presenting symptom. 8 Small-cell carcinoma of the urinary bladder behaves aggressively, often with locally advanced or metastatic disease at the time of presentation.…”

Primary Small Cell Carcinoma of the Urinary Bladder - Mini-review of the Literature

“…Surgical resection of urinary bladder in such setting has a dubious curative implication as majority of the patients present with metastasis either through bladder wall or pelvic lymph nodes at the time of the diagnosis even if not clinically evident. Hence, cystectomy is recommended only in patients with early stage disease where the tumor is localized to the bladder (Podesta et al, 1989;Lopez et al, 1994). Neoadjuvant or adjuvant combination chemotherapy with cystectomy is increasingly being practiced owing to high incidence of distant relapse following surgery for organ confined disease and due to good survival efficacy.…”

“…Other surgical modality acknowledged is partial cystectomy in combination with chemotherapy and/or radiation therapy. Podesta and True reported two cases of pT3 tumors that underwent partial cystectomy with adjuvant radiation therapy offered to one of the patient reported disease free at 78 months (Podesta et al, 1989). There is also mention of bladder sparing strategy of transurethral resection [TURBT] followed by chemo-RT being practiced but as reported in previous cases, the outcome following TURBT is very poor (Trias et al, 2001;Helpap et al, 2002).…”

Neuroendocrine carcinomas of the prostate and urinary bladder: a diagnostic and therapeutic challenge

“…Compared with all carcinomas of both organs, however, the frequency of high-grade NECs is very low, ranging from 0.2% to 1.0% [12,43,47,49,51,54,60,63]. The tumour usually affects patients who are older than 50 years and, in the case of bladder carcinoma, far more men than women.…”