Elsevier

Seminars in Oncology

Volume 34, Issue 1, February 2007, Pages 15-21
Seminars in Oncology

Extrapulmonary Small Cell Carcinoma of the Bladder

https://doi.org/10.1053/j.seminoncol.2006.10.025Get rights and content

Among the many sites for primary small cell cancer is the genitourinary system. The majority of cases have been observed in the bladder and prostate. Small cell carcinoma accounts for less than 1% of all bladder tumors. Definitive predisposing factors are unknown; however, small cell carcinoma of the bladder has been associated with cigarette smoking, long-standing cystitis, bladder calculus, and augmented cystoplasty. Contrary to the early theory of derivation from Kulchitsky cells, it is now believed that small cell carcinoma of the bladder originates from the totipotent stem cells present in the submucosa of the bladder wall. A number of chromosomal aberrations have been reported in small cell cancer of the bladder. There are no specific clinical features that differentiate these patients from transitional cell carcinoma of the bladder; however, some patients may have associated paraneoplastic conditions. Diagnosis is established by cystoscopic-assisted biopsy. Like small cell carcinoma of the lung, small cell carcinoma of the bladder has a propensity for early metastases. There is no standard therapy for small cell carcinoma of the bladder and the prognosis is poor; however, patients treated with cisplatin-based chemotherapy regimens seem to have a better prognosis.

Section snippets

Epidemiology

Although the true incidence of small cell cancer of the urinary bladder is unknown, many retrospective case series report an incidence between 0.3% and 1%.3, 5, 6, 17, 18, 19 In a retrospective review of 552 consecutive bladder cancer patients, only six patients had a diagnosis of small cell carcinoma.17 Similarly, Hind et al reported three patients with small cell carcinoma in 110 cystectomy specimens.19 In China, of 334 cases of bladder cases reviewed over a 6-year period, only two cases of

Histology

The origin and histogenesis of small cell cancer in the urinary bladder remains unclear. Many theories have been proposed. Nevertheless, one must account for the mixed histologic pattern that is commonly seen in these tumors. It was earlier believed that this neoplasm arises either from Kultchitsky type cells of the bladder wall or from a metaplastic mucosal epithelium; however, the most widely accepted view is that small cell carcinoma of the bladder arises from transformation of totipotent

Clinical Features

The majority of patients present with painless gross hematuria.1, 3, 6, 10, 17, 37 Less frequent symptoms include microscopic hematuria, passage of blood clots, dysuria, nocturia, hematospermia, bladder irritability, supra-pubic pain and cramps, diffuse abdominal pain, and obstruction of the ureteric orifice causing hydroureter and hydronephrosis, as well as bladder outlet obstruction producing urinary retention and acute renal failure.3, 6, 15, 20, 37, 39, 47, 48, 49, 50, 51 A case of small

Diagnosis

In the era before computed tomography (CT) scanning, gross hematuria was evaluated by intravenous pyelography (IVP).41 The presence of a filling defect in the urinary bladder was suggestive of bladder pathology.20, 26, 41, 56, 57, 58 Additional radiologic findings noted are calcifications in the tumor, nonvisualization of the ureter, ureteric obstruction, hydroureter, and hydronephrosis.17, 32, 52, 53, 59, 60, 61 Similar information was also obtained from a retrograde pyelogram.57 These

Staging

All new cases of bladder small cell cancer should be staged with chest, abdomen, and pelvic CT scans. Magnetic resonance imaging (MRI) of the brain and a bone scan may be reserved for specific situations.

CT of the chest is required to eliminate a primary pulmonary small cell carcinoma since bladder metastases from a lung primary have been observed.77, 78 CT of the pelvis or MRI of the pelvis delineates bladder tumors from surrounding structures and may help in planning surgical resection.50, 51

Treatment

There is no consensus on the standard approach to treat small cell carcinoma of the bladder. Due to the rarity of the disease, prospective clinical trials have not been performed; hence recommendations for managing these tumors have been extrapolated from individual case reports, retrospective reviews of case series, and treatment of small cell carcinoma of the lung. Nonetheless, it is apparent that without treatment, the disease is rapidly fatal.24, 26, 49

For early T-stage tumors, surgical

Prognosis

Despite early treatment, the majority of patients die of their disease.3, 83, 91 The prognosis is worse than poorly differentiated transitional cell carcinoma of the bladder.11 Fourteen of 18 patients had died at a median follow-up of 9 months.11 The over all median survival in the Mayo Clinic series was 1.7 years with recurrences in regional lymph nodes or distant disease.3 Few patients were alive beyond 5 years. Mangar et al reported only one survivor at 7 years after diagnosis among 14

References (91)

  • T.D. Jones et al.

    Thyroid transcription factor 1 expression in small cell carcinoma of the urinary bladder: An immunohistochemical profile of 44 cases

    Hum Pathol

    (2005)
  • N.B. Atkin et al.

    Chromosome abnormalities and p53 expression in a small cell carcinoma of the bladder

    Cancer Genet Cytogenet

    (1995)
  • D.M. Blunt et al.

    Imaging of small cell carcinoma of the male urogenital tract

    Clin Radiol

    (1996)
  • L.J. Wang et al.

    Small cell carcinoma of the bladder mimicking a gynecological tumor

    J Urol

    (2001)
  • F. Abbas et al.

    Small cell carcinoma of the bladder and prostate

    Urology

    (1995)
  • M.P. Davis et al.

    Successful management of small cell carcinoma of the bladder with cisplatin and etoposide

    J Urol

    (1989)
  • J.Y. Lee et al.

    Anaplastic carcinoma of urinary bladder with oat cell features

    Urology

    (1986)
  • D.L. Cheng et al.

    Successful treatment of metastatic small cell carcinoma of the bladder with methotrexate, vinblastine, doxorubicin and cisplatin therapy

    J Urol

    (1995)
  • J.C. Kim

    CT features of bladder small cell carcinoma

    Clin Imag

    (2004)
  • C. Cheng et al.

    Oat cell carcinoma of urinary bladder

    Urology

    (1992)
  • A.O. Siefker-Radtke et al.

    Evidence supporting preoperative chemotherapy for small cell carcinoma of the bladder: A retrospective review of the M. D. Anderson cancer experience

    J Urol

    (2004)
  • M. Amichetti et al.

    Undifferentiated small cell neuroendocrine carcinoma of the urinary bladder

    Eur J Cancer

    (1992)
  • J. Cassidy et al.

    An unusual case of haematuria

    Eur J Cancer

    (1993)
  • R. Kickuth et al.

    Magnetic resonance imaging of bone marrow metastasis with fluid-fluid levels from small cell neuroendocrine carcinoma of the urinary bladder

    Magn Reson Imag

    (2002)
  • S.F. Cramer et al.

    Neurosecretory granules in small cell invasive carcinoma of the urinary bladder

    Cancer

    (1981)
  • L. Koss

    Tumours of the Urinary Bladder

  • N.W. Choong et al.

    Small cell carcinoma of the urinary bladderThe Mayo Clinic experience

    Cancer

    (2005)
  • C. Lohrisch et al.

    Small cell carcinoma of the bladder: Long term outcome with integrated chemoradiation

    Cancer

    (1999)
  • I. Trias et al.

    Small cell carcinoma of the urinary bladderPresentation of 23 cases and review of 134 published cases

    Eur Urol

    (2001)
  • S.Z. Ali et al.

    Small cell neuroendocrine carcinoma of the urinary bladderA clinicopathologic study with emphasis on cytologic features

    Cancer

    (1997)
  • L. Cheng et al.

    Small cell carcinoma of the urinary bladder: A clinicopathologic analysis of 64 patients

    Cancer

    (2004)
  • C.E. Blomjous et al.

    Small cell carcinoma of the urinary bladderA clinicopathologic, morphometric, immunohistochemical, and ultrastructural study of 18 cases

    Cancer

    (1989)
  • D.J. Grignon et al.

    Small cell carcinoma of the urinary bladderA clinicopathologic analysis of 22 cases

    Cancer

    (1992)
  • B. Helpap

    Morphology and therapeutic strategies for neuroendocrine tumors of the genitourinary tract

    Cancer

    (2002)
  • G. Nabi et al.

    Primary small cell neuroendocrine carcinoma of urinary bladder: An uncommon entity to be recognized

    Int Urol Nephrol

    (2001)
  • J.I. Lopez et al.

    Small cell carcinoma of the urinary bladderA clinicopathological study of six cases

    Br J Urol

    (1994)
  • K.Y. Lam et al.

    Bladder tumours in Chinese: A 6 year study

    Aust NZ J Surg

    (1994)
  • A. Hind et al.

    Histopathological study of 110 cystectomy specimens for bladder cancer by an original mapping method

    J Exp Clin Cancer Res

    (1998)
  • D.J. Oblon et al.

    Bladder preservation and durable complete remission of small cell carcinoma of the bladder with systemic chemotherapy and adjuvant radiation therapy

    Cancer

    (1993)
  • R. Bastus et al.

    Small cell carcinoma of the urinary bladder treated with chemotherapy and radiotherapy: Results in five cases

    Eur Urol

    (1999)
  • T. McClellan et al.

    Small cell neuroendocrine carcinoma of the urinary bladder: Case report of a rare primary tumor

    West VA Med J

    (2001)
  • M. Bruyneel et al.

    Extrapulmonary small cell carcinoma following augmentation enterocystoplasty

    Acta Clin Belg

    (2004)
  • C.K. Kim et al.

    Small cell carcinoma of urinary bladderUltrastructural study

    Urology

    (1984)
  • J.W. Barrington et al.

    Tumors in bladder remnant after augmentation enterocystoplasty

    J Urol

    (1997)
  • J. Golomb et al.

    Primary small cell carcinoma of the augmented urinary bladder

    Br J Urol

    (1992)
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