Urothelial carcinoma of the sarcomatoid variant in a young patient with spina bifida: a case report and review of the literature
© Nomikos et al; licensee BioMed Central Ltd. 2009
Received: 5 November 2009
Accepted: 22 December 2009
Published: 22 December 2009
Patients with neurogenic bladder due to spina bifida are considered to be at increased risk for aggressive bladder cancer. We present a unique case of a 32-year-old woman with spina bifida diagnosed with sarcomatoid urothelial carcinoma of the bladder and report diagnosis and management.
A 32 year old woman with neurogenic bladder managed with intermittent self catheterisations, presented with gross hematuria. On cystoscopy, she had a bulky bladder mass on the posterior bladder wall. Bladder biopsies revealed sarcomatoid variant of bladder transitional cell carcinoma. Treatment included radical cystectomy with ileal conduit diversion and adjuvant chemotherapy with excellent intermediate term follow up.
Patents with neurogenic bladder managed with intermittent self catheterisations need periodical follow up due to increased risk for aggressive bladder cancer. Immediate radical cystectomy with adjuvant chemotherapy is the suggested treatment approach.
Patients with neurogenic bladder due to spina bifida (SB) are considered to be at increased risk for bladder cancer. These tumours are composed of urothelial, glandular or small cell component showing variable degrees of differentiation, while a small subset may have a prominent myxoid stroma . We report a case of bladder cancer of the sarcomatoid variant in a young woman with neurogenic bladder due to SB, and we present the diagnostic evaluation, management and intermediate-term outcome.
Patients with neurogenic bladder are considered to be at high risk for bladder malignancies, with the vast majority of data coming from series of spinal cord injured patients . Less than 20 cases of patients with spina bifida (SB) and urothelial bladder cancer have been reported in the literature, mainly presenting with locally advanced or metastatic disease . Patients present with nonspecific symptoms, since gross hematuria was the main symptom in only 63% of cases. The median survival was only 6 months .
This is the first report of bladder urothelial carcinoma of the sarcomatoid variant diagnosed in a young patient with spina bifida.
Recent molecular studies support a monoclonal cell origin and suggest that clonal divergence may occur during tumor progression and differentiation .
Sarcomatoid carcinoma is a biphasic malignant neoplasm exhibiting morphologic and/or immunohistochemical evidence of epithelial and mesenchymal differentiation By immunohistochemistry, epithelial elements react with cytokeratins, whereas stromal elements react with Vimentin or specific markers corresponding to the mesenchymal differentiation. Microscopically, it is composed of spindle cell/sarcomatoid elements with high mitotic rate and atypical mitoses .
In conclusion, high index of clinical suspicion and appropriate usage of immunohistochemical techniques are essential for fast diagnosis of this rare clinical entity. Primary radical cystectomy and adjuvant chemotherapy may improve outcomes.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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