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Small cell carcinoma of the bladder


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Other Names:
Small cell bladder cancer; Small cell bladder carcinoma; Small cell carcinoma of the urinary bladder; Small cell bladder cancer; Small cell bladder carcinoma; Small cell carcinoma of the urinary bladder; Poorly differentiated neuroendocrine carcinoma of the bladder; SCCB See More
Categories:
Endocrine Diseases; Kidney and Urinary Diseases; Rare Cancers
This disease is grouped under:
Bladder cancer

Summary Summary


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The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
orphanet

Orpha Number: 284400

Definition
Small cell carcinoma of the bladder (SCCB) is a very rare, poorly differentiated neuroendocrine epithelial bladder tumor characterized clinically by hematuria and/or dysuria and a highly aggressive course.

Epidemiology
SCCB is extremely rare with an annual incidence of less than 1-9/1,000,000. Since 1980, fewer than 1,500 cases have been identified. The demographic profile of SCCB is similar to that of patients with bladder transitional cell carcinoma (TCC). The majority of patients are male, with a mean sex ratio of 5:1, and a range between 1:1 to 16:1. The vast majority of cases were reported in the Caucasian population.

Clinical description
Most knowledge on SCCB is based on retrospective investigations and on a few prospective studies. Mean age at diagnosis is 67 years (range 32 to 91 years). The clinical features of SCCB are similar to those of bladder TCC and reflect the presence of a tumoral mass. The main symptom is gross hematuria (63 to 88% of cases) with dysuria as the second most common symptom. Urinary obstruction, abdominal pain, urinary tract infection and weigh loss are occasionally present. SCCB often occurs with other types of carcinoma and is frequently found combined with other histological forms of bladder cancer: TCC, adenocarcinoma and squamous cell carcinoma. SCCB is generally believed to have a high metastatic potential. Rare cases of paraneoplastic syndromes such as ectopic ACTH secretion and hypercalcemia have also been reported.

Etiology
The etiology of the disease is unknown but a multipotent stem cell anomaly is thought to be a likely mechanism. A history of smoking is found in 65% to 79% of cases.

Diagnostic methods
Histology and immunohistochemistry show a tumor that is indistinguishable from small cell lung cancer (SCLC; see this term). Diagnosis of SCCB relies mainly on histopathological data obtained by cystoscopy and transurethral resection of the bladder tumor. Immunochemistry staining is useful in establishing the diagnosis. Light microscopy reveals packed cells having scant cytoplasm containing few organelles. Tumors are composed of nests of small round malignant cells with pyknotic round to oval nuclei and evenly dispersed ''salt and pepper chromatin''. More than 95% of SCCB cases are diagnosed at muscle invasive stage T2 or later.

Differential diagnosis
The differential diagnosis includes direct invasion of the bladder by SCC of the prostate, metastatic SCC from another source, usually the lung, and primary lymphomas of the bladder.

Management and treatment
The staging system used is the TNM-staging of bladder transitional cell carcinoma. Treatment is extrapolated from that of SCLC. However, many patients with SCCB undergo radical resection which is rarely performed in SCLC. Patients with surgically resectable disease should be managed with multimodal therapy associating chemotherapy, surgery and/or radiotherapy. Neoadjuvant chemotherapy using 4 chemotherapy cycles followed by radical cystectomy is the most effective therapeutic sequence. Patients with unresectable disease should be managed with palliative chemotherapy based on neuroendocrine-type regimens comprising a platinum drug (cisplatin in fit patients). Prophylactic cranial irradiation should be considered in stages III/IV bladder SCC.

Prognosis
The prognosis of the disease is poor mainly in the case of pure small cell carcinoma.

Visit the Orphanet disease page for more resources.
Last updated: 4/1/2012

Symptoms Symptoms


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This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 6 |
Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Bladder neoplasm 0009725
Hematuria
Blood in urine
0000790
30%-79% of people have these symptoms
Dysuria
Painful or difficult urination
0100518
5%-29% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ]
0002027
Hypercalcemia
High blood calcium levels
Increased calcium in blood
[ more ]
0003072
Recurrent urinary tract infections
Frequent urinary tract infections
Repeated bladder infections
Repeated urinary tract infections
Urinary tract infections
Urinary tract infections, recurrent
[ more ]
0000010
Showing of 6 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 1/1/2021
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Research Research


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Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Small cell carcinoma of the bladder. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations


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Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • Bladder Cancer Advocacy Network
    4915 St. Elmo Avenue, Suite 202
    Bethesda, MD 20814
    Toll-free: 888-901-BCAN
    Telephone: 301-215-9099
    E-mail: info@bcan.org
    Website: http://www.bcan.org/
Do you know of an organization? We want to hear from you.

Learn More Learn More


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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Small cell carcinoma of the bladder. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers


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Have a question? Contact a GARD Information Specialist.
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