Rare Gastroenterology News

Disease Profile

Urachal cancer

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Urachal carcinoma

Categories

Rare Cancers

Summary

Urachal cancer is a type of bladder cancer, making up less than 1% of all bladder cancers.[1] The urachus is a structure normally only present during development in the womb that connects the bellybutton and the bladder. This connection normally disappears before birth, but in some people remains.[2][1] Urachal cancers are usually diagnosed in adults in their 50's and 60's and may develop at the dome or anterior wall of the bladder, along the midline of the body (including the belly button), and between the pubis symphasis and the bladder.[3]

Most urachal cancers are adenocarcinomas (cancers that develop from gland cells). Others may be sarcomas (which develop from connective tissue - such as leiomyosarcoma, rhabdomyosarcoma, and malignant fibrous histiocytoma), small cell carcinomas, transitional cell cancer, and mixed neoplasias. Most individuals with urachal cancer have symptoms of with hematuria (blood in urine). Other symptoms may include abdominal pain, a palpable abdominal mass, mucinuria, and bacteriuria. Treatment usually involved surgery to remove the cancer.[1][3]

Treatment

Treatment for urachal cancer usually involves surgery to remove affected areas of the urachus, umbilicus (belly button), surrounding tissue, and all or part of the bladder (cystectomy). The role of chemotherapy and radiation therapy for the treatment of urachal cancer is unclear, although some studies show that chemotherapy can be useful in instances where the cancer spreads (metastasis) to other areas of the body and when the cancer returns after treatment (recurrence).[3][4]

Organizations

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

    Organizations Providing General Support

      Learn more

      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.

      Where to Start

      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.

        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.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Urachal cancer. Click on the link to view a sample search on this topic.

          References

          1. Molina JR, Quevedo JF, Furth AF, Richardson RL, Zincke H, Burch PA. Predictors of survival from urachal cancer. Cancer. December 1, 2007; 110(11):2434-40. https://www.ncbi.nlm.nih.gov/pubmed/17932892.
          2. Urachal Abnormalities. Urology Care Foundation. https://www.urologyhealth.org/urology/index.cfm?article=41. Accessed 4/11/2017.
          3. Behrendt MA, van Rhijn BWG. Genetics and biological markers in urachal cancer. Transl Androl Urol. October 2016; 5(5):655-661. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5071183/.
          4. Kim IK, Lee JY, Kwon JK et al. Prognostic Factors for Urachal Cancer: A Bayesian Model-Averaging Approach. Korean J Urol. September 2014; 55(9):574-580. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4165919/.
          5. Abel EJ, Heckman JE, Downs T. Partial Cystectomy. Medscape. October 3. 2016; https://emedicine.medscape.com/article/446101.

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