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Olfactory neuroblastoma | Genetic and Rare Diseases Information Center(GARD) – an NCATS Program

Olfactory neuroblastoma | Genetic and Rare Diseases Information Center(GARD) – an NCATS Program

National Center for Advancing and Translational SciencesGenetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences



New on the MedlinePlus Nasal Cancer page:



Olfactory Neuroblastoma
09/28/2016 02:21 PM EDT

Source: Genetic and Rare Diseases Information Center - NIH



Other Names:
 
Esthesioneuroblastoma
Categories:
 
Olfactory neuroblastoma is a rare cancer of the upper part of the nasal cavity called the cribiform plate, which is a bone deep in the skull between the eyes, and above the ethmoid sinuses. It accounts for about 5% of all cancers of the nasal cavity and paranasal sinuses. It develops in nerve tissue associated with the sense of smell (olfactory nerve). It can occur at any age, but typically is found in adulthood.[1][2][3] Symptoms may be nonspecific and include a blockage of the nasal passageways due to the tumor, facial pain, runny nose, and nosebleeds. Treatment usually includes surgery to remove the tumor, radiation therapy, and sometimeschemotherapy.[2]
Last updated: 6/29/2016
The most common symptom of olfactory neuroblastoma is blockage of the nasal passageway secondary to the tumor. Other signs and symptoms may include:[2][4]
  • Loss of smell (anosmia) 
  • Chronic sinus infections (sinusitis)
  • Nasal bleeding
  • Nasal discharge
  • Pain
Some symptoms might be associated with a spread (metastasis) of the tumor to other structures in the face. These symptoms might include:[2][4]
  • Sinus pain and headache 
  • Visual changes
  • Ear pain 
Last updated: 6/29/2016
The cause of olfactory neuroblastoma is not well understood. No specific environmental or genetic causes have been confirmed, although comprehensive genetic testing of affected individuals has identified regions within chromosomes 2, 5, 6, 7, and 20 that may be involved.[3][4]
Last updated: 6/29/2016
A diagnosis of olfactory neuroblastoma may be suspected based on presenting signs and symptoms and imaging studies such as MRI and CT scans. The diagnosis is confirmed through biopsy of the tumor.[2]
Last updated: 6/29/2016
There are no standard guidelines for treatment of olfactory neuroblastoma. Treatment is dependent on the stage of the cancer. Most patients are initially treated with surgical removal of the tumor if possible. Radiation therapy is most commonly administered after surgery. The role of chemotherapy for olfactory neuroblastoma remains unclear. Although several studies have utilized chemotherapy, it is not clear whether it improves the long-term outlook (prognosis) compared to surgery and radiation therapy.[2][4]
Last updated: 6/29/2016
Do you have updated information on this condition? Let us know.
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 studying or have studied Olfactory neuroblastoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
    Nonprofit support and advocacy groups bring together patients, families, medical professionals, and researchers. These groups often raise awareness, provide support, and develop patient-centered information. Many are the driving force behind research for better treatments and possible cures. They can direct people to research, resources, and services. Many groups also have experts who serve as medical advisors. Visit their website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

    Organizations Providing General Support

      Do you know of an organization? Send us your suggestions.
      Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

      Financial Resources

        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

          In-Depth Information

          • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
          • 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 Olfactory neuroblastoma. Click on the link to view a sample search on this topic.

            NCATS Co-Sponsored Conferences

            • Overcoming Barriers to International Clinical Trials for Rare Cancers Friday, December 10, 2010 
              Location: Bethesda, Maryland
              Description: The goals of this conference were to introduce key institutional players to the topic of international clinical trials in rare cancers and to establish an ongoing dialogue. Participants left the meeting with a set of specific priorities that need to be enacted to promote these trials. The meeting promoted consensus on the way that resources are prioritized to address rare cancers. Participants were asked to convey the content of the meeting to their constituencies and to follow up with pilot concepts.
              Contact: Jack Welch, M.D., Ph.D., jack.welch@nih.govjack.welch@nih.gov
              Co-funding Institute(s): National Cancer Institute, Office of Rare Diseases Research
            Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question
            • I think I may have an olfactory neuroblastoma. Can you provide me with more information? See answer

            Have a question? Contact a GARD Information Specialist.
            1. Nasal Cavity and Paranasal Sinuses Cancer. American Cancer Society. 3/2/2015;http://www.cancer.org/cancer/nasalcavityandparanasalsinuscancer/detailedguide/nasal-cavity-and-paranasal-sinuses-cancer-what-is-nasal-paranasal.
            2. Carl Snyderman, Derrick Lin, Bruce E Brockstein, Robert Foote, Jerome B Taxy. Olfactory neuroblastoma (esthesioneuroblastoma). UpToDate. Jan 8, 2016; http://www.uptodate.com/contents/olfactory-neuroblastoma-esthesioneuroblastoma.
            3. Michael Somenek. Esthesioneuroblastoma. Medscape. April 14, 2015;http://emedicine.medscape.com/article/278047.
            4. Nalavenkata SB, Sacks R, Adappa ND, Palmer JN, Purkey MT, Feldman MD, Schlosser RJ, Snyderman CH, Wang EW, Woodworth BA, Smee R, Havas TE, Gallagher R, Harvey RJ. Olfactory Neuroblastoma: Fate of the Neck--A Long-term Multicenter Retrospective Study.. Otolaryngol Head Neck Surg.. December 15, 2015; 154(2):283-9. http://www.ncbi.nlm.nih.gov/pubmed/26671900.
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