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Disease Profile

Sinonasal undifferentiated carcinoma

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)

SNUC; Highly aggressive undifferentiated carcinoma of the nasal cavity and paranasal sinuses

Categories

Rare Cancers

Summary

Sinonasal undifferentiated carcinoma (SNUC) is a type of cancer that develops in the sinuses of the head. SNUC occurs when cells from the layer of tissue that lines the sinuses (called the epithelium) rapidly increase in number, forming a mass. The first symptoms of SNUC may include difficulty breathing though the nose or mild facial pain. As SNUC progresses, other symptoms may include nosebleeds, facial numbness, or tingling in the face. SNUC is an aggressive cancer, meaning it grows rapidly and has a tendency to spread into healthy tissues nearby and to more distant parts of the body (metastasis).[1][2] The cause of SNUC is still unknown.[3] When possible, the treatment is the complete removal of the tumor, combined with radiation therapy and chemotherapy. In cases where the tumor can't be removed completely, the treatment is with radiation and chemotherapy. The risk of the tumor coming back (relapse) is high and the probability of cure is modest.[4]

Cause

The exact cause of this tumor is still unknown. There are several risk factors that may increase the chances of developing it, such as:[5]

  • Occupational exposure: Breathing in certain substances while working, such as dust from the manufacturing of wood, textiles, leather, flour, and metals (including nickel and chromium). 
  • Being infected with human papillomavirus (HPV).
  • Being male and older than 40 years.
  • Smoking.

Treatment

Treatment is determined for each affected individual based on the size of the tumor and whether it has spread to other parts of the body.[1] Currently, it is believed that the best treatment for sinonasal undifferentiated carcinoma consists of multiple therapies, including surgery to remove as much of the tumor as possible, and chemotherapy and/or radiation therapy to destroy any cancer cells that may remain in the body.[1][6] If surgery can remove the tumor completely, and it is followed by chemotherapy and radiation therapy, the survival outcome is better. In cases where the tumor can't be completely removed by surgery, chemotherapy and radiotherapy are indicated.[7]

Other treatment options are currently being studied, such as boron neutron capture therapy, autologous bone marrow transplant, and neoadjuvant selective intra-arterial cisplatin with concurrent radiation therapy, which showed limited success. Other researches are investigating the use of TK-inhibitors targeting c-KIT (e.g. Imatinib/Gleevac).[8]

Follow-up treatment can involve dental prostheses, eye prostheses, and visits with dentists and ophthalmologists, in addition to regular follow-up with the original treating doctors for cancer follow-up. This follow-up consists of regular magnetic resonance imaging (MRI) and, if necessary, tissue biopsy.[9]

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 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

    • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

      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 Sinonasal undifferentiated carcinoma. Click on the link to view a sample search on this topic.

        References

        1. Schmidt ER & Berry RL. Diagnosis and treatment of sinonasal undifferentiated carcinoma: report of a case and review of the literature. Journal of oral and maxillofacial surgery. 2008; 66:1505-1510. https://www.ncbi.nlm.nih.gov/pubmed/18571040.
        2. Xu CC, Dziegielewski PT, McGaw WT & Seikaly H. Sinonasal Undifferentiated Carcinoma (SNUC): the Alberta experience and literature review. Journal of Otolaryngology Head & Neck Surgery. 2013; 42:2. https://journalotohns.biomedcentral.com/articles/10.1186/1916-0216-42-2.
        3. Sinonasal Undifferentiated Carcinoma. NORD. 20014; https://rarediseases.org/rare-diseases/sinonasal-undifferentiated-carcinoma/.
        4. Khan MN, Konuthula N, Parasher A, Genden EM, Miles BA, Govindaraj S & Iloreta AM. Treatment modalities in sinonasal undifferentiated carcinoma: an analysis from the national cancer database. Int Forum Allergy Rhinol. October 7 2016; https://www.ncbi.nlm.nih.gov/pubmed/27717204.
        5. American Cancer Society. Nasal Cavity and Paranasal Sinus Cancers. January 2013; https://www.cancer.org/acs/groups/cid/documents/webcontent/003123-pdf.pdf. Accessed 1/22/2013.
        6. Reiersen DA, Pahilan ME, Devaiah AK. Meta-analysis of treatment outcomes for sinonasal undifferentiated carcinoma. Otolaryngology head and neck surgery. 2012; 147:7-14. https://www.ncbi.nlm.nih.gov/pubmed/22460731.
        7. Kuo P, Manes RP, Schwam ZG & Judson BL. Survival Outcomes for Combined Modality Therapy for Sinonasal Undifferentiated Carcinoma. Otolaryngol Head Neck Surg. October 4, 2016; https://www.ncbi.nlm.nih.gov/pubmed/27703092.
        8. Xu CC, Dziegielewski PT, McGaw WT & Seikaly H. Sinonasal Undifferentiated Carcinoma (SNUC): the Alberta experience and literature review. Journal of Otolaryngol-Head & Neck Surgery. 2013; 42(2):https://journalotohns.biomedcentral.com/articles/10.1186/1916-0216-42-2.
        9. Khan MN & cols. Treatment modalities in sinonasal undifferentiated carcinoma: An analysis from the National Cancer Database. Int Forum Allergy Rhinol. 2016; https://onlinelibrary.wiley.com/wol1/doi/10.1002/alr.21861/full.

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