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Disease Profile


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.


US Estimated

Europe Estimated

Age of onset





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


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.


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.


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


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.


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.


Not applicable


Other names (AKA)

Malignant nodular/clear cell hidradenoma; Malignant acrospiroma; Malignant clear cell acrospiroma;


Rare Cancers


Hidradenocarcinoma is a rare tumor caused by the abnormal growth of cells in a sweat gland. It is a type of cancer that usually begins as a single spot (lesion) on the skin of the head or neck, but can be been found on other parts of the body. This type of tumor most often develops in people between the ages of 30-60 years old, but has been found in children. Hidradenocarcinoma is usually painless and tends to grow slowly, but may spread into nearby tissues, especially lymph nodes, or to more distant parts of the body (metastasis). The cause of the tumor is unknown. Diagnosis is usually made by careful examination and a series of special skin tests. The main treatment is surgical removal of the tumor often followed by radiation therapy and/or chemotherapy. Long term outlook is best when the cancer is found early.[1][2]


The cause of hidradenocarcinoma is unknown. The only known risk factor is the prior presence of a benign hidradenoma. Molecular genetic changes in the cells of the hidradenocarcinoma have been found in some cases, but more studies must be performed before medical researchers can determine if a pattern exists.[1][2][3][4][5] 

Please note, molecular genetic changes are also called somatic mutations or acquired mutations. You are not born with an acquired mutation, instead the change happens some time during your life. This change in the gene occurs in one cell, and then is passed on to any new cells that are the offspring of that cell. This kind of mutation cannot be passed on to your children. Acquired mutations are much more common than inherited mutations. Most cancers are caused by acquired mutations.[6] 


Because hidradenocarcinoma is quite rare, there are no established guidelines for treatment. The most accepted form of treatment is wide local excision of the tumor, but due to the high risk that the tumor will grow back and frequent metastasis to nearby lymph nodes and other parts of the body, some doctors also suggest radiation therapy and/or chemotherapy after surgery.[1][2]

Newer targeted therapies like trastuzumabepidermal growth factor receptor (EGFR) inhibitors, PI3K/Akt/mTOR pathway inhibitors (targets a pathway believed to be involved in controlling cell growth and division), hormonal agents like antiandrogens, and electrochemotherapy (the use of electrical pulses to help get the chemo drugs into the cancer cells) may also be offered. Most of these therapies have only been reported in isolated case reports and often with conflicting results. Therefore it is difficult to determine which therapy or combination of therapies are the best treatment option. Options are usually determined by the size and location of the cancer and the extent to which cancer cells may have spread to nearby tissues.. Many researchers are hoping if there is a better understanding of the molecular genetic changes which happen in the cells of hidradenocarcinoma, the use of targeted therapies will improve long term prognosis.[1][2]


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.

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


      1. Hidradenocarcinoma of Skin. DoveMed. January 7 2016; https://www.dovemed.com/diseases-conditions/hidradenocarcinoma-skin.
      2. Soni A, Bansal N, Kaushal V, and Chauhan AK. Current management approach to hidradenocarcinoma: a comprehensive review of the literature. Ecancermedicalscience. March 19 2015; 9:517. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4370612.
      3. Gupta E, Guthrie KJ, Krishna M, Asmann Y, Parker AS, and Joseph RW. Whole exome sequencing of a patient with metastatic hidradenocarcinoma and review of the literature. Rare Tumors. February 25 2015; 7(1):5719. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4387361.
      4. Kazakov DV1, Ivan D, Kutzner H, Spagnolo DV, Grossmann P, Vanecek T, Sima R, Kacerovska D, Shelekhova KV, Denisjuk N, Hillen U, Kuroda N, Mukensnabl P, Danis D, and Michal M. Cutaneous hidradenocarcinoma: a clinicopathological, immunohistochemical, and molecular biologic study of 14 cases, including Her2/neu gene expression/amplification, TP53 gene mutation analysis, and t(11;19) translocation. Am J Dermatopathol. May 2009; 31(30:236-47. https://www.ncbi.nlm.nih.gov/pubmed/19384064.
      5. Nash JW1, Barrett TL, Kies M, Ross MI, Sneige N, Diwan AH, and Lazar AJ. Metastatic hidradenocarcinoma with demonstration of Her-2/neu gene amplification by fluorescence in situ hybridization: potential treatment implications. J Cutan Pathol. January 2007; 34(1):49-54. https://www.ncbi.nlm.nih.gov/pubmed/17214855.
      6. Genes and Cancer. American Cancer Society. June 2014; https://www.cancer.org/acs/groups/cid/documents/webcontent/002550-pdf.pdf.
      7. Gauerke S, Driscoll JJ. Hidradenocarcinomas: A Brief Review and Future Directions. Archives of Pathology and Laboratory Medicine. May 2010; 134(5):781-5. https://www.ncbi.nlm.nih.gov/pubmed/20441512.

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