Rare Pulmonology News

Disease Profile

Malignant eccrine spiradenoma

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)

Eccrine spiradenoma, malignant; MES

Categories

Rare Cancers

Summary

Malignant eccrine spiradenoma is a type of tumor that develops from a sweat gland in the skin. It starts as a rapidly-growing bump on the head or abdomen, and may cause tenderness, redness, or an open wound.[1] The exact cause of malignant eccrine spiradenoma is unknown, though it is thought that sun exposure or problems with the immune system (immunosuppression) may contribute to the development of this tumor.[2] Because malignant eccrine spiradenoma is quite rare, there are no established treatment guidelines; however, in practice, surgery is often performed to remove the tumor and additional treatments may follow, depending on the severity and extent of the cancer.[1][2]

Treatment

Surgery to remove as much of the tumor as possible is usually the first step of treatment for malignant eccrine spiradenoma. Both a traditional surgical technique known as wide local excision and the newer Mohs micrographic surgery are thought to be effective for treating this cancer. Additional treatment may include radiation therapy to destroy any cancer cells that might remain after surgery. Though chemotherapy has been used in cases of malignant eccrine spiradenoma, it is thought to be of limited help in treating this disease.[1]

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 Malignant eccrine spiradenoma. Click on the link to view a sample search on this topic.

References

  1. Andreoli MT, Itani KM. Malignant eccrine spiradenoma: a meta-analysis of reported cases. American Journal of Surgery. 2011; 201:695-699. https://www.ncbi.nlm.nih.gov/pubmed/20851376. Accessed 11/8/2011.
  2. Kampshoff JL, Cogbill TH. Unusual skin tumors: Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans. Surgical Clinics of North America. 2009; 89:727-738. https://www.ncbi.nlm.nih.gov/pubmed/19465208. Accessed 11/8/2011.

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