Malignant eccrine spiradenoma

Title

Other Names:

Eccrine spiradenoma, malignant; MES

Categories:

Rare Cancers

Summary 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]

Last updated: 11/14/2011

Treatment 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]

Last updated: 11/14/2011

Prognosis Prognosis

There is one article which reviews treatment and survival in 72 patients with malignant eccrine spiradenoma. For those patients whose cancer remained in the original location and had not spread to nearby lymph nodes, all of them were alive and disease-free during the time they were followed by their physicians (the follow-up time for these individuals ranged from six months to 12 years, with an average follow-up time of three years for the group). Of the patients whose tumor had spread to lymph nodes, half were alive and disease-free during follow-up (the follow-up time for these individuals ranged from two months to 8 years, with an average follow-up time of four years for the group). Survival was the lowest for those patients whose tumor had spread beyond the lymph nodes to more distant parts of the body (metastasized).^[1]

Last updated: 11/14/2011

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In-Depth Information

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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.

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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
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References References

Andreoli MT, Itani KM. Malignant eccrine spiradenoma: a meta-analysis of reported cases. American Journal of Surgery. 2011; 201:695-699. http://www.ncbi.nlm.nih.gov/pubmed/20851376. Accessed 11/8/2011.
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. http://www.ncbi.nlm.nih.gov/pubmed/19465208. Accessed 11/8/2011.

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