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Disease Profile

Stewart Treves syndrome

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)

Angiosarcoma associated with chronic lymphedema; Postmastectomy extremity angiosarcoma; Lymphangiosarcoma following mastectomy

Categories

Hereditary Cancer Syndromes

Summary

Stewart Treves syndrome is a rare angiosarcoma that develops in people with long-standing lymphedema. Most affected people have a history of breast cancer that was treated with a radical mastectomy. Early signs of the condition vary and may include purplish patches that develop into skin nodules, a mass under the skin, or a poorly healing eschar with recurrent bleeding and oozing. The exact underlying cause of the condition is poorly understood. Although Stewart Treves syndrome is always associated with long-standing lymphedema, other unknown factors also appear to contribute to the development of the condition. Early diagnosis and aggressive surgical treatment may improve the long-term outlook of the condition. Chemotherapy, radiation therapy, and immunotherapy continue to be studied as possible treatment options and may be recommended in some cases.[1][2][3]

Symptoms

The signs and symptoms of Stewart Treves syndrome often appear approximately 5-15 years after treatment with radical mastectomy and long-standing (chronic) edema. Early signs of Stewart Treves syndrome may include the following which develop in areas of chronic edema:[1][3][2]

  • A purplish patch that develops into a skin nodule
  • A mass under the skin
  • A poorly healing eschar with recurrent bleeding and oozing

The condition may begin as multiple, separate skin lesions that can grow together and form an enlarging lesion. As Stewart Treves syndrome advances, the skin over the lesion may become thin and ulcerate, leading to recurrent episodes of bleeding and infection.[1][3]

Stewart Treves syndrome is a very aggressive form of cancer and often spreads quickly to other parts of the body, especially the lungs.[3]

Cause

The exact underlying cause of Stewart Treves syndrome is not fully understood. However, all cases are associated with long-standing lymphedema. Although most people who develop Stewart Treves syndrome have a history lymphedema following treatment with radical mastectomy, it can also develop in other forms of lymphedema that are not related to this surgery. For example, other causes of lymphedema may include trauma, filariasis, pooling of blood in veins, and morbid obesity.[1][2]

Of note, certain types of lymphedema , such as that caused by cardiac or renal disease, is not associated with Stewart Treves syndrome. Therefore, lymphedema alone is not sufficient to cause the condition. This suggests that other factors such as a genetic predisposition may also be present in affected people.[1][2]

Diagnosis

A diagnosis of Stewart Treves syndrome is often suspected based on the presence of characteristic signs and symptoms when a person has a history of radical mastectomy and/or long-standing lymphedema. Additional testing can then be order to confirm the diagnosis and determine the severity of the condition. These may include:[1]

Treatment

Stewart Treves syndrome is generally treated with surgery. Early diagnosis with amputation or wide local excision offers the best chance for long-term survival. Chemotherapy, radiation therapy, and immunotherapy continue to be studied as possible treatment options and may be recommended in some cases.[1][2][3]

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 Stewart Treves syndrome. Click on the link to view a sample search on this topic.

References

  1. Robert A Schwartz, MD, MPH. Stewart-Treves Syndrome. Medscape Reference. June 22, 2016; https://emedicine.medscape.com/article/1102114-overview.
  2. Sharma A, Schwartz RA. Stewart-Treves syndrome: pathogenesis and management. J Am Acad Dermatol. December 2012; 67(6):1342-1348.
  3. Cui L, Zhang J, Zhang X, Chang H, Qu C, Zhang J, Zhong D. Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature. Int J Clin Exp Pathol. September 2015; 8(9):11108-11115.

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