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Author: Roy Spires, Medical Student, University of Auckland, Auckland, New Zealand. Medical Editor: Dr Helen Gordon, Auckland, New Zealand. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. September 2020.
Stewart–Treves Syndrome is a rare and aggressive form of cutaneous lymphangiosarcoma. It is named after two doctors who first described it in a series of patients with lymphoedema following mastectomy for breast cancer.
Stewart–Treves syndrome is a cutaneous lymphangiosarcoma that develops in lymphoedema following mastectomy with axillary node clearance or axillary radiation. However, the term is also used wherever lymphangiosarcoma arises in primary congenital or secondary chronic lymphoedema. It typically only arises in substantial and persistent lymphoedema.
Stewart–Treves syndrome occurs in approximately 1 in 200 patients who survive mastectomy for more than 5 years. The average time interval between mastectomy and development of Stewart-Treves syndrome is 10.5 years. The incidence of chronic lymphoedema and subsequent Stewart-Treves syndrome is decreasing with advancements in surgical techniques and new alternative adjuvant therapies used for breast cancer.
The exact pathophysiology of Stewart-Treves syndrome remains unknown. It is believed the impairment of immune function in the region due to poor lymphatic flow may allow a malignancy to develop. In a recent study the c-myc oncogene was overexpressed.
Stewart-Treves syndrome usually develops in the ipsilateral upper arm to a mastectomy. It has also been reported to arise in the involved breast and in the ipsilateral axilla.
The clinical findings in a setting of chronic lymphoedema raise the possible diagnosis of Stewart–Treves syndrome, with confirmation on skin biopsy.
The histology of the lymphangiosarcoma in Stewart–Treves syndrome is characterised by:
Imaging with CT and MRI is used for staging the tumour.
The differential diagnosis for Stewart–Treves syndrome may include:
The treatment for Stewart–Treves syndrome depends on the stage of the disease. Wide local excision or amputation of the affected limb is recommended if no metastatic disease is found.
Chemotherapy and radiotherapy have not been reported to improve survival.
It is thought that Stewart–Treves syndrome can be prevented by appropriate treatment for lymphoedema following breast cancer surgery.
Early detection and treatment is crucial. However, the outcome for Stewart-Treves syndrome remains poor with a median survival of 2.5 years after diagnosis. Untreated patients usually die within 5 to 8 months after diagnosis.
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