Stewart–Treves syndrome

Stewart–Treves Syndrome

Stewart–Treves syndrome is a rare and aggressive form of angiosarcoma that typically arises in the context of chronic lymphedema. It is most commonly associated with post-mastectomy lymphedema, but can occur in any situation where chronic lymphedema is present.

Pathophysiology

Stewart–Treves syndrome develops due to the chronic lymphatic obstruction and subsequent immune dysfunction that occurs in long-standing lymphedema. The stagnant lymph fluid creates an environment conducive to the development of malignant endothelial cells, leading to the formation of angiosarcoma. The exact molecular mechanisms are not fully understood, but it is believed that chronic inflammation and impaired immune surveillance play significant roles.

Clinical Presentation

Patients with Stewart–Treves syndrome typically present with a purplish, bruise-like lesion on the skin overlying the area of chronic lymphedema. These lesions can rapidly progress to form nodules or ulcerated masses. The condition is most frequently observed in the upper extremities of women who have undergone radical mastectomy with axillary lymph node dissection, but it can also occur in other areas affected by lymphedema.

Diagnosis

The diagnosis of Stewart–Treves syndrome is primarily clinical, supported by histopathological examination. A biopsy of the lesion typically reveals atypical, malignant endothelial cells forming irregular vascular channels. Immunohistochemical staining is often positive for markers such as CD31, CD34, and factor VIII-related antigen, which are indicative of vascular origin.

Treatment

The mainstay of treatment for Stewart–Treves syndrome is surgical excision with wide margins to ensure complete removal of the tumor. Due to the aggressive nature of the disease, amputation of the affected limb may be necessary in some cases. Adjuvant therapies, including radiation and chemotherapy, may be considered, although their effectiveness is limited. Early detection and treatment are crucial for improving outcomes.

Prognosis

The prognosis for patients with Stewart–Treves syndrome is generally poor, with a high rate of local recurrence and distant metastasis. The five-year survival rate is low, emphasizing the importance of early diagnosis and intervention.

Epidemiology

Stewart–Treves syndrome is a rare condition, with an estimated incidence of less than 1% in patients with chronic lymphedema. It predominantly affects women who have undergone breast cancer treatment, but can occur in any individual with long-standing lymphedema.

History

The syndrome was first described by Dr. Fred Stewart and Dr. Norman Treves in 1948, who reported cases of angiosarcoma in patients with post-mastectomy lymphedema. Their work highlighted the potential for malignant transformation in chronic lymphedema, leading to increased awareness and research into the condition.

Also see

• Lymphedema
• Angiosarcoma
• Breast cancer
• Mastectomy
• Oncology

Resources

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Source: Data courtesy of the U.S. National Library of Medicine. Since the data might have changed, please query MeSH on Stewart–Treves syndrome for any updates.

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