Stewart-Treves syndrome

Stewart-Treves syndrome is a rare and aggressive form of angiosarcoma that typically arises in the setting of chronic lymphedema. This condition is most commonly associated with long-standing lymphedema following mastectomy for breast cancer, but it can also occur in other contexts of chronic lymphedema.

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 angiosarcoma, a malignant tumor originating from the endothelial cells of blood vessels.

Clinical Presentation

Patients with Stewart-Treves syndrome typically present with a rapidly enlarging, purplish-red lesion in the area affected by lymphedema. These lesions can be nodular or plaque-like and may ulcerate or bleed. The condition is often painful and can be mistaken for benign lymphedema-related changes or other skin conditions.

Diagnosis

The diagnosis of Stewart-Treves syndrome is confirmed through a combination of clinical examination, imaging studies, and histopathological analysis. Biopsy of the lesion reveals the characteristic features of angiosarcoma, including atypical endothelial cells and irregular vascular channels.

Treatment

The primary treatment for Stewart-Treves syndrome is surgical excision with wide margins to ensure complete removal of the tumor. Radiation therapy and chemotherapy may be used as adjunctive treatments, although the prognosis remains poor due to the aggressive nature of the disease.

Prognosis

The prognosis for patients with Stewart-Treves syndrome is generally poor, with a high rate of local recurrence and distant metastasis. Early detection and aggressive treatment are crucial for improving outcomes.

History

Stewart-Treves syndrome was first described by Dr. Fred Stewart and Dr. Norman Treves in 1948. They reported cases of angiosarcoma developing in patients with chronic lymphedema following mastectomy for breast cancer.

Related Pages

• Angiosarcoma
• Lymphedema
• Mastectomy
• Breast cancer
• Radiation therapy
• Chemotherapy