Stewart-Treves Syndrome

Stewart-Treves Syndrome (STS) is a rare, aggressive form of lymphangiosarcoma, a type of cancer that develops in the lymphatic system. This condition typically arises in the setting of long-standing lymphedema, most commonly following surgical procedures such as a mastectomy for breast cancer, where lymph nodes are removed or damaged. The syndrome was first described in 1948 by Fred Stewart and Norman Treves, highlighting its association with post-mastectomy lymphedema. However, it can also occur in other parts of the body affected by chronic lymphedema.

Etiology and Pathogenesis

The exact cause of Stewart-Treves Syndrome is not fully understood, but it is believed to be related to the chronic inflammation and impaired lymphatic drainage associated with lymphedema. This environment may facilitate the development of malignant cells. The syndrome is exceedingly rare, with a few hundred cases reported in the medical literature.

Clinical Presentation

Patients with Stewart-Treves Syndrome typically present with a rapidly enlarging, often painful, mass within an area of chronic lymphedema. The skin over the mass may appear bruised or exhibit a purplish discoloration. Additional symptoms may include increased local warmth, fever, and a general feeling of being unwell.

Diagnosis

Diagnosis of Stewart-Treves Syndrome involves a combination of clinical examination, imaging studies such as ultrasound, MRI, and CT scan, and biopsy of the lesion. Histopathological examination of the biopsy specimen is crucial for confirming the diagnosis, showing the characteristic features of lymphangiosarcoma.

Treatment

The treatment of Stewart-Treves Syndrome is challenging due to its aggressive nature and the poor prognosis associated with the condition. Treatment options may include surgery to remove the tumor, radiation therapy, and chemotherapy. However, the effectiveness of these treatments is limited, and the overall survival rate remains low.

Prognosis

The prognosis for patients with Stewart-Treves Syndrome is generally poor, with a high rate of local recurrence and distant metastasis. The median survival time from diagnosis is typically less than three years.

Prevention

Preventing the development of lymphedema following surgery, through measures such as early mobilization, physical therapy, and compression garments, may reduce the risk of developing Stewart-Treves Syndrome. Regular monitoring of patients with chronic lymphedema for any signs of skin changes or masses is also crucial for early detection and management of this condition.

See Also

• Lymphangiosarcoma
• Lymphedema
• Mastectomy
• Cancer

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