Stewart–Treves syndrome

Stewart–Treves syndrome, refers to a lymphangiosarcoma, a rare complication that forms as a result of chronic, long-standing lymphedema. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer, it may also describe lymphangiosarcomas that result from congenital and other causes of chronic secondary lymphedema. Lymphangiosarcoma arising from cancer-related lymphedema has become much less common with better surgical techniques, radiation therapy, and conservative treatment. The prognosis, even with wide surgical excision and subsequent radiotherapy, is poor.

Result of chronic, long-standing lymphedema. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer.

Diagnosis is most commonly made on the basis of prior history of Breast Carcinoma, history of Mastectomy and clinical features that usually occur 10 to 12 years after the Modified Radical Mastectomy.

Treatment

The treatment of choice is a large resection or amputation of the affected limb. Radiation therapy can precede or follow surgical treatment. Tumors that have advanced locally or have metastasized can be treated with mono or polychemotherapy, systemically or locally. However, chemotherapy and radiation therapy have not been shown to improve survivorship significantly. In cases of upper limbs, forequarter amputation (disarticulation of upperlimb along with clavicle and scapula) is preferred.

Prognosis

Early detection is key. Untreated patients usually live 5 to 8 months after diagnosis.

Incidence

In the 1960s, the incidence 5 years after a radical mastectomy varied from 0.07% to 0.45%. Today, it occurs in 0.03% of patients surviving 10 or more years after radical mastectomy.

History

It was discovered by Fred W. Stewart and Norman Treves in 1948.

See also

• Lymphangiosarcoma
• Angiosarcoma
• Mastectomy
• Postcardiotomy syndrome
• List of cutaneous conditions
• Lymphedema

External links

Classification
External resources	eMedicine: article/1102114