Stewart–Treves syndrome refers to a

lymphangiosarcoma Lymphangiosarcoma is a rare cancer which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedematous extremities but is most common in upper extremities. Although its name implies lymphat ...

, a rare disorder marked by the presence of an angiosarcoma (a malignant tumor of blood or lymph vessels) in a person with chronic (long-term)

lymphedema Lymphedema, also known as lymphoedema and lymphatic edema, is a condition of localized swelling caused by a compromised lymphatic system. The lymphatic system functions as a critical portion of the body's immune system and returns interstitial fl ...

. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from

mastectomy Mastectomy is the medical term for the surgical removal of one or both breasts, partially or completely. A mastectomy is usually carried out to treat breast cancer. In some cases, women believed to be at high risk of breast cancer have the operat ...

and/or

radiotherapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...

for

breast cancer Breast cancer is cancer that develops from breast tissue. Signs of breast cancer may include a lump in the breast, a change in breast shape, dimpling of the skin, milk rejection, fluid coming from the nipple, a newly inverted nipple, or a re ...

, 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.

Cause

Common risk factors that may lead to the development of lymphangiosarcoma include lymphatic blockage, radiotherapy, mastectomy, cardiovascular diseases, and hypertension. The sarcoma first appears as a bruise mark, a purplish discoloration or a tender skin nodule in the extremity, typically on the anterior surface. * Lymphangiosarcoma is caused by chronic lymphedema. * Causes of lymphedema include: ** Primary lymphedema *** Congenital *** Precox (adolescence) *** Tarda (adulthood) ** Secondary lymphedema *** Malignancy *** Recurrent cellulitis *** Connective tissue disease *** Infection (filariasis) *** Contact dermatitis *** Lymphatic damage (surgery, radiation therapy, burns, etc.).

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. Prognosis is generally poor, and the 5 year survival rate of patients with lymphangiosarcoma is less than 5%.

Incidence

In the 1960s, the incidence five 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

History

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

References

External links

{{DEFAULTSORT:Stewart-Treves syndrome Breast cancer Vascular diseases Vascular-related cutaneous conditions Syndromes affecting the breast