Stewart-Treves syndrome is a rare, deadly cutaneous angiosarcoma…………..

Yesterday I scanned a patient with the diagnosis of angiosarcoma. I was not familiar with the disease and had to do a little research before we did the procedure. She had a history of lymphedema for 13 years after her breast surgery for cancer. Her entire right arm was swollen. Her doctor had done a biopsy on two lesions on her arm and confirmed angiosacroma. She had a CT of the Chest and A MRI of the Forearm and Humerus. Here is some background info on Angiosacroma.

figure7.jpg this is not the patient I scanned it is only an example in angiosarcoma

Stewart-Treves syndrome is a rare, deadly cutaneous angiosarcoma that develops in long-standing chronic lymphedema. Most commonly, this tumor is a result of lymphedema induced by radical mastectomy to treat breast cancer. Unfortunately, although the breast cancer may be cured with such radical surgery, this second primary cancer may be responsible for the patient’s worsening course. The term Stewart-Treves syndrome is broadly applied to an angiosarcoma that arises in a chronically lymphedematous region due to any cause, including congenital lymphedema and other causes of secondary lymphedema unassociated with mastectomy. As reported by Durr et al in 2004, this lymphangiosarcoma occurs as a rare complication. Lymphangiosarcoma is a misnomer because this malignancy seems to arise from blood vessels instead of lymphatic vessels. A more appropriate name is hemangiosarcoma.

In 1906, Lowenstein first described angiosarcoma in a patient’s arm that had been affected by severe posttraumatic lymphedema for 5 years. In 1948, Stewart and Treves reported this rare secondary malignancy in 6 cases of angiosarcoma in postmastectomy lymphedema. They recognized that an edematous arm after radical mastectomy for breast cancer may suggest recurrent breast cancer, but that long-standing chronic edema without recurrent cancer may occasionally produce “a heretofore unrecognized and unreported sequel … long after the malignant breast neoplasm has apparently been arrested … a new specific tumor.” Stewart and Treves suggested that these angiosarcomas were probably not observed previously because they were mistaken for recurrent, inoperable, cutaneous manifestations of breast cancer.

Frequency:

  • Internationally: Currently, approximately 400 cases are reported in the world literature. In 1962, Schirger calculated that the incidence of this disease is 0.45% in patients who survive at least 5 years after radical mastectomy. Others have noted a much lower incidence of 0.07% in patients after mastectomy.

 Lymphangiosarcomas are extremely aggressive tumors with a high local recurrence rate and a tendency to metastasize early to many areas

  • Metastatic angiosarcoma to the lungs and chest wall are the most common cause of death in patients with Stewart-Treves syndrome.

  • Metastases to the liver and bones can also occur.

  • Lymphangiomas are associated with a high rate of local recurrence and metastasis, even after aggressive surgical treatment

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3 thoughts on “Stewart-Treves syndrome is a rare, deadly cutaneous angiosarcoma…………..

  1. I see that this is a post from 2008 …have there been any developements in treatment for the stewart-Treves-Syndrome since then?
    My mother was just diagnosed with the disease.

  2. I found some treatment info on medscape i don’t know how recent it is. I hope it helps.

    here’s the link.
    http://emedicine.medscape.com/article/1102114-treatment

    Chemotherapy and irradiation continue to be evaluated as adjuvants to surgery for the treatment of Stewart-Treves syndrome. Currently, these treatment options offer little benefit.

    In 2000, Grobmyer and associates found no statistical significant difference in the survival rates of patients treated with chemotherapy compared with those treated with irradiation.[29] Although long-term survivors after either radiation therapy or systemic chemotherapy have been reported,[30, 31] the overall results have been discouraging. A questionable response to weekly paclitaxel has been described.[32] As a result of these findings, these treatment options are reserved for patients with inoperable, advanced disease or those who refuse surgery.

    Intra-arterial mitoxantrone/paclitaxel was used for angiosarcoma of the lower limb associated with chronic lymphedema (Stewart-Treves syndrome) in a patient with cervical cancer.[33]

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