Stewart-Treves Syndrome Treatment & Management

Updated: Apr 06, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

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.

Multimodal therapy including hyperthermic isolated limb perfusion with tumor necrosis factor-alpha and melphalan, combined with radical resection of the affected skin and subcutaneous tissue including the fascia, with large safety margins, may provide enhanced survival. [43]

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. [44] Although long-term survivors after either radiation therapy or systemic chemotherapy have been reported, [45, 46] the overall results have been discouraging. A questionable response to weekly paclitaxel has been described. [47] 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. [48]

In 1994, Furue et al demonstrated that immunotherapy may be beneficial as palliative treatment for pleural effusions caused by metastatic angiosarcoma. [49]

Expression of VEGF-C makes this angiosarcoma a good potential candidate for targeted antilymphangiogenic therapy. [17]

Complications from metastatic disease, such as pleural effusions, may require hospitalization of the patient. A CT scan may identify bilateral pulmonary involvement. Stewart-Treves syndrome patients may need further inpatient care for pain control.

Stewart-Treves syndrome occurring in the abdominal wall was successfully treated with eribulin mesylate, a structurally modified analog of halichondrin B, originally isolated from the marine sponge Halichondria okadai. [50]

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Surgical Care

Early amputation or wide local excision provides the best chance of long-term survival in patients with Stewart-Treves syndrome. Some authorities favor radical ablative surgery with an early diagnosis, in order to confer a reasonable prognosis with this rare but aggressive disease. [32, 51] A nihilistic approach is undesirable.

The most common approach in patients with lymphangiosarcoma is amputation of the limb or forequarter rather than wide local surgical excision. Even in cases with early surgical treatment, the prognosis is disappointing, with a high rate of local recurrence and metastasis. Metastatic disease should exclude surgical treatment unless surgery is useful for symptomatic improvement.

Chemotherapy, immunotherapy, and/or radiation therapy can be used as adjuvants to surgery (see Medical Care).

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Prevention

The most efficient way to avoid Stewart-Treves syndrome, a highly lethal disease, is by preventing or treating long-standing lymphedema, which predisposes individuals to this lymphangiosarcoma years later.

Other complications commonly associated with chronic lymphedema, such as erysipelas and deep venous thromboses, must be prevented by regularly examining these areas and then treating them.

Early biopsy of any suggestive lesion should be performed because early diagnosis and surgical treatment offers the highest rate of long-term survival.

Prevention of tumor progression in Stewart-Treves syndrome with the use a low dose of pazopanib has been suggested. [52]

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