Stewart–Treves syndrome

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What is Stewart–Treves syndrome?

Stewart–Treves Syndrome is a rare and aggressive form of cutaneous lymphangiosarcoma. It is named after two doctors who first described it in a series of patients with lymphoedema following mastectomy for breast cancer.

Who gets Stewart–Treves syndrome?

Stewart–Treves syndrome is a cutaneous lymphangiosarcoma that develops in lymphoedema following mastectomy with axillary node clearance or axillary radiation. However, the term is also used wherever lymphangiosarcoma arises in primary congenital or secondary chronic lymphoedema. It typically only arises in substantial and persistent lymphoedema.

Stewart–Treves syndrome occurs in approximately 1 in 200 patients who survive mastectomy for more than 5 years. The average time interval between mastectomy and development of Stewart-Treves syndrome is 10.5 years. The incidence of chronic lymphoedema and subsequent Stewart-Treves syndrome is decreasing with advancements in surgical techniques and new alternative adjuvant therapies used for breast cancer.

What causes Stewart–Treves syndrome?

The exact pathophysiology of Stewart-Treves syndrome remains unknown. It is believed the impairment of immune function in the region due to poor lymphatic flow may allow a malignancy to develop. In a recent study the c-myc oncogene was overexpressed.

What are the clinical features of Stewart–Treves syndrome?

Stewart-Treves syndrome usually develops in the ipsilateral upper arm to a mastectomy. It has also been reported to arise in the involved breast and in the ipsilateral axilla.

• Chronic lymphoedema results in limb swelling with skin thickening and scale.
• Lymphangiosarcoma presents as:
  • Subcutaneous lump
  • Non-healing eschar
  • Multiple blue-purple nodules.

What are the complications of Stewart–Treves syndrome?

Chronic lymphoedema can be complicated by recurrent erysipelas or cellulitis, and deep vein thrombosis of the affected limb.

How is Stewart–Treves syndrome diagnosed?

The clinical findings in a setting of chronic lymphoedema raise the possible diagnosis of Stewart–Treves syndrome, with confirmation on skin biopsy.

The histology of the lymphangiosarcoma in Stewart–Treves syndrome is characterised by:

• Proliferating vascular channels dissecting through collagen bundles
• Pleomorphic and hyperchromatic endothelial tumour cells
• Mitotic figures
• Immunohistochemical staining positive for Factor VIII-related antigen, CD31, laminin, cytokeratin, and collagen IV.

Imaging with CT and MRI is used for staging the tumour.

What is the differential diagnosis for Stewart–Treves syndrome?

The differential diagnosis for Stewart–Treves syndrome may include:

• Recurrence of breast cancer — this would more likely arise on the breast, rather than the upper arm
• Kaposi sarcoma — this can arise without lymphoedema and can be distinguished by staining for human herpesvirus 8
• Melanoma
• Other malignancy with cutaneous metastases.

What is the treatment for Stewart–Treves syndrome?

The treatment for Stewart–Treves syndrome depends on the stage of the disease. Wide local excision or amputation of the affected limb is recommended if no metastatic disease is found.

Chemotherapy and radiotherapy have not been reported to improve survival.

It is thought that Stewart–Treves syndrome can be prevented by appropriate treatment for lymphoedema following breast cancer surgery.

What is the outcome for Stewart-Treves syndrome?

Early detection and treatment is crucial. However, the outcome for Stewart-Treves syndrome remains poor with a median survival of 2.5 years after diagnosis. Untreated patients usually die within 5 to 8 months after diagnosis.