Lymphangiosarcomas are rare malignant soft tissue neoplasms represent a vascular tumor (angiosarcoma) that occurs in patients with chronic lymphoedema.

It affects females more than males, as it is commonly associated with post-mastectomy lymphoedema. 

Chronic lymphoedema with skin and soft tissue tumor in the involved limb. A history of previous radical mastectomy for breast cancer is supportive in cases of upper limb lymphangiosarcoma.

It is an aggressive malignant tumor that arises from vascular channels of soft tissues in limbs with chronic lymphoedema. The pathogenesis is still unclear. Hypothesises suggest chronic lymphoedema may act as carcinogens or cause local immunodeficiency.

Most commonly this occurs in upper limbs after chronic lymphoedema (called Steward-Treves syndrome). Other causes include primary congenital lymphoedema, trauma or filarial lymphoedema.

Often, it is radiologically difficult to identify the extent of the tumor. Distant metastasis usually goes to the lung.

Enhancing vascular nodular soft issue tumor involves soft tissue and skin of the involved limbs associated with signs of edema within subcutaneous tissues. It is best evaluated by MRI and seen as intermediate T1 signal and low T2 signal masses with post-contrast enhancement.

The tumor shows avid uptake on PET/CT.

This is a poor prognostic tumor, the mean survival rate is 2.5 years. The treatment is aggressive surgical resection and amputation of limbs is frequently recommended, even though the recurrence rate still high.

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Article information

rID: 65350
System: Vascular
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Steward-Treves syndrome

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