Last revised by Daniel J Bell on 2 Sep 2022

Lymphangiosarcomas are rare malignant soft tissue neoplasms that generally occur in patients with chronic lymphedema.

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

Chronic lymphedema 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.

Lymphangiosarcoma is an aggressive malignant tumor that arises from vascular channels of soft tissues in limbs with chronic lymphedema. The pathogenesis is still unclear. Hypotheses suggest chronic lymphedema may act as a carcinogen or cause local immunodeficiency.

Most commonly this occurs in upper limbs after chronic lymphedema (called Steward-Treves syndrome).

Other causes include primary congenital lymphedema, trauma or filarial lymphedema.

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

Lymphangiosarcoma is an enhancing vascular nodular soft tissue tumor which involves soft tissue and skin of the involved limbs and is 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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