Revision 4 for 'Lymphangiosarcoma'

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Lymphangiosarcoma

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

Epidemiology

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

Presentation

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.

Pathology

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

Etiology

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

Radiographic features

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

MRI

Enhancing vascular nodular soft issue tumor involve 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.

Nuclear medicine

The tumor shows avid uptake on PET/CT.

Treatment and prognosis

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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