Revision 2 for 'Lymphangiosarcoma'

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Lymphangiosarcoma

Lymphangiosarcoma  Is rare malignant soft tissue neoplasm represent vascular tumor ( angiosarcoma) usually involve limbs with heavy chronic lymphedema .

Epidemiology:

One of rarest malignant soft tissue tumors , the common issue is underlying lymphedema . most commonly  occurs in upper limbs in  post- mastectomy with chronic upper limb edema. Hence, it affects Females more than males

Pathology:

Aggressive malignant tumor arise from vascular channel of soft tissues ( angiosarcoma) develop in limbs with chronic lymph edema . The pathogenesis still unclear. Hypothesis's suggest chronic lymphedema may act as carcinogens or local immunodeficiency.

 

Etiology:

Most common cause is Steward-Treves syndrome (cutaneous lymphangiosarcoma in upper limbs occur after chronic lymphedema after radical mastectomy). Other  causes is primary congenital lymphedema, traumatic or filarial lymphedema.

Presentation:

Chronic lymphedema with skin and soft tissue tumor in the involved limb. History of previous radical mastectomy for previous breast cancer is supportive in case of upper limb lymphangiosarcoma.

Radiographic features:

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

The tumor shows avid uptake on PET/CT.

It is radiologically difficult to identify the extent of tumor.

Distant metastasis is usually Lung metastasis.

Treatment and prognosis:

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 recurrence rate still high.

 

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