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lymphangiosarcoma

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Also known as Lymphangiosarcoma of Stewart and Treves, Stewart-Treves syndrome (disorder), malignant Lymphangioendothelioma, skin lymphangiosarcoma, Stewart-Treves syndrome, Lymphangiosarcoma of the Skin, Lymphangiosarcoma of Skin

Lymphangiosarcoma is a rare cancer which occurs in long-standing cases of primary or secondary lymphedema (swelling due to lymphatic system obstruction). It involves either the upper or lower lymphedematous extremities but is most common in upper extremities. Although its name implies lymphatic origin, it is believed to arise from endothelial cells and may be more accurately referred to as angiosarcoma.

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NCI Thesaurus ID
C4490
ICD-9-CM
171.2
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  • Signs and symptoms
  • Causes
  • Treatment
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Lymphangiosarcoma is a rare cancer which occurs in long-standing cases of primary or secondary lymphedema (swelling due to lymphatic system obstruction). It involves either the upper or lower lymphedematous extremities but is most common in upper extremities. Although its name implies lymphatic origin, it is believed to arise from endothelial cells and may be more accurately referred to as angiosarcoma.

==Signs and symptoms== Lymphangiosarcoma may present as a purple discoloration or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting to an extensive necrotic focus involving the skin and subcutaneous tissue. It metastasizes quickly. Lymphangiosarcoma mostly occurs in the upper arm, forearm, elbow, and anterior chest wall. Furthermore, in post-mastectomy patients, lymphangiosarcoma is associated with sarcomatous degeneration, especially in patients who have survived five or more years and who have severe lymphedema.

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