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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. [1]
Lymphedema is most frequently a complication of cancer treatment or parasitic infections, but it can also be seen in a number of genetic disorders. Tissues with lymphedema are at high risk of infection because the lymphatic system has been compromised. [3] Though incurable and progressive, a number of treatments may improve symptoms. [2]
Edema (American English), also spelled oedema (British English), and also known as fluid retention, dropsy and hydropsy, is the build-up of fluid in the body's tissue, [1] a type of swelling. [4] Most commonly, the legs or arms are affected. [1] Symptoms may include skin that feels tight, the area feeling heavy, and joint stiffness. [1]
Individuals affected by RS3PE typically have repeated episodes of inflammation of the lining of their synovial joints and swelling of the end portion of the limbs. [8] The arms and hands are more commonly affected than the legs and feet. [8] Both sides are usually involved though RS3PE can affect only one side in certain cases. [8]
Characteristic features are edema (swelling due to excess fluid) of the face and arms and development of swollen collateral veins on the front of the chest wall. Shortness of breath and coughing are quite common symptoms; difficulty swallowing is reported in 11% of cases, headache in 6% and stridor (a high-pitched wheeze) in 4%.
Patients initially treated at cancer centers have lower recurrence rates than those initially treated by community centers. [24] Surgery has been the most common form of treatment for both localized [3]: 101 [5]: 361 and diffuse TGCT.
After a diagnosis and before treatment, cancer is staged. This refers to determining if the cancer has spread, and if so, whether locally or to distant sites. Staging is reported as a grade between I (confined) and IV (spread). The stage of a lymphoma helps predict a patient's prognosis and is used to help select the appropriate therapy. [44]
Indolent lymphoma, also known as low-grade lymphoma, is a group of slow-growing non-Hodgkin lymphomas (NHLs). [3] Because they spread slowly, they tend to have fewer signs and symptoms when first diagnosed and may not require immediate treatment.
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