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There is no standard approach to the treatment of lymphangiomatosis and treatment often is aimed at reducing symptoms. [2] [17] Surgical intervention may be indicated when complications arise and a number of reports of response to surgical interventions, medications, and dietary approaches can be found in the medical literature. [2] [16] [17]
Given the phenotypic variability, there are no established diagnostic criteria. Instead, the diagnosis is made based on clinical evidence and supporting histopathology that demonstrates the presence of positive lymphatic markers, such as podoplanin (D2-40) or lymphatic vessel endothelial hyaluronan receptor 1 (), as well as negative glucose transporter 1 ().
Treatment for cystic hygroma involves the removal of the abnormal tissue; however, complete removal may be impossible without removing other normal areas. Surgical removal of the tumor is the typical treatment provided, with the understanding that additional removal procedures will most likely be required as the lymphangioma grows.
Treatment is often pursued for troubling symptoms (itching, pain) or for cosmetic reasons. Surgical removal (excision) of the affected skin layers is the most common and effective treatment. Ablative carbon dioxide laser therapy is a less invasive method that can improve the appearance.
Gorham's disease (pronounced GOR-amz), also known as Gorham vanishing bone disease and phantom bone disease, [1] is a very rare skeletal condition of unknown cause.It is characterized by the uncontrolled proliferation of distended, thin-walled vascular or lymphatic channels within bone, which leads to resorption and replacement of bone with angiomas and/or fibrosis.
Lymphohemangioma is a disease characterized by swelling of the lymph nodes and blood vessels.It is variously described as a "mixture of clear fluid and blood-filled cysts", [citation needed] a mass of abnormal swollen veins and lymph nodes, or a tumorous growth of lymph and blood vessels.
Acquired progressive lymphangioma, also known as benign lymphangioendothelioma is a group of lymphangiomas that occur anywhere in young individuals, grow slowly, and present as bruise-like lesions or erythematous macules.
The most successful treatment for lymphangiosarcoma is amputation of the affected limb if possible. Chemotherapy may be administered if there is evidence or suspicion of metastatic disease. Evidence supporting the effectiveness of chemotherapy is, in many cases, unclear due to a wide variety of prognostic factors and small sample size.
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