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Undifferentiated pleomorphic sarcoma (UPS), also termed pleomorphic myofibrosarcoma, [1] high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma, [2] is characterized by the World Health Organization (WHO) as a rare, poorly differentiated neoplasm (i.e., an abnormal growth of cells that have an unclear identity and/or cell of origin). [3]
Low-grade sarcomas have a better prognosis than higher-grade sarcomas, and are usually treated surgically, although sometimes radiation therapy or chemotherapy are used. [3] [4] Intermediate- and high-grade sarcomas are more frequently treated with a combination of surgery, chemotherapy, or radiation therapy. [15]
In one review of 109 individuals with MFS: overall survival for the entire group was 80% at 3 years and 76% at 5 years; local recurrence-free survival was 95% at 3 years and 88% at 5 years; median survival following local recurrence was 68 months; distant metastasis-free survival was 78% at 3 and 77% at 5 years; and 18 of 25 patients (72%) died ...
Spindle cell sarcoma is a type of connective tissue cancer. The tumors generally begin in layers of connective tissue , as found under the skin, between muscles, and surrounding organs, and will generally start as a small, inflamed lump, which grows in size.
Features associated with poor prognosis include a large primary tumor (over 5 cm across), high grade disease, co-existent neurofibromatosis, and the presence of metastases. [5] It is a rare tumor type, with a relatively poor prognosis in children. [6] In addition, MPNSTs are extremely threatening in NF1.
These tumors display high heterogeneity and extremely poor differentiation. The pleomorphic cells may be diffuse or localized, with the diffuse variation correlating to a worse prognosis. [16] It occurs most often in adults, rarely in children, and is often discovered in the extremities.
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