Fibrosarcoma

Fibrosarcoma is a tumor of mesenchymal origin composed of malignant fibroblasts and collagen. It may appear as a soft tissue mass or a primary or secondary bone tumor. The two types of fibrosarcoma of bone are primary and secondary. Primary fibrosarcoma is a fibroblastic neoplasm that produces variable amounts of collagen. Origin may be central in the bone marrow or peripheral channel starting in perost. Secondary fibrosarcoma of bone arises out of a preexisting lesion or after radiotherapy of soft tissue and bone areas. It is an aggressive tumor with a negative prognosis.There is no known definitive cause of fibrosarcoma, although genetic mutations play a role. Several hereditary syndromes are associated with neurofibromatosis-sarcomas. Fibrosarcoamelor increased incidence has been reported in association with metal implants used for reconstruction of fractures or joints. The cause of this transformation is unknown.
Fibrosarcoma is only 10% of musculoskeletal sarcomas and less than 5% of primary tumors of bone. It is more common in men than in women. It can be diagnosed in patients of all ages but occurs most frequently in the fourth decade of life. It is located in the lower extremities, especially the tibia and femur.Fibrosarcoma of soft tissues usually affects a wider variety of patients from that of bone, with an average age of 35-45 years.Appears on facial soft tissues of the posterior thigh and knee. It is a large mass, painless deep fascia with bad edges defined. There is also a form of infantile fibrosarcoma. Unlike the adult has an excellent prognosis, even in front of metastasis at presentation, when treated with chemo therapy and resection.Sarcomas involving the bones often present with pain and swelling after a long period of evolution. They can grow enough to threaten the structural integrity of bone and cause pathological fractures.
Radiation and chemotherapy can improve the appearance and local control of tumor extension. Chemotherapy is used in bone lesions. Radiotherapy is used in correlation with fibrosarcoamele surgery for soft tissue, with or without chemotherapy.Fibrosarcoma Treatment involves a combination of local control of tumor extension and metastatic disease by avoiding surgical techniques can preserve bone and totally or partially affected limb or amputate. Local Recurrence of the tumor occurs in 60% of cases and pre-or postoperative radiotherapy is recommended.Local Recurrence is reduced to 255 when postoperative irradiation is used.Like other sarcomas, prognosis and evolution of cancer depends on many interrelated factors. These include tumor size and location, or histological grade and the presence of metastatic lung disease. These factors are important in assessing tumor-staging and determining the success of treatment, secondary prevention of disease extension.
Fibrosarcoma - PathogenesisFibrosarcoma etiology has not yet defined researcher. Genetic mutations are considered incriminating, metal implants in joints and bone fractures and sarcomatous association with syndromes.Fibrosarcoamele secondary lesions are observed and background, such as heart and lesions associated with fibrous dysplasia, chronic osteomyelitis and Paget's disease of bone or irradiated areas. This form of fibrosarcoma is very aggressive and is associated with a poor prognosis than primary.
The tumor may present different degrees of differentiation: differentiated-low grade, intermediate and high-anaplastic neoplasia. Depending on the differentiation of tumor cells may be similar to mature fibroblasts, secrete collagen, with rare mitoses.These cells are arranged in short bundles divide and come together, giving the appearance of fish bones. Poorly differentiated tumors are composed of atypical cells, pleomorfe, giant, multinucleated, with numerous atypical mjitoze and reduced production of collagen. This immature blood vessels without endothelium favors-sarcomatous marrow metastasis.