Fibrosarcoma

Fibrosarcoma: Description, Causes and Risk Factors: FibrosarcomaA malignant neoplasm derived from deep fibrous tissue, characterized by bundles of immature proliferating fibroblasts arranged in a distinctive herringbone pattern with variable collagen formation, which tends to invade locally and metastasize by the bloodstream. Fibrosarcoma is a malignant neoplasm of the fibroblastic origin. It can occur in any location being the bone extremities the main affected site. Fibrosarcoma is found most commonly around the knee in the distal femur and proximal tibia followed by the pelvis. It presents in adults age 30 to 60 years old and affects men and women equally. Occurrences in the maxilla are rare with an incidence ranging from 0 to 6.1% of all primary fibrosarcoma of the bone. Fibrosarcoma rarely affects the jaws bone. It is a tumor of mesenchymal origin and the epidemiologic factors are still unknown, but many authors report radiation therapy history as a possible one. Fibrosarcoma can arise in soft tissues or within bone. Intraosseous fibrosarcomas may develop endosteally or possibly periosteally, the latter affecting bone by spread from adjacent soft tissue. Occasionally, the lesion erodes the roots of involved teeth. Fibrosarcoma can be graded in low and high grade of malignancy. Low grade fibrosarcoma show spindle cells arranged in fascicles with low-to-moderate cellularity with a herringbone appearance. There is a mild degree of nuclear pleomorphism and rare mitosis, with a collagenous stroma. High-grade lesion showed an intense nuclear pleomorphism, greater cellularity, and atypical mitosis. The nuclei can be spindle shaped, oval or round nuclei. The prognosis depends on the history, clinical signs, and characteristics of the fibrosarcoma present. Small tumors with aggressive surgical resection may never recur. Larger tumors, or tumors that are incompletely resected, will certainly recur and become locally aggressive within a matter of months after surgery. With a combination of radiation, surgery, and chemotherapy, however, the long term prognosis is much improved with remission periods in the 2-3 year range. Symptoms: The symptoms depend on the area of the body where the tumor is located.Clinically the lesion can cause pain, swelling, paraesthesia.Typically, the only early symptom is a slight pain in the affected area. As the disease progresses, a noticeable lump or mass will become apparent and it is tender to touch. Diagnosis: Diagnosis of fibrosarcoma is made by biopsy of the tumor. This is a surgical procedure done under general anesthesia. The suspicious mass or pieces of it are removed and looked at under a microscope. Other tests may be performed to see if the disease has spread including a CT and MRI scans, radioisotope scans and/or a bone marrow aspirate. Treatment: Surgery is the first-line treatment for fibrosarcoma. Because the tumor is locally aggressive, wide surgical margins are essential. Even with wide surgical margins, local recurrence is common. For this reason, adjuvant therapy is often indicated. Radiation therapy is the primary mode of recommended adjuvant therapy and may be performed prior to or after surgery. In addition to this, chemotherapy can have additional beneficial effects. The chemotherapy drugs used for this disease generally cause few evident side effects and are well tolerated. NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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