Fibrosarcoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Malignant fibromatous neoplasm, fibroblastic sarcoma
Overview
Fibrosarcoma (fibroblastic sarcoma) is a malignant tumor derived from fibrous connective tissue and characterized by immature proliferating fibroblasts or undifferentiated anaplastic spindle cells.
Classificaion
Grade
Fibrosarcoma may be classified according to degrees of differentiation into three subtypes: low grade malignancy (differentiated), intermediate malignancy and high grade malignancy (anaplastic)
Stage
Fibrosarcoma is classified into 3 stages based on grade and extent of lesion.
- Stage I
Stage IA: Low grade lesion that is confined to its anatomic compartment
Stage IB: Low grade lesion that have extended outside of its compartment
- Stage II
Stage IIA: High grade lesion that is confined to its anatomic compartment
Stage IIB: High grade lesion that have extended outside of its compartment
- Stage III
lesions are any grade or anatomic site that have metastasized
Pathophysiology
Gross Pathology
The tumor is typically well demarcated but not encapsulated.
Microscopic Pathology
Tumor cells may resemble mature fibroblasts (spindle-shaped), secreting collagen, with rare mitoses. These cells are arranged in short fascicles which split and merge, giving the appearance of "fish bone". Poorly differentiated tumors consist in more atypical cells, pleomorphic, giant cells, multinucleated, numerous atypical mitoses and reduced collagen production. Presence of immature blood vessels (sarcomatous vessels lacking endothelial cells) favors the bloodstream metastasizing. The malignant cells are Characteristically arranged in a "herringbone" pattern.
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Epidemiology and Demographics
Fibrosarcoma makeup about 5% of all primary bone sarcomas. Men and women affected equally.
Incidence
Fibrosarcoma is a rare disease that tends to affect approximately one among two million people annually.
Age
Patients of all age groups may develop Fibrosarcoma, but are most common between the third and sixth decades of life. In infants, fibrosarcoma is usually congenital. Infants presenting with fibrosarcoma usually do so in the first two years of their life.
Natural History, Complications, and Prognosis
Prognosis
Fibrosarcoma is associated with a 5 year survival rates of 30% among patients with high grade medullary lesions and 50-80% in surface fibrosarcomas and low grade fibrosarcomas. The presence of eccentric permeative lesions, primary tumor in the axial skeleton is associated with a particularly poor prognosis among patients with fibrosarcoma.
Risk Factors
Common risk factors in the development of Fibrosarcoma are preexisting benign lesions such as giant cell tumor, enchondroma, fibrous dysplasia, bizarre parosteal osteochondromatous proliferation or chronic osteomyelitis. Other risk factors include Paget's disease, radiation therapy, surgically treated fracture and infarction of bone.
Screening
There is no known screening test available for early diagnosis of Fibrosarcoma.
History and Symptoms
The most common symptoms of Fibrosarcoma include Pain, swelling and loss of range of motion. Pain with weight-bearing that is relieved by rest and night pain may be observed. Many patients with sarcoma neither feel sick nor experience classic symptoms of cancer such as weight loss and fatigue.
See also
References