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.
Classification
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 may be 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 may be localized at the end of the long bones. Most often it affects the upper end of tibia or lower end of femur. 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. It affects men and women 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
Prognosis of fibrosarcoma is dependent on the tumor grade. It 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. Secondary sarcoma, presence of eccentric permeative lesions, primary tumor in the axial skeleton are 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
- Localized Pain
- Swelling
- Loss of range of motion
Pain with weight-bearing that is relieved by rest and night pain may be observed. Many patients with fibrosarcoma neither feel sick nor experience classic symptoms of cancer such as weight loss and fatigue. Sometimes, patients with fibrosarcoma may present with pathological fracture of affected bone.
Differential Diagnosis
Fibrosarcoma must be differentiated from other conditions that cause pain, swelling, and lesions that may appear similar to fibrosarcoma on radiological imaging such as
- Osteosarcoma
- Chondrosarcoma
- Leiomyosarcoma
- Malignant fibrous histiocytoma
- Myofibromatosis
- Desmoplastic fibroma
Intra-osseous fibrosarcoma that affects the jaw must be differentiated from odontogenic sarcomas such as
- Ameloblastic fibrodentinosarcoma
- Ameloblastic fibrosarcoma
- Odontogenic carcinosarcoma
Diagnosis
X Ray
The radiological picture of fibrosarcoma typically shows the osteolytic lesion with a permeative or moth-eaten appearance. Margins of lesion can range from well-demarcated to ragged appearance.
CT scan
CT scan is performed at initial diagnosis of fibrosarcoma to determine the metastasis of the tumor to the lungs. The lungs are most common site of sarcoma metastasis.
MRI
MRI of entire bone is necessary in fibrosarcoma patients. The benefits include
- Determine the extent of bone marrow and soft tissue involvement
- Identify non-contiguous skip lesions that can arise within the same bone
See also
References