Fibrosarcoma

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List of terms related to Fibrosarcoma

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. Fibrosarcoma can be intramedullary or periosteal lesion of bone.

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 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 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.

Micrograph of a tumour with the herringbone pattern as may be seen in fibrosarcoma. H&E stain.

Epidemiology and Demographics

Fibrosarcoma makeup about 5% of all primary bone sarcomas. Fibrosarcoma is found most commonly around the knee in the proximal tibia and distal femur. 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 pathologic 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 images such as

• Osteosarcoma
• chondrosarcoma
• leiomyosarcoma
• malignant fibrous histiocytoma
• Myofibromatosis
• Desmoplastic fibroma

Intra-osseous fibrosarcoma that affects the jaw must be differentiated from odontogenic sarcomas like

• 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

The lungs are most common site of sarcoma metastasis.To determine if there is metastasis of the tumor to the lungs, computed tomography (CT) of the chest is performed at initial diagnosis.

MRI

Imaging of the entire bone with lesion is necessary to determine the lesions extended beyond the area delineated by X ray. The benefits of MRI Scans in Fibrosarcoma patients are the following

• Identify non-contiguous skip lesions that can arise within the same bone
• Determine the extent of bone marrow and soft tissue involvement

See also

• Fibrous connective tissue
• Fibroma
• Neurofibrosarcoma