Fibroblasts are the foundation cells, in your body; their job is to produce the fibrous tissue. More specifically, fibroblasts create the collagen fibres and assemble its cementing ground substance, which is present in almost every part of your body. When these fibroblasts start to proliferate uncontrollably, it becomes a fibrosarcoma. Put simply, a fibrosarcoma is a malignant tumour of these very fibroblasts.
It can occur in any location, either affecting the bone or the other soft tissues. When it affects the bones, the tumour by and large does so in an extremity, especially the lower extremities. The soft tissue fibrosarcomas occur mainly in the subcutaneous, muscular and tendinous tissues (tendons attach muscle to bones).
What causes fibrosarcoma ?
It is a tumor of mesenchymal origin. The etiology of these tumours is obscure. Trauma is frequently named as a causative factor, although there is little medical evidence to validate this. Previous irradiation for another pathological condition such as other cancers is a possible contributing factor.
Where does fibrosarcoma occur ?
Fibrosarcoma can arise in soft tissues or within bone. The tumours in the bone are called intra-osseous and the tumours found in the soft tissue are called extra-osseous. As the name suggests, intra-osseous fibrosarcomas may develop either inside the bone, or at the outer layers of bone. The outer layers of bone can be affected by spread from adjacent soft tissue.
What are the clinical features of fibrosarcoma ?
It occurs in any age group but it is common between 20-60 years. A fibrosarcoma in its earlier growth phase is often seen as an innocuous nodule.
The history is usually that of the development of a painless lump which easily escapes detection when it is small. In time, it steadily enlarges in size. When there is nerve encroachment or impingement on other vital structures, it may become painful. If it is removed inadequately, it may begin to grow very rapidly, hence adequate removal is important.
How is fibrosarcoma diagnosed ?
Radiologic features of fibrosarcoma
Radiologically, it is diagnosed through Magnetic resonance Imaging Tests or Computerized Tomography Scans.
Histologic features of fibrosarcoma
In histological (microscopic) examination, after cutting a biopsy out, the degree of differentiation can vary, from a benign fibroma, at one end to an exceedingly malignant tumour, at the other end. This makes it a difficult diagnosis.
Fibrosarcoma can be graded in low to high grades of malignancy.
Low grade fibrosarcoma shows spindle-shaped fibroblasts ordered in fascicles with low to moderate cellularity. The fascicles have a herringbone appearance. There is a mild degree of malignant cellularity. The tumour cells are surrounded by a collagenous stroma.
The High-grade lesion shows an intense nuclear pleomorphism, greater cellularity, atypical mitosis and is aggressive in behaviour.
Immunohistochemical features of fibrosarcoma
The positive, affirmative immunostaining for vimentin, combined with negativity for other immunomarkers help to diagnosis the fibrosarcoma.
What is the differential diagnosis of fibrosarcoma ?
The histological appearance of high-grade fibrosarcoma may be similar to other tumours, like malignant fibrous histiocytoma, liposarcoma or synovial sarcoma. Another lesion that is very similar to fibrosarcoma is fibromatosis. However, in fibromatosis, usually mitoses are lacking and cellular atypia is absent.
There may be significant complexity in distinguishing between a reparative process and fibrosarcoma, or between benign fibromatosis and fibrosarcoma, or between some forms of liposarcoma and fibrosarcoma.
How is fibrosarcoma treated ?
The treatment of choice in fibrosarcoma is its removal. This is done by radical surgery.
The other two forms of treatment, radiation therapy and chemotherapy can be used in the serious, untreatable, or non-resectable cases as a palliative treatment (it can make the symptoms better, but it is not curative). Prognosis is directly related to the tumour grade in histology, if its large or small, and whether or not adequate surgery has been done with margins free of tumour.
Fibrosarcomas are best treated by complete and comprehensive surgical excision. Inadequate excision in the original treatment is a key cause for failure.
How to find and reach cancer specialists for the treatment of fibrosarcoma ?
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Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.
