What is Fibrosarcoma (Bone)?
Fibrosarcoma (Bone) is a fast-growing and spreading (malignant) tumour or excessive proliferation of fibroblasts. Fibroblasts are those cells that synthesize collagen and extracellular matrix. The extracellular matrix is composed of collagen, enzymes, and glycoproteins that provide structural and biochemical support to the surrounding cells. Fibrosarcoma (Bone) usually develops in the long bones, most often at the distal end of the femur.Disease Epidemiology
It accounts for 2% to 4% of primary bone tumours. The average age of development of bone fibrosarcoma is 70 years.Disease Etiology (Causes)
The cause is unknown, but certain genetic mutations and other risk factors such as previous exposure to radiation, long-term exposure to certain chemicals such as vinyl chloride, arsenic and dioxin, and a weak lymphatic system are responsible for the development of fibrosarcoma (bone).Signs and symptoms
The patient may suffer from the following signs and symptoms. These are;- Mass or swelling around the bone
- Persistent pain at the place of the tumour
- Difficulty in moving affected joint or limb ( arm or leg)
- Numbness of the limb or adjacent part of the body where the tumour is located
Diagnosis
Definite diagnostic tests to diagnose the fibrosarcoma bone are tumour biopsy, histopathology, and cytogenetic study of the specimen. Radiological techniques like CT scan, MRI, USG, and X-ray also recommended locating the size and site of the tumour.Treatment
- Simple, partial or radical Vulvectomy (surgical removal of the whole or tumour part of the vulva) with or without surgical removal of femoral and inguinal lymph nodes according to the extent of the tumour.
- Surgical excision with adjuvant local radiation therapy.
- Chemotherapy and radiotherapy are the treatment options for tumours that spread to the distant parts of the body and not confined to its site of origin.