Fibrosarcoma is a spindle cell malignant neoplasm originating from fibroblasts. It can occur as a soft tissue tumor or as a bone tumor, both primary and secondary. What are the causes of the development of fibrosarcoma? What are the symptoms of fibrosarcoma and how is it treated?
Fibrosarcoma( fibrosarcoma ) is a rarely diagnosed cancer, accounting for only about 3 percent of adult sarcomas. It is much more often diagnosed in middle-aged and older people than in children and adolescents. Men get sick more often.
Cancer, growing in tissues, creates extensive, deep, compact masses. It is characterized by slow growth, so it often takes many years before it is diagnosed.
About 20 percent of these cancers develop secondary to pre-existing bone changes, such as Paget's disease, after radiation therapy, or secondary to bone infarction.
Fibrosarcoma typically grows in the epiphyses of long bones, flat bones, and deep tissues of the lower limbs, but it can be found anywhere in the body.
Fibrosarcoma (fibrosarcoma): causes
So far, no factors that could predispose to the occurrence of fibrosarcomas have been discovered. However, it is believed that irradiation of the body may have an impact on tumor growth.
Worse course of the disease in adults and adolescents may be caused by the presence of translocations (12, 13).
Fibrosarcoma (fibrosarcoma): symptoms
Typical symptoms of fibrosarcoma depend on the location and histopathological type of the tumor. Most often it is a painful or not pathological mass, visible and palpable through the body shell.
Sometimes the first symptom of the disease is a pathological fracture of the bones covered by the neoplastic process. The skin above the lesion may be red and tense, sometimes with ulcerations.
Fibrosarcoma in children
Fibrosarcoma is the most common soft tissue sarcoma in children under one year of age, and is rarely diagnosed in children over 2 years of age.
There have been cases of congenital cancer, already diagnosed during prenatal ultrasound examinations.
Fibrosarcoma is more common in boysthan in girls and has a good prognosis.
The tumor typically develops in the tissues of the lower limbs, upper limbs and the trunk, less often in the head and neck area and in the retroperitoneal space.
Unlike adult fibrosarcomas, the pediatric type rarely produces distant metastases, but in rare cases these may involve the lymph nodes, the lungs, and the retroperitoneal space.
The treatment of choice is the surgical removal of the neoplastic tumor. In the case of a tumor that is too large or distant metastases, chemotherapy is used.
Fibrosarcoma (fibrosarcoma): diagnosis
The doctor's clinical picture and the results of the patient's imaging tests lead to the suspicion of a neoplastic disease. The following are used in initial imaging diagnostics:
- ultrasonography
- computed tomography
- magnetic resonance imaging
The latter is the most accurate and best illustrates changes in soft tissues.
Based on these results, it is only possible to determine whether there is a tumor in the tissues and what area it covers, while to determine the type of tumor it is necessary to extend the diagnosis by histopathological examination of the tissue material.
Core-needle biopsy is most often used to obtain a tumor fragment. It is an examination performed under local anesthesia.
The obtained material is marked with immunohistochemical methods and assessed by a pathomorphologist under an electron microscope.
Fibrosarcoma (fibrosarcoma): differentiation
Fibrosarcomas should be differentiated from other malignant neoplasms, such as:
- liposarcoma (liposarcoma)
- desmoid tumors
- fibromatosis
- synovial sarcoma
- low grade myxosarcoma.
Fibrosarcoma (fibrosarcoma): clinical stage
The clinical stage of fibrosarcoma is assessed according to the international TNM classification.
It specifies 3 parameters:
- size of the primary tumor (T - tumor)
- occupying nearby lymph nodes (N - node)
- presence of distant metastasis (M - metastasis).
Based on the TNM classification, the optimal method of cancer treatment is determined.
Fibrosarcoma (fibrosarcoma): treatment
Treatment of fibrosarcoma consists of surgical resection of the tumor, chemotherapy and / or radiotherapy (combination therapy).
The initial treatment of choice is a wide tumor resection with an appropriate marginhe althy tissues. In some cases, this may mean the amputation of an affected limb.
Chemotherapy is used in the advanced stages of the disease in neoadjuvant, adjuvant and palliative treatment.
In cases of high malignancy and large primary tumor size, pre- and postoperative chemotherapy is used.
In the treatment of chemosensitive tumors, the first-line regimen is vincristine and dactinomycin. Irradiation may be indicated in palliative treatment or in the treatment of recurrence.
Fibrosarcoma (fibrosarcoma): prognosis
The prognosis of patients with fibrosarcoma depends on many factors, including from:
- primary tumor location
- its size
- histological type
- differentiation
- of the mitotic index
- cellular
- presence of necrosis
- presence of distant metastases.
Fibrosarcomas can be composed of more or less differentiated cells, which determines their level of malignancy.
Less differentiated tumors are more malignant than those composed of well-differentiated cells.
Adult fibrosarcoma is a much worse prognosis than childhood fibrosarcoma.
After surgical treatment and local tumor resection, recurrences may occur in over 50% of patients.
Blood metastases are seen in more than 25 percent of patients, usually involving the lungs and the axial skeleton.
5-year survival is 41 percent, 10-year survival is 29 percent.
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- Synovial sarcoma
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