What are the other Names for this Condition? (Also known as/Synonyms)
- AFS (Adult Fibrosarcoma)
- Fibrosarcoma of Soft Tissue - Adult
What is Adult Fibrosarcoma? (Definition/Background Information)
- Adult Fibrosarcoma (AFS) is a malignant tumor that is typically seen in middle-aged and older adults. It is an aggressive and high-grade tumor (in most cases), implying that it has a high malignancy potential for metastasis
- There are no specifically identified risk factors for Adult Fibrosarcoma, but it has been seen to occur in a background of radiation therapy and surgical implants
- The most common site of the tumor is the arms and legs followed by the trunk. Large Adult Fibrosarcomas can cause hemorrhaging/bleeding
- The treatment of Adult Fibrosarcoma may include a combination of surgery, chemotherapy and radiation therapy, based on the assessment by the healthcare provider
- However, despite appropriate therapy, the prognosis of Adult Fibrosarcoma is poor. The prognosis is dependent upon a set of factors that includes the size, location, and grade of the tumor
Who gets Adult Fibrosarcoma? (Age and Sex Distribution)
- Most cases of Adult Fibrosarcoma are seen in middle-aged and older adults in the 40-60 year age group (average age of 56 years). It can be occasionally seen in children
- Adult Fibrosarcoma constitutes 1% to 3.5% of all soft tissues sarcomas. Earlier, AFS was believed to be a common soft tissue sarcoma in adults
- Both males and females are affected equally; but, there is a slight male predilection according to some studies
- All racial and ethnic groups may be affected
What are the Risk Factors for Adult Fibrosarcoma? (Predisposing Factors)
Currently, there are no specific risk factors associated with Adult Fibrosarcoma.
- Sometimes, a history of radiation therapy has been observed
- AFS has also been observed against a background of surgical implants (foreign body)
It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.
Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.
What are the Causes of Adult Fibrosarcoma? (Etiology)
The exact cause of Adult Fibrosarcoma formation is not known. However, chromosomal abnormalities have been detected in several tumors.
What are the Signs and Symptoms of Adult Fibrosarcoma?
The signs and symptoms of Adult Fibrosarcoma may include:
- Most tumors are asymptomatic. However, in others, a wide-range of behavior is observed
- The tumors may grow at a slow rate
- Presence of a firm mass with an absence of pain. Large-sized tumors that compress other organs may cause pain
- The size of the tumor may range from a few cms to over 20 cm
- They are mostly found in the deep tissues of the limbs, chest and back, head and neck region
- In very rare cases, tumors are also observed in the retroperitoneum
- Most AFS tumors are high-grade sarcomas (some studies indicate that over 80% of the tumors may be of high-grade)
How is Adult Fibrosarcoma Diagnosed?
A diagnosis of Adult Fibrosarcoma is typically established by eliminating the possibility of other tumors. It is a diagnosis through exclusion and may involve the following tests and exams:
- A physical examination and evaluation of complete medical history
- Imaging studies, such as X-rays, CT and MRI scans, of the affected region
- Tissue biopsy of the tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.
What are the possible Complications of Adult Fibrosarcoma?
The possible complications from Adult Fibrosarcoma include:
- Emotional stress due to a concern of cancer
- High-grade tumors may show hemorrhage (bleeding)
- Surgically excised tumors are known to return; the recurrence rate varies from 15% to 80%
- Low-grade tumors on recurrence may become high-grade sarcomas
- The rate of metastasis is about 25%; the lungs and axial skeleton bones (vertebral column, ribcage, bones in the head and skull) may be involved. Lymph node metastasis is rarely seen
- Damage to vital nerves, blood vessels, and surrounding structures during surgery to remove the tumor
- Side effects from chemotherapy and radiation therapy
How is Adult Fibrosarcoma Treated?
Treatment measures for Adult Fibrosarcoma may include the following:
- Aggressive lesions may require multi-disciplinary management
- Surgical excision of AFS with removal of the entire tumor is generally adopted
- Embolization is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
- Radiation therapy and/or chemotherapy may be used to destroy the tumor cells, based on the assessment by the healthcare provider
- Post-operative care is important; a minimum activity level is to be ensured until the surgical wound heals
- Follow-up care with regular screening and check-ups are important
How can Adult Fibrosarcoma be Prevented?
- Current medical research has not established a way of preventing Adult Fibrosarcoma
- Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory due to its high metastasizing potential and chances of recurrence. Often several years of active vigilance is necessary
What is the Prognosis of Adult Fibrosarcoma? (Outcomes/Resolutions)
The prognosis of Adult Fibrosarcoma also depends upon a set of several factors, which include:
- Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
- Grade of tumor: Higher grade tumors have worse prognosis than lower-grade tumors
- Location of tumor: Tumors at locations with potential to damage vital organs (such as tumors in the head and neck region) have poor prognosis than tumors that are located in the extremities (arms or legs)
- The surgical respectability of the tumor (meaning, if the tumor can be removed completely)
- Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
- Age of the individual: Older individuals generally have poorer prognosis than younger individuals
- Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
- Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
In general the prognosis of Adult Fibrosarcoma is poor, with survival rate of 70% and less over 2 years; 55% and less over 5 years.
Additional and Relevant Useful Information for Adult Fibrosarcoma:
Fibrosarcomas that occur in adults and children are histologically similar to each other.
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