What are the other Names for this Condition? (Also known as/Synonyms)
- GCF (Giant Cell Fibroblastoma)
- Giant Cell Fibroblastoma of Children
What is Giant Cell Fibroblastoma? (Definition/Background Information)
- Giant Cell Fibroblastoma (GCF) is a rare, low-grade sarcoma that occurs in children
- These tumors can be locally aggressive, meaning that the tumor may spread to local areas
- It is a slow-growing tumor, which can be treated through a complete surgical excision
Who gets Giant Cell Fibroblastoma? (Age and Sex Distribution)
- Giant Cell Fibroblastoma is a rare tumor that occurs in children, less than 10 years of age
- Both males and females are affected by the tumor; though it is more common in males than females
- No racial/ethnic preference, or geographical restriction is observed
What are the Risk Factors for Giant Cell Fibroblastoma? (Predisposing Factors)
No definitive risk factors have been identified for Giant Cell Fibroblastoma occurrence.
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 Giant Cell Fibroblastoma? (Etiology)
- The exact cause for the development of a Giant Cell Fibroblastoma tumor is unknown.
- The tumor is supposed to arise from certain cells in the body, called fibroblasts
- Studies have shown that GCF tumors possess certain abnormal genetic mutations
- The tumor is related to dermatofibrosarcoma protuberans, which also has similar genetic mutations. Giant Cell Fibroblastoma tumor is considered (by some) to be a variant of this dermatofibrosarcoma protuberans, which commonly occurs in young individuals
- Currently, research is underway to improve our understanding of the GCF tumor
What are the Signs and Symptoms of Giant Cell Fibroblastoma?
No particular signs and symptoms are typically associated with Giant Cell Fibroblastoma tumors. However, the following may be observed:
- GCF is slow-growing tumor of the skin, which is generally painless. The tumor is firm to touch and occasionally may be painful (upon touching)
- It commonly occurs in the chest, back, shoulder, head and neck region, arms and legs
- The skin over the tumor is usually not pigmented or discolored. Normally, the skin is also not ulcerated, unless the tumor has progressed to an advanced stage
How is Giant Cell Fibroblastoma Diagnosed?
The diagnostic tests performed for Giant Cell Fibroblastoma include:
- A thorough physical examination and evaluation of medical history
- A biopsy of the mass/tumor: The mass is examined by a pathologist under a microscope to arrive 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 Giant Cell Fibroblastoma?
The possible complications of Giant Cell Fibroblastoma include:
- There may be a rapid growth of the tumor resulting in ulceration of the overlying skin with the tumor. This may invade into surrounding tissue causing tissue damage. However, distance metastasis of the tumor from its primary site is not reported
- The tumor may recur after surgical excision if incompletely removed
How is Giant Cell Fibroblastoma Treated?
Giant Cell Fibroblastoma is treated as follows:
- Complete surgical excision of the tumor is generally considered to be curative
- If the tumors are not completely removed, then they may recur after a period of time. Hence, it is important to completely remove them through surgery
- If the tumor has advanced into local structures, then a radiation therapy may be considered by your physician
How can Giant Cell Fibroblastoma be Prevented?
Currently there are no mechanisms to prevent Giant Cell Fibroblastoma occurrence. Prompt treatment and early tumor recognition will help in having an optimal outcome.
What is the Prognosis of Giant Cell Fibroblastoma? (Outcomes/Resolutions)
- Giant Cell Fibroblastoma is considered to be a low-grade sarcomatous malignancy, meaning that the tumor has a tendency to recur after removal. It also has a tendency to invade into surrounding tissues
- When the tumor is diagnosed early, a complete excision of the tumor is considered curative. If GCF is recognized at a later stage, then chances of its recurrence, increases after surgery
- Early diagnosis and treatment results in an excellent prognosis
Additional and Relevant Useful Information for Giant Cell Fibroblastoma:
Giant Cell Fibroblastoma (GCF) is considered to be a variant of dermatofibrosarcoma protuberans (DFSP). Both tumors have very similar genetic mutations. The main difference between the two is that unlike GCF, which usually occurs within the first 10 years of life, DFSP occurs during the later stages. Both tumors are usually treated in the same manner and have a similar prognosis.
- The pathologist may perform additional immunostains to help diagnose the tumor. The tumor is positive for CD34 immunostain and negative for S100 protein, smooth muscle actin, von Villibrandt factor, and CD31
- The above immunostain profile helps in arriving at a definitive diagnosis, by distinguishing a Giant Cell Fibroblastoma from other tumors, which may present similar findings under a microscope