About the disease
Infantile fibrosarcoma is a congenital malignant tumor, which develops in soft tissue and most commonly manifests itself in a child before the age of 1. Infantile fibrosarcoma is considered to be an aggressive tumor, as it spreads fast and can become large if not treated on time. Most commonly, tumor develops in arms and legs. Later, it can spread to the trunk, that`s why it's so crucial to find it early. In rare cases, infantile fibrosarcoma can develop in the chest or back. It is not common for infantile fibrosarcoma to develop in any organs, as it almost always appears in soft tissues. Although the exact cause of infantile fibrosarcoma has not been definitely identified, doctors believe that gene aberration is the leading cause. In children with infantile fibrosarcoma gene fusion of ETV6 and NTRK3 has been identified.
If infantile fibrosarcoma becomes large in size, a child can experience ulceration of the skin, followed by the bleeding of the place where tumor is located. According to DoveMed, approximately 36-80% of all cases of infantile fibrosarcoma has been diagnosed right after the birth of a child. This condition is slightly more common among males than females. Infantile fibrosarcoma can be very large in size, in some cases exceeding 25 cm in length.
Symptoms
- Tumor, most commonly in the area of extremities
- Ulceration and bleeding at the site where tumor is located
- Tumor grows very quickly
- Weight loss
- Mood swings
- Pain in the area of tumor
Diagnosis
- During a physical examination, the doctor will palpate the area where tumor is located. Most commonly, it can be felt deeply under the skin or be visible if tumor grew large. The doctor will also ask about a family history of a patient to determine if infantile fibrosarcoma could be inherited.
- An MRI and CT scan of the tumor help to determine its exact location, size and also dynamics of growth.
- A biopsy of the affected tissue is important for a conclusive diagnosis, as it helps the doctor to determine for sure if the tumor is benign or malignant.
Treatment
- Surgery resection is used for excision of the tumor. In some cases, several resections need to be performed to completely resect the tumor. Overall, this surgery has good prognosis and most children who underwent a surgery recover even if this tumor has been large. The most important prerequisite for a definite good prognosis is for a tumor not to spread to the internal organs.
- Radiation therapy and chemotherapy are used to shrink the tumor and prevent it from spreading faster. These two therapies are also very beneficial in case a tumor has spread to internal organs.
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