Treatment of Infantile fibrosarcoma in children
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University Hospital Heidelberg
Department of Pediatric Oncology, Hematology, Immunology and Pulmonology
University Hospital RWTH Aachen
Department of Pediatric and Adolescent Medicine
University Hospital Hamburg-Eppendorf
Department of Pediatric Hematology and Oncology
University Hospital Würzburg
Department of Pediatric and Adolescent Medicine
Charite University Hospital Berlin
Department of Pediatric Oncology and Hematology
University Hospital Tuebingen
Department of General Pediatrics, Pediatric Hematology and Oncology
University Hospital Bonn
Department of Pediatric Hematology and Oncology
University Hospital Duesseldorf
Department of Pediatric Oncology, Hematology and Clinical Immunology
University Hospital Muenster
Department of Pediatric Hematology and Oncology
University Hospital Halle (Saale)
Department of General Pediatrics, Pediatric Hematology, Oncology and Neuropediatrics
University Hospital Jena
Department of Pediatric and Adolescent Medicine
University Hospital Marburg UKGM
Department of Pediatric and Adolescent Medicine
University Hospital Rechts der Isar Munich
Department of Pediatrics and Adolescent Medicine
University Hospital Essen
Department of Pediatric Oncology, Hematology, Pulmonology, Cardiology and Rheumatology
University Hospital Erlangen
Department of Pediatric and Adolescent Medicine
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.
- 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
- 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.
- 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.
Authors: Dr. Nadezhda Ivanisova, Dr. Farrukh Ahmed