About bone and soft tissue sarcomas in children
What is a bone or soft tissue sarcoma?
A bone or soft tissue sarcoma is a tumor of bone, muscle or soft tissue that can come from many different types of tissue and affect any part of the body.
Our team of childhood cancer experts cares for all types of sarcomas from the most common, osteosarcoma, to rare tumors such as desmoplastic small round blue cell tumor. We are dedicated to treating the whole child, not just the disease.
What types of bone and soft tissue sarcomas do you treat?
Types of bone/soft tissue sarcomas we treat include:
Osteosarcoma (osteoblastic, chondroblastic, fibroblastic, telangiectatic, parosteal, periosteal)
Osteosarcoma is the most common type of bone cancer, affecting 400 children, teens and young adults in the U.S. a year.
Osteosarcoma is a type of cancer that begins in the cells that form your bones. It most often affects the long bones of the legs or arms, but can form in any bone in the body.
Osteosarcoma can cause pain and/or swelling in the bone or joint that is affected. It can also cause fractures as it weakens the bone. It is diagnosed initially by a tissue sample after an x-ray or MRI shows the tumor. For complete staging, a chest CT and bone scan or PET/CT is needed to look for distant metastasis.
Some inherited bone conditions can raise the risk of osteosarcoma. Children treated with radiation for another cancer also have a higher risk of developing osteosarcoma, especially very young children. The disease is more common in boys.
We screen all children after radiation and those with certain known genetic mutations like hereditary retinoblastoma, Bloom syndrome, Li-Fraumeni syndrome, Rothmund-Thomson syndrome and Werner syndrome.
Osteosarcoma is treated with chemotherapy and surgical resection.
Ewing sarcoma is a rare form of cancer that can affect bone or connective tissues. It most often affects children and teenagers. The areas most commonly affected are the pelvis, ribs, arms, shoulder and thigh bones, but it can also be found in the soft tissue. It affects 300-400 people in the United States each year and is slightly more common in boys.
It can present with pain and swelling, sometimes fever or a broken bone.
It can be first seen on an x-ray or MRI of the affected area. A small sample of the tumor is taken and we look at the cells and DNA.
To stage the tumor, we then require imaging of the lungs and PET/CT to look for distant metastasis, as well as a bone marrow biopsy to look for bone marrow involvement.
Ewing’s is quite different from osteosarcoma, as the two cancers come from vastly different cells and the genetics of the two are very different.
Ewing’s and Ewing’s like sarcomas often have a gene translocation. A translocation is when two pieces of DNA in different genes break and come together to form a new gene. In Ewing’s this is typically EWS/FLI1. There are others, and in Ewing’s like sarcomas, the most common are CIC-, and BCOR-.
Ewing’s and Ewing’s like sarcomas are treated by chemotherapies, surgery and/or radiation. They are very responsive to radiation therapy, while osteosarcoma is not.
Rhabdomyosarcoma (Embryonal, Alveolar, Botryoid, Pleomorphic)
Rhabdomyosarcoma (RMS) is a type of sarcoma that forms in the muscles. It is a very rare tumor affecting 350 children, teens and adults in the U.S. It often occurs before the age of 5, more often in boys than girls.
There are two different types and grades of rhabdomyosarcoma and they require different treatment approaches: Embryonal rhabdomyosarcoma (the more common type) and Alveolar rhabdomyosarcoma.
A sample of the tumor, also called a biopsy, or the complete tumor is removed to look at the genes and make-up of the cells. Imaging tests may include CT scanning, MRI, PET scanning and bone scans. Sometimes we will also examine bone marrow if needed.
Treatment depends on the type and stage of rhabdomyosarcoma.
Typically, people with low-risk rhabdomyosarcoma need less therapy than those in the higher-risk groups. Alveolar rhabdomyosarcoma may need more intensive treatment than the embryonal type.
After biopsy or complete removal, patients are given chemotherapy to shrink or treat the remaining cells. We follow this by surgery to completely remove the remaining tumor. Then patients will likely undergo intensity-modulated radiation therapy (IMRT), a very precise form of radiation therapy.
There are known inherited cancer syndromes that predispose children to develop RMS like Li-Fraumeni syndrome, neurofibromatosis type I and Costello syndrome. Early identification can help with future screening for other tumors.
Nonrhabdmyosarcoma soft tissue sarcomas (NRSTS) include a large group of different types of soft tissue sarcomas.
These can occur in any connective tissue in the body including bone, muscle, cartilage, blood vesicles and fat cells.
The diagnostic accuracy is improving due to the availability of immunohistochemical and molecular markers.
Surgery is the most common treatment since many of them are insensitive to chemotherapy, but with improved molecular markers we are also able to treat some with targeted agents.
Learn more about our nationally ranked cancer care