Sarcoma in children:

What it is, overcoming challenges to diagnosis and treatment

Cancers of the head and neck account for one out of every five tumors affecting children. While sarcoma of the head/neck region is rare, this group of tumors presents unique challenges to proactive diagnosis and effective treatment. At the Pediatric Head and Neck Cancer Foundation, we seek to support early diagnoses and better treatments (with minimal risks) for all children, regardless of the type of cancer. With continued research and funding support, we can remove the present challenges to evaluating for and treating sarcomas.

What is sarcoma?

Derived from sarx, the Greek word for “flesh,” sarcoma is a type of cancer that develops in the body’s connective tissues—the bones, muscles, cartilage, and fat. While these cancers are largely classified as “soft tissue sarcoma” and “bone sarcoma,” there are more than 70 different sub-types. Each type is associated with different symptoms, characteristics, and treatment approaches, which can add to the complexity of diagnosing such cancers.

What is the most common type of sarcoma in children?

Rhabdomyosarcoma is the most common soft tissue sarcoma in children, as well as the most common sarcoma of the head and neck region. In fact, it is localized to HN sites in 40% of cases.  As it relates to bone tumors, the most common type that affects children, teens, and young adults is osteosarcoma.

How does rhabdomyosarcoma develop?

Rhabdomyosarcomas usually begins in the cells of the striated muscle or the muscles attached to the skeleton that support movement. There are four main types (embryonal, alveolar, sclerosing, and pleomorphic). Embryonal represents the type that is most likely to occur in the head and neck region. This is also the most common type of rhabdomyosarcoma, which usually originates in infants and very young children. Children with certain genetic disorders are at elevated risk of developing embryonal rhabdomyosarcoma, which can manifest as a persistent and growing lump or swelling, bulging or crossed eyes, headaches, and nose bleeds.

And how does osteosarcoma develop?

Osteosarcoma tends to affect older children during periods of growth spurts. The pain and swelling associated with this bone cancer may be mistakenly attributed to “growing pains” or to sports injuries among active adolescents and teens. While it most commonly affects the long bones of the legs (the bottom of the thigh bone and the top of the larger shin bones), 6% to 10% of pediatric osteosarcomas start in the head and neck. HN osteosarcomas in children usually originate in the lower jaw and, less frequently, the upper jaw. Taller boys with certain bone diseases or rare genetic syndromes are at greater risk of developing this cancer.

How are sarcomas generally treated in children?

Specific treatment recommendations are guided by many factors, not limited to the type of sarcoma but also to its “stage” or how much it has grown. Since there are so many different subtypes, treatments can vary significantly. Complete surgical resection or removal of the cancer continues to be among the most relevant factors affecting a child’s prognosis. However, sarcomas like rhabdomyosarcoma tend to be “unresectable,” which adds to the challenges of treatment. In these cases, such tumors may require radiation therapy. Traditionally, therapies like radiation presented their own unique concerns (such as immediate and long-term side effects). Still, new technologies like computer-assisted surgery or intraoperative navigation are helping minimize acute and long-term effects from surgical treatment. 

How can I help children with sarcoma?

You can support the Pediatric Head and Neck Cancer Foundation’s efforts by contacting our team today at pediatricheadneckcancer@gmail.com. With your help, we can be catalysts for change to fight the childhood HN cancer crisis. We also welcome hearing from any individuals or families affected by HN cancers. We strive to be a support system for all.