Overcoming challenges to treating invasive desmoid tumors in children
Desmoid refers to close-knit fibers or bundles. Similarly, desmoid tumors are dense, fibrous masses that form in the body’s connective tissues, which bind and support other tissues and organs. Also known as aggressive or desmoid-type fibromatosis, these tumors are generally quite rare, but their incidence peaks between the ages of six and 15. Adolescent girls are more susceptible to these invasive growths. In pediatrics, desmoid tumors are often found in the abdominal area, though infants and very young children are more likely to have head and neck involvement (a rare site for adults).
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Are desmoid tumors cancerous?
These soft-tissue tumors are non-cancerous or benign. However, these growths can be uniquely challenging to treat and can still present health threats and quality of life issues. These scar tissue-like masses are known for their aggressive growth and rate of recurrence following surgery to excise or remove the tumor. They can be dangerous and are associated with many uncomfortable and distressing symptoms when they compress the blood vessels, kidneys, intestines, lungs, and other organs. They tend to intertwine with the surrounding tissues, which adds to the complexity of removing them.
What do desmoid tumors look and feel like?
Symptoms can vary significantly based on the location and size of the tumor. They are known to be slow-growing and painless. So, they tend to go undiagnosed in children and are more likely to be detected in young adults as the tumor grows and causes more dramatic and noticeable changes. Your child may have a single, rubbery-feeling, swollen area or lump. The lump may not hurt. If the tumor is pressing against the nerves or muscles, it can be painful and cause soreness. When desmoid tumors affect the legs, it can alter your child’s gait. A common site, desmoid tumors of the abdominal region, are also associated with symptoms like cramping, nausea, and rectal bleeding.
How do these tumors present in the head and neck region?
Infants and younger children, in particular, may develop solid, immobile masses in sensitive areas such as their cheeks and neck. X-rays may reveal that this growth has spread to surrounding areas, such as the upper lip. Children may also experience functional changes such as trismus, which refers to jaw stiffness and muscle spasms in the jaw joints.
What causes desmoid tumors?
The exact cause of desmoid tumors is not known. Certain genetic conditions like Garner syndrome are associated with the development of these growths. Other factors that may play a role include trauma (such as past/repeated surgeries) and estrogen, which may explain the higher incidence of desmoid tumors in younger girls/teens and premenopausal women (up to the age of 40).
What are the best options for treatment?
For infants and young children with desmoid tumors, the standard treatment is surgical excision. Surgery to remove the tumor may sometimes be followed by radiation therapy. Select cases may also benefit from medications/pharmacological treatment. Chemotherapy may be reserved for certain types of challenging or recurrent desmoid tumors, such as those in deep-seated, extra-abdominal areas (outside of the belly and abdominal wall). As always, we must account for factors such as the location of the tumor when making recommendations for care. Here again, surgical resection/removal is generally the best approach for kids when the tumor can be removed without considerable negative and lasting cosmetic and functional complications.
With your support, the Pediatric Head and Neck Cancer Foundation is advancing research on clinical care guidelines and setting a new standard in the treatment of desmoid tumors and cancerous HN tumors that account for the distinctive needs of children. To donate your time, talent, or treasure or to connect with a team member for resources to support your family or clinical practice, contact us today at pediatricheadneckcancer@gmail.com