Desmoid tumor in children and adolescents: The influence of age

Desmoid tumor is a soft tissue neoplasm that can occur in children as well as adults. Formally classified as an intermediate-grade neoplasm, it is known to have a locally invasive growth that can lead to severe and sometimes life-threatening problems. The historical standards of therapy for desmoid tumor center on surgical resection, with or without radiation therapy. However, depending on the site or size of the disease, surgical resection may substantially compromise form or function, making its use especially problematic in children who may be physically and psychologically immature. Further, there is some evidence that radiation is less effective against desmoid tumor in children, as compared to adults with this disease. As such, the treatment of a child with desmoid tumor may present unique problems. In this chapter, we review the pathology, biology, clinical presentation, and treatment of children with desmoid tumor as we highlight the similarities and differences from desmoid tumor in adults.