Management of rapidly growing recurrent extra-abdominal pediatric desmoid tumor: case report

: Desmoid tumors are rare, benign soft tissue tumors that occur spontaneously, or less frequently, in patients with a genetic predisposition. With an unpredictable course, desmoid tumors are often locally aggressive and have a high rate of recurrence even after complete surgical excision. Desmoid tumors often become a chronic problem for patients, making them a major source of morbidity, and may even lead to mortality. Management of these tumors can be frustrating, with patients often requiring multiple modes of therapy in pursuit of a cure. We present a patient with a history of familial adenosis polyposis (FAP) who presented early on in life with an extra-abdominal desmoid tumor that proved to be a major source of morbidity during his childhood. We review a chronological approach to the management of our patients’ tumor with the resulting outcomes following each mode of therapy, which included observation, surgical resection, systemic therapy, and radiotherapy. Additionally, we review the currently literature with regards to the recommended management of desmoid tumors in different settings, including an in-depth discussion on the outcomes and failures of management following observation, systemic therapy with non-cytotoxic and cytotoxic therapies, surgical resection alone, radiation therapy (RT) alone, and a combination of surgical resection and RT.