Desmoid Tumor

Excerpt

Desmoid tumors are mesenchymal neoplasms that are considered locally invasive but nonmetastasizing. They are also known as aggressive fibromatosis, deep fibromatosis, and musculoaponeurotic fibromatosis. Even though they do not metastasize, they are locally invasive and cause significant morbidity and mortality. There is no standard approach to treating desmoid tumors; managing them is challenging and requires discussion at a multidisciplinary tumor board.

Despite surgical resection, desmoid tumors have a high rate of local recurrence; accordingly, they have been labeled as intermediate locally aggressive tumors by the World Health Organization (WHO). According to WHO, desmoid tumor is a "clonal fibroblastic proliferation that arises in the deep soft tissues and is characterized by infiltrative growth and a tendency toward local recurrence but an inability to metastasize," even though it may be multifocal in the same limb or body part. Systemic treatment is used in patients where surgery and radiation therapy are either not feasible or will not achieve a cure. Recently, several new drugs have been licensed by the United States Food and Drug Administration (FDA) to treat patients with DT.