title: Desmoid tumors and current status of management.
abstract: Desmoid tumors, also known as aggressive fibromatosis, are rare fibroblastic tumors that exhibit a wide range of local aggressiveness, from largely indolent to locally destructive. Understanding of the pathogenesis and the great heterogeneity in the natural history of desmoid tumors is invaluable to the development of therapeutic strategies. The optimal treatment protocol has not yet been established and, in many cases, a multidisciplinary approach including surgery, chemotherapy, and radiation therapy has been employed. The rarity of cases in even major tumor centers has traditionally limited the ability to study this disease. Several novel pharmacologic and biologic treatment approaches are actively being developed, although long-term follow-up is needed for their substantiation.
authors.affiliation: Department of Orthopedic Oncology, University of Pennsylvania, Philadelphia, 19106, USA.
authors.forename: Harish S
authors.initials: HS
authors.lastname: Hosalkar
authors.forename: Edward J
authors.initials: EJ
authors.lastname: Fox
authors.forename: Thomas
authors.initials: T
authors.lastname: Delaney
authors.forename: Jesse T
authors.initials: JT
authors.lastname: Torbert
authors.forename: Christian M
authors.initials: CM
authors.lastname: Ogilvie
authors.forename: Richard D
authors.initials: RD
authors.lastname: Lackman
mesh_terms: D000293:Adolescent; D000328:Adult; D001707:Biopsy, Needle; D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy / Q000379:methods; D005260:Female; D018222:Desmoid Tumors / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D018570:Risk Assessment; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome