title: Recurrent giant cranial desmoid tumor in a 3-year-old boy with familial adenomatous polyposis requiring bifrontoparietal cranioplasty: case report.
abstract: Desmoid tumors, also known as aggressive fibromatosis, are locally infiltrating musculoaponeurotic neoplasms arising in connective tissues. Desmoid tumors may be associated with familial adenomatous polyposis (FAP), a genetic disorder that presents with hundreds to thousands of precancerous colorectal polyps. The authors report the case of an 18-month-old boy who underwent resection of a right temporal desmoid tumor (initially diagnosed as cranial fasciitis) and developed a bilateral frontoparietal calvarial desmoid tumor 2 years later. The patient underwent gross-total resection of the tumor that required a large cranioplasty. He was subsequently diagnosed with FAP. The patient has been without tumor recurrence for 9 years afterwards and has not required revision of his cranioplasty. This is the first report describing a recurrent cranial desmoid tumor in a pediatric patient with FAP. The authors believe, however, that some of the cases previously reported as cranial fasciitis are likely desmoid tumors pathobiologically and genetically.
authors.affiliation: Departments of 1 Neurological Surgery and.
authors.forename: Luyuan
authors.initials: L
authors.lastname: Li
authors.affiliation: Plastic Surgery, Medical College of Wisconsin/Children's Hospital of Wisconsin, Milwaukee, Wisconsin; and.
authors.forename: John N
authors.initials: JN
authors.lastname: Jensen
authors.affiliation: Department of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Sara
authors.initials: S
authors.lastname: Szabo
authors.affiliation: Department of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Peter
authors.initials: P
authors.lastname: VanTuinen
authors.affiliation: Departments of 1 Neurological Surgery and.
authors.forename: Sean M
authors.initials: SM
authors.lastname: Lew
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D018222:Desmoid Tumors / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D005624:Frontal Bone / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D010294:Parietal Bone / Q000000981:diagnostic imaging / Q000601:surgery*; D012888:Skull Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*
keywords: APC = adenomatous polyposis coli; FAP = familial adenomatous polyposis; PMMA = polymethylmethacrylate; adenomatous polyposis coli; aggressive fibromatosis; cranioplasty; desmoid tumor; familial adenomatous polyposis; oncology