Faculty Collaboration Database

Recurrent giant cranial desmoid tumor in a 3-year-old boy with familial adenomatous polyposis requiring bifrontoparietal cranioplasty: case report. J Neurosurg Pediatr 2016 Dec;25(6):703-707

Date

09/17/2016

Pubmed ID

27635978

DOI

10.3171/2016.6.PEDS15741

Scopus ID

2-s2.0-85012858127 (requires institutional sign-in at Scopus site)   2 Citations

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.

Author List

Li L, Jensen JN, Szabo S, VanTuinen P, Lew SM

Author

Sean Lew MD Chief, Professor in the Neurosurgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adenomatous Polyposis Coli
Child
Child, Preschool
Fibromatosis, Aggressive
Frontal Bone
Humans
Male
Neoplasm Recurrence, Local
Parietal Bone
Skull Neoplasms