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Recurrent giant cranial desmoid tumor in a 3-year-old boy with familial adenomatous polyposis requiring bifrontoparietal cranioplasty: case report
Journal article   Peer reviewed

Recurrent giant cranial desmoid tumor in a 3-year-old boy with familial adenomatous polyposis requiring bifrontoparietal cranioplasty: case report

Luyuan Li, John N Jensen, Sara Szabo, Peter VanTuinen and Sean M Lew
Journal of neurosurgery. Pediatrics, Vol.25(6), pp.703-707
12/2016
DOI: 10.3171/2016.6.PEDS15741
PMID: 27635978
url
https://doi.org/10.3171/2016.6.PEDS15741View
Published (Version of record) Open Access

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.
Adenomatous Polyposis Coli - complications Adenomatous Polyposis Coli - diagnostic imaging Adenomatous Polyposis Coli - surgery Child Child, Preschool Fibromatosis, Aggressive - complications Fibromatosis, Aggressive - diagnostic imaging Fibromatosis, Aggressive - surgery Frontal Bone - diagnostic imaging Frontal Bone - surgery Humans Male Neoplasm Recurrence, Local - complications Neoplasm Recurrence, Local - diagnostic imaging Neoplasm Recurrence, Local - surgery Parietal Bone - diagnostic imaging Parietal Bone - surgery Skull Neoplasms - complications Skull Neoplasms - diagnostic imaging Skull Neoplasms - surgery

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