%0 Journal Article
%T Desmoid-type fibromatosis of paranasal sinuses with intracranial extension in a child-acase-based review.
%A Peroša N
%A Urbančič J
%A Felbabić T
%A Stefanović M
%A Pižem J
%A Bošnjak R
%J Childs Nerv Syst
%V 37
%N 12
%D Dec 2021
%M 34611763
%F 1.532
%R 10.1007/s00381-021-05200-0
%X <B>OBJECTIVE: </B>Desmoid-type fibromatosis (DF) is clonal fibroblastic proliferation that arises in the deep soft tissues, tends to reoccur, and is locally invasive. Desmoid-type fibromatosis of paranasal sinuses with intracranial extension is a rare condition that is even rarer in a small child. We aim to share with the reader our literature review, decision-making, and endoscopic endonasal operation procedure that combined gained us favorable results against this benign tumor with unpredictable natural history and disease course.<BR><B>METHODS: </B>We describe the decision-making process in the management of a 3-year-old boy with a history of sudden vision loss and vomiting. MR showed an expansive well-delineated homogeneous tumor in the sphenoid sinus with intracranial extension and optic nerves compression. The diagnosis of a sporadic form of desmoid-type fibromatosis was made using genetic testing of tumor tissue. A total gross removal was carried out with endoscopic endonasal microsurgical approach. At a 3-month follow-up, the patient is without any signs of recurrance.<BR><B>CONCLUSIONS: </B>The treatment of children with desmoid-type fibromatosis requires a multidisciplinary approach by clinicians experienced with the management of pediatric cancer. While the desmoid-type fibromatosis is a benign, locally invasive tumor, observation should be the first step in the management. In case of life-threatening or symptomatic cases, operations that preserve function and structure should be the first choice for this benign tumor with unpredictable natural history and disease course.