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Rare case of desmoid-type fibromatosis of the mandibular region in a child: diagnosis and surgical management

Type:  Articles

Pubblication date:  /1/2013

Authors:  D. Burlini*, G. Conti**, E. Bardellini***, F. Amadori***

Language:  English

Institution:  *AO Spedali Civili Brescia, Dep. Pediatric Maxillo Facial Surgery **University of Milano- IRCCS “ Ca Granda” Ospedale Maggiore ***Dental Clinic, University of Brescia, Italy

Publication:  European Journal of Paediatic Dentistry

Publisher:  Ariesdue Srl

Keywords:  Children; Desmoid-type fibromatosis; Maxillofacial surgery.

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Title:  Rare case of desmoid-type fibromatosis of the mandibular region in a child: diagnosis and surgical management

Abstract:  Background Desmoid-type fibromatosis is a broad group of rare disorders that originate from musculoaponeurotic structures. They represent less than 0,1% off all tumors and the annual incidence is 2-4 cases per million, with two peaks between 6 and 15 years of age and between puberty and age 40. They are rare in the oral and maxillofacial regions. Its pathogenesis is multifactorial. The treatment is still mainly represented, both in children and adults, by surgical excision. Case report A case is reported of a 8-year-old girl with desmoid fibroma in the mandible who presented at the Department of Pediatric Dentistry with a swelling measuring 4x4 cm on the lower edge of the right mandible which had appeared a few months earlier and slowly developed. The family dentist had initially diagnosed it as an odontogenic abscess from the lower right deciduous molars, but the antibiotic therapy was unsuccessful. After x-ray examination, which showed a large osteolytic lesion, mandibular CT revealed a solid expanding mass. The child was referred to the Department of Paediatric Maxillofacial Surgery where the whole mass was surgically removed. At the 2-year follow-up no relapse was noted. Conclusion This case stresses the importance, especially for paediatric dentists, of further diagnostic steps if suspect lesions do not heal after conventional treatment.

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