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

Authors:

ABSTRACT

Aim

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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