Introduction: Desmoid tumors are benign soft tissue tumors, but their infiltration character
is responsible for big morbidity, especially in the cervicofacial location. Their management,
therefore, represents a real challenge.Clinical case:
Reporting a case of a 4-year-old girl presenting a desmoid tumor at the expense
of the mandibular corpus and the right ascending ramus, with loco-regional infiltration,
and whose treatment consisted of surgical resection of the tumor with Hemi-mandibulectomy
and reconstruction according to the Masquelet technique. Conclusion: The traditional
treatment consists of a large local excision with negative surgical margins. Head
and neck fibromatosis, while rare, present a complex problem as they are often aggressive,
and invade local bone, nerve and muscles, and can leave patients with a cosmetic
deformity.In our case, it was a mandibular reconstruction after a large resection of
a desmoid tumor with a location on the head and neck. With good consolidation, absence
of recurrence, and without aesthetic sequelae at the control.
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