Abstract
INTRODUCTION: Infantile desmoid fibromatosis (IDF) is a rare, benign soft tissue tumor, with locally infiltrative behavior. It presents two forms: solitary and disseminated. This condition can affect any location, although IDF of the mandibular is exceptionally rare. Its locally aggressive nature and proximity to critical anatomical structures present significant management challenges. Its management modalities are still controverted. CASE PRESENTATION: We report a rare case mandibular IDF, highlighting its clinical features and management. A two-year-old child with no known medical history, presented with a 5-month history of a right lateral cervical mass. Physical examination revealed a fixed 3 cm mass in the sub-mandibular region. Cervical computed tomography showed a 4 cm heterogeneous lesion at the right mandibular angle, with bone lysis and contact with the ipsilateral submandibular gland and the hyoid bone. A biopsy was performed and histological examination confirmed the diagnosis of an IDF. The patient underwent a wait and see approach, and after two years of follow-up, both clinical and ultrasound evaluation showed a significant regression of the mass, with no associated symptoms. No artificial intelligence (AI) tools were used in the research, analysis, or writing of this manuscript. CLINICAL DISCUSSION: The diagnosis of IDF is challenging and relies on histological examination. The treatment is multidisciplinary and still controverted. It involves a wait and see approach, chemotherapy and surgery. Given the possibility of spontaneous regression of the lesion, a conservative approach is preferred in pediatric patients. CONCLUSION: This study highlights a rare occurrence of a mandibular IDF in a child to emphasize the importance of making the appropriate diagnosis and management modality.