A Rare Case Of Desmoid Tumor In The Lower Mandibular Sector Of An Adolescent: Surgical Intervention And Outcomes

Abstract

Introduction: Desmoid tumors are rare, benign neoplasms known for their locally aggressive behavior and high recurrence rates. They arise from fibroblasts and are characterized by their infiltrative growth, making surgical management challenging. This report presents a case of a desmoid tumor in the lower mandibular sector of a 16-year-old male, focusing on surgical intervention and molecular insights.

Case Report: A 16-year-old male presented with a growing mass in the lower right mandible, causing pain and mobility of tooth 47. Imaging revealed an infiltrative lesion eroding the mandibular bone. Histopathological analysis confirmed a desmoid tumor with spindle-shaped fibroblasts and nuclear β-caténine expression. Molecular testing identified a CTNNB1 gene mutation, confirming the diagnosis.

Discussion: Desmoid tumors, despite being benign, are challenging due to their aggressive local behavior. Surgical excision with clear margins is the primary treatment, as these tumors are resistant to chemotherapy and radiotherapy. This case highlights the importance of precise surgical planning and the role of β-caténine and CTNNB1 mutations in diagnosis and prognosis.

Conclusion: Effective management of desmoid tumors in adolescents requires thorough surgical intervention and comprehensive diagnostic analysis. Long-term follow-up is crucial to monitor for recurrence. The molecular characteristics of the tumor, such as CTNNB1 mutations, play a significant role in guiding treatment and predicting outcomes.

Keywords:

Desmoid tumor, mandibular neoplasm, fibromatosis, β-caténine, CTNNB1 mutation

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