Aggressive Juvenile Trabecular Ossifying Fibroma in a 4-Year-Old: Case Report Bringing Diagnostic Challenges and Surgical Management.

Juvenile trabecular ossifying fibroma (JTOF) is an uncommon benign fibro-osseous neoplasm of the craniofacial skeleton that predominantly affects children and may clinically and radiographically resemble other aggressive lesions. We describe the case of a 4-year-old boy with a rapidly enlarging, painless maxillary mass. Imaging revealed a well-defined expansile lesion with internal radiopaque foci and ground-glass attenuation, extending into the nasal cavity and orbit. Histopathology showed immature trabecular bone with osteoblastic rimming in a fibroblastic stroma, along with aneurysmal bone cyst-like changes. Molecular testing using a next-generation sequencing (NGS) panel was negative for GNAS (Guanine Nucleotide binding protein, Alpha Stimulating activity) mutations, aiding in the exclusion of fibrous dysplasia and supporting the diagnosis of JTOF. Notably, this case represents the use of preoperative embolization in JTOF management, which was successfully performed prior to total hemi maxillectomy. Postoperative CT scans demonstrated no evidence of recurrence through 16 months of follow-up. A multidisciplinary approach was essential to the patient's recovery, with pediatric dental collaboration enabling the fabrication of a custom denture that restored jaw function and improved quality of life. This report underscores the diagnostic challenges associated with JTOF and highlights the value of integrating molecular testing, individualized surgical planning, and coordinated multidisciplinary team care to achieve favorable outcomes in managing this uncommon pediatric lesion, which included involving a pediatric dentist to fabricate a custom denture to support functional recovery and improve the patient's quality of life.