小児における顔面骨骨化線維腫：症例シリーズにおける臨床的多様性と外科的結果

Juvenile ossifying fibroma (JOF) is a rare benign fibro-osseous tumor affecting the craniofacial skeleton in children and adolescents. Although nonmalignant, it may behave aggressively and extend into adjacent structures such as the orbit or anterior skull base. Early symptoms are often subtle, delaying diagnosis. Two histologic variants exist - trabecular and psammomatoid - each with distinct clinical behavior and recurrence potential. We describe a case series of three pediatric patients (an 8-year-old male, a 13-year-old female, and a 16-year-old male) diagnosed with craniofacial JOF between March 2022 and January 2025. Presenting symptoms included progressive facial deformity, nasal obstruction, and proptosis. Imaging demonstrated expansile, well-defined lesions involving the ethmoid, maxillary, frontal, and sphenoid sinuses, with orbital displacement in all cases and anterior cranial fossa extension in one. All patients underwent endoscopic tumor resection; two required combined external approaches (Caldwell-Luc and Lynch incision) to achieve complete access. Significant intraoperative bleeding occurred in two cases but was successfully controlled. No permanent visual or neurological deficits were observed. At the six-month follow-up, none showed radiologic recurrence. JOF in pediatric patients is rare and may exhibit locally aggressive behavior, posing diagnostic and surgical challenges. Radiologic and histopathologic evaluation are essential for accurate differentiation from other fibro-osseous lesions. Complete excision via endoscopic or combined approaches provides excellent visualization and low morbidity while preserving function. Early recognition and multidisciplinary management optimize outcomes. Long-term imaging surveillance remains crucial due to the risk of late recurrence.