Juvenile Aggressive Ossifying Fibroma of Paranasal Sinuses: An Unusual Presentation in an 18-Year-Old Female

Ullah Asad,Abdelsayed Rafik,Kavuri Sravan,Sliker Taylor,Byrd Kenneth,Mattox Samantha N,Palacios Guillermo A

doi:10.36959/605/548

Abstract

Abstract Juvenile aggressive ossifying fibroma (JAOF) is a rare variant of ossifying fibroma. Conventional ossifying fibromas are slow growing neoplasms thought to originate from osseous mesenchyme, commonly arising in the mandible. Conversely, JAOF occurs more frequently in the paranasal bones with rapid growth leading to local destruction, facial asymmetry, nasal obstruction, and proptosis. JAOF has two microscopic variants, psammomatoid and trabecular. Most JAOFs occur in patients less than 12-years-old with slight male predominance. We present an 18-year-old female with facial asymmetry for two years duration and recent onset of severe headaches. Examination revealed a bulging left maxillary bone and left upward proptosis. MRI showed an 81.2 × 44 × 39 mm, lobulated, well-defined, expansile osseous lesion with calcified matrix and soft tissue component involving the left maxillary sinus with extension into the left nasal cavity, frontal and ethmoid sinuses, and left orbit. An endoscopic biopsy procedure was performed to reveal sinonasal mucosa infiltrated by a cellular, fibro-osseous neoplasm consisting primarily of numerous, closely packed, round, uniform basophilic psammoma bodies with eosinophilic rings, interspersed by cellular, benign fibroblastic proliferations. Cystic changes and foci of necrosis were also noted. Complete excision of the JAOF is critical as recurrence and corresponding complications may develop in up to 50% of patients.

Highlights

Conventional ossifying fibromas (OF) are benign, slow growing neoplasms, typically found in the gnathic bones, and thought to originate from the osseous mesenchyme or the periodontal ligament [1,2]
Juvenile aggressive ossifying fibromas (JAOF) typically occur in the paranasal sinuses and craniofacial bones with rapid growth leading to local destruction, facial deformity, and associated complications
Ossifying fibromas are subdivided into cemento-ossifying fibroma (COF) and juvenile ossifying fibroma (JOF) dependent upon the presence of specific histologic features

Summary

Introduction

Conventional ossifying fibromas (OF) are benign, slow growing neoplasms, typically found in the gnathic bones, and thought to originate from the osseous mesenchyme or the periodontal ligament [1,2] These tumors typically present in females in their third and fourth decade of life, with a propensity for the teeth-bearing areas of the mandible or maxilla [3]. We present a case of juvenile psammomatoid ossifying fibroma of the left craniofacial bones with extensive growth and expansion into the frontal and ethmoid sinuses, nasal cavity, orbit, and skull base. The entire left maxillary sinus was blocked by tumor, causing right sided septal deviation Upon debulking this area, multiple blood vessels, believed to be branches of the sphenopalatine artery, were noted inside the tumor without appreciable invasion. Post-operative follow-up in the form of regular interval imaging and consideration of an anterior cranio-facial resection, if tumor burden is significantly high, were recommended

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