Juvenile psammomatoid ossifying fibroma (JPOF)

This case series reports on three patients with a JPOF between 8 and 15 years. Two of three patients described a nasal obstruction for a longer time. One patient had an onset of bulbar motility disorder with a feeling of pressure. In the clinical examination all patients showed a complete obstruction of the right nasal cavity by a well-bounded but hard mass of tumor. The radiological examination showed a contrast enhanced lesion in ethmoid bone in two patients. One patient had a tumor with ossification in the roof of maxillary sinus. The tumors in the ethmoid bone were resected by an endonasal surgery (2), the tumor at the roof of the maxillary sinus roof by an incision in the vestibule of the mouth (1). The histopathological examination diagnosed a JPOF with secondary aneurysmal bone cyst, which triggered the symptoms. In two cases a revision surgery was necessary in a time period between 7 to 10 months. Since then, all patients are clinically and radiologically free of recurrence. JPOF is a rare, benign tumor that commonly occurs in children and adolescents, and less commonly in adults. They are two variants of juvenile ossifying fibroma, juvenile trabecular and psammomatoid. It has a predilection for the paranasal sinuses, maxilla and mandible. Most patients are initially asymptomatic. But a rapid growth of tumor mass can cause nasal obstruction or facial asymmetry. The diagnosis is based on histopathological examination. Complete removal of the tumor is recommended because of its aggressive and locally invasive expansion. Furthermore, the tumor has a high recurrence rate. Therefore, long-term clinical and radiological observation are necessary.

Publikationsverlauf

Artikel online veröffentlicht:
24. Mai 2022