Bilateral Psammomatoid Ossifying Fibroma of Maxilla

Abstract

Ossifying-Fibroma (OF) is a benign neoplasm histopathologically composed of fibrocellularstroma and variable amount of mineralized material. Generally, these tumours in jaw bones are found as solitary lesions. Occurrence of these tumours as Multiple Ossifying Fibromas (MOF) in jaw bones is very rare. The studies reported till now have found an association between MOF and Hyperparathyroid Jaw Tumour Syndrome (HPT-JT). HPT-JT is always associated with the genetic mutation in tumour suppressor gene HRPT2 which encodes protein parafibromin. The simultaneous presentation of ossifying fibroma and other features of HPTJT may or may not be present. Thus, the expression of parafibromin needs to be evaluated in MOF to rule out it’s association with HPT-JT. The present case of 24 years old female patient, showed bilateral maxillary Psammomatoid Juvenile Ossifying Fibroma (PsJOF). The patient was further investigated for association with HPT-JT. Other finding such as blood serum calcium and parathyroid assay were normal. A negative expression of parafibromin in the tumour tissue suggested an association with HPT-JT. Thus, evaluation of parafibromin can help in accurate diagnosis and treatment of MOF of jaws.

Keywords

• hyperparathyroid jaw tumour syndrome
• jaw
• multiple ossifying fibroma (mof)
• parafibromin