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Juvenile Psammomatoid Ossifying Fibroma (JPOF) of Proximal Radius: A Rare Entity
Abstract
Background:
Juvenile psammomatoid ossifying fibroma (JPOF) is a rare fibro-osseous lesion that usually occurs in the facial bones. The pathognomonic histopathologic feature is the presence of spherical ossicles, which are similar to psammoma bodies. It is considered to be a unique lesion because of its reported tendency to occur in children and adolescents and its tendency for locally aggressive growth. Because this lesion is aggressive in nature with high recurrence rate, early detection and complete surgical excision are essential.
Case Report:
Herein, we present a case of 11 year old girl, who presented to our clinic with history of gradual onset pain around right elbow associated with limitation of right forearm rotation for 3 months. After getting informed consent, we performed needle biopsy and histopathologically it was diagnosed as Psammomatoid type juvenile ossifying fibroma (JPOF) of proximal radius. We performed extensive debridement, curettage of entire cystic lesion involving the proximal radius and filled the cavity with allograft bone granules and intramedullary titanium elastic nail through fibular strut graft, bridging the cystic lesion.
Conclusion:
As far as our knowledge there is no reported case of psammomatoid type of juvenile ossifying fibroma of proximal radius. We believe that such tumors in long bones are aggressive and have tendency for recurrence. Definitive diagnosis is utmost important for proper planning, surgical excision and reconstruction of long bones and need regular follow up to look for any recurrence or malignant transformation.