Juvenile psammomatoid ossifying fibroma: clinical analysis of8consecutive cases
2022, 20 (3):
PURPOSE: To analyze the clinical and imaging features, treatment options, and prognosis of juvenile psammomatoid ossifying fibroma (JPOF). METHODS: Clinical and imaging data of 8 JPOF patients (male, n=4; female, n=4) who were admitted during February 2016 and October 2020 were collected and follow-ups were made to summarize the clinical manifestations, imaging features, treatment options and prognosis. RESULTS: Eight JPOF cases were included into this study with an average age of 14.1±7.9 years. Among these JPOF cases, the lesions were found to originate in the jaw(n=3), ethmoid sinus (n=2), maxilla/maxillary sinus (n=1), nasal cavity (n=1), and frontal sinuses (n=1), averaging (37.0±9.7) mm in diameter. The clinical manifestations were predominantly nasal congestion and discharge, as well as local swelling and pain. Of the 8 JPOF patients, four received CT, three received MRI, and the remaining one was examined through CT and MRI. CT scans exhibited space-occupying lesions with a circular or oval inner core circumscribed by a sclerotic shell of bone, while MRI findings demonstrated mixed-signal of cystic-solid tumors. JPOF patients all received surgical treatments. To be specific, transnasal endoscopic procedures were adopted for radical curettage of JPOF in the ethmoid sinus, maxilla/maxillary sinus, and nasal cavity. JPOFs arising in the frontal sinuses were removed via open surgery; of the three patients with JPOFs in their jaws, two underwent curettage of the lesion involving the jaw and partial removal of the surrounding bone containing the lesional tissues, in which cases a relapse was identified 6 months later. Segmental resection of the jaw was performed on the third patient in combination with a vascularized iliac crest bone graft for jaw reconstruction, and no recurrent JPOF was detected up to the last follow-up date. CONCLUSIONS: JPOF is a rare benign bone tumor that most frequently occurs among teenagers. As the clinical and imaging features are inconspicuous, pathology remains to be the mainstay in diagnosing JPOF. Considering its invasive growth, it is recommended that radical curettage be carried out to reduce the risk of recurrence.
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