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DOI: 10.1055/s-0040-1702716
Aggressive Neurocranial Juvenile Psammomatoid Ossifying Fibroma
Publication History
Publication Date:
05 February 2020 (online)
Background: Juvenile ossifying fibromas are a group of benign craniofacial neoplasms, divided into histologically juvenile psammomatoid ossifying fibromas (JPOF) or juvenile trabecular ossifying fibroma (JTOF). JPOF is predominantly sinonasal, with rare intracranial extension; aggressive subtypes are associated with aneurysmal bone cyst (ABC) formation. Recurrence is high in JPOF, occurring in 30–65% of patients — particularly following subtotal resection. We report a novel case of extensive, hyper-acute JPOF progressing over 3 months, in association with new meningoencephalocele development.
Methods: Case Report
Results: A 26-year-old woman initially presented elsewhere with headache, fever, and jaw pain radiating to the ear. MRI demonstrated a large, destructive, heterogeneous mass involving the anterior fossa, with erosion through the cribriform plate and extension into the ethmoid sinus, tuberculum sella and bilateral orbits. The patient underwent endonasal biopsy which diagnosed JPOF with ABC changes. Correspondingly, the patient underwent a right pterional craniotomy and subtotal resection, elsewhere. Residual tumor was left on the tuberculum sella and left skull base.
The patient presented to our institution for second opinion regarding ongoing management. Repeat imaging, 3 months after the initial operation elsewhere, demonstrated remarkable interval tumor growth, as well as the development of a large meningoencephalocele extending from the left skull base into the resection cavity. We performed a combined bifrontal craniotomy with endonasal sphenoidotomy and aggressive JPOF and ABC resection to the tuberculum sella, with decompression of the left optic nerve, amputation of the meningoencephalocele, and skull base reconstruction with autologous fascia lata and vascularized pericranium. The operation proceeded without complication, and as of the patient's last follow-up at four month post-repeat resection, the patient's neurologic deficits had resolved, and MRI demonstrated no evidence of tumor or meningoencephalocele recurrence.
Conclusion: JPOF are rare neoplasms with marked risk of recurrence—particularly following subtotal resection, with prior reports documenting recurrence as early as 6 months. The current report demonstrates the potential for dramatic regrowth in these lesions, suggesting that a more aggressive approach may be indicated at primary resection. If clinical circumstances or patient-specific considerations mandate a staged approach, we recommend updating intracranial imaging at a shorter postresection interval, ideally 4–6 weeks following the initial operation.
