Two-stage surgical treatment for a giant sellar psammomatoid juvenile ossifying fibroma: case report

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Psammomatoid juvenile ossifying fibroma (PJOF) is an uncommon, benign fibro-osseous tumor. It is a purely surgical disease, and a review of the literature revealed that adjuvant therapies, including chemotherapy and radiation, play a limited role. The authors report the case of a 16-year-old male refugee who presented with a giant sinonasal PJOF with parasellar invasion, after having been unable to undergo earlier surgical treatment. The delay in presentation resulted in a large lesion with bilateral optic nerve compression, blindness, and frontal lobe compression. The patient was surgically treated with a two-stage combined cranial and endoscopic endonasal surgical approach. The delay in treatment and significant neurological compromise, which necessitated a two-stage approach, are unique from previously reported cases of PJOF.

ABBREVIATIONS PJOF = psammomatoid juvenile ossifying fibroma.

Article Information

Correspondence Douglas L. Brockmeyer: Primary Children’s Hospital, University of Utah, Salt Lake City, UT. neuropub@hsc.utah.edu.

INCLUDE WHEN CITING Published online January 4, 2019; DOI: 10.3171/2018.10.PEDS18537.

Disclosures Dr. Couldwell has served as a consultant for International Neurosurgeon’s Circle.

© AANS, except where prohibited by US copyright law.

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    Preoperative axial (A), coronal (B), and sagittal (C) noncontrasted CT scans demonstrate a large sellar PJOF with extension into the nasopharyngeal sinuses and compression of the bilateral optic nerves and frontal lobe. Heterogeneous hyperdensity within the lesion suggested significant calcification, which was instead found to be gelatinous intraoperatively. Axial (D) noncontrasted T1-weighted MRI scan demonstrates a homogeneous lesion. Axial (E) and sagittal (F) T2-weighted MRI scans show a heterogeneous lesion with internal cystic structures. Diffusion-weighted image (G) with hypointensity suggests hypocellularity of the lesion. Axial T1-weighted contrast-enhanced image (H) shows a heterogeneous lesion with intralesional cyst-like enhancement. Time-of-flight image (I) of the intracranial vessels obtained for surgical planning demonstrates displacement of the anterior cerebral and middle cerebral arteries posteriorly.

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    Axial (A) and sagittal (B) T1-weighted images with contrast enhancement obtained after the stage 1 surgical procedure, showing decompression of the frontal lobes with resection of the lesion up to the anterior skull base. Axial (C) and sagittal (D) CT images obtained after the stage 2 surgical procedure, showing complete lesion resection within the sellar and nasopharyngeal cavities.

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    Intraoperative photograph of the PJOF, superior view (A) from a bifrontal craniotomy, demonstrates areas of midline tumor (asterisk) medial to the unroofed globe (arrowhead). Endoscopic endonasal tumor resection (B, arrow) and bifrontal approach for tumor resection (double arrows) are shown. Micro-Doppler ultrasound was used to insonate the carotid artery (C, double arrows), and endoscopic visualization (arrow) aided in identifying deep tumor boundaries (asterisk). Final postresection cranial view (D) from a bifrontal approach, with retractors placed on the frontal lobes. This view shows the globes (arrowheads) and a transbasal trajectory to the upper clivus. Figure is available in color online only.

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    Low-power view (A) of the PJOF demonstrates significant calcified psammoma bodies within a uniform stromal bed. High-power view (B) demonstrates the lesion (above the arrows) adjacent to, but not invading, sinus mucous glands (below the arrows). High-power view (C) further demonstrates the lesional psammoma bodies (asterisks) without stroma showing hyperproliferation, as well as no mitotic bodies or irregular nuclei. Bar = 200 µm (A), 50 µm (B), 20 µm (C). Figure is available in color online only.

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