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Images in Neurology |

Giant Cell Tumor of the Sphenoid

Maria Margarita Company, MD; Rafael Ramos, MD
Arch Neurol. 2009;66(1):134-135. doi:10.1001/archneurol.2008.509.
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A 19-year-old man was seen for progressive visual loss and exophthalmia of the left eye occurring over a period of 5 months. Ophthalmologic examination showed decreased visual acuity of the left eye and mild visual field abnormalities. The remaining neurological examination was normal. Cranial magnetic resonance imaging showed an enhancing tumor at the junction of the orbital apex, ethmoidal sinus, and sphenoid sinus that extended intracranially toward the left frontal and temporal lobes and toward the ethmoid sinus, infiltrating the sphenoid fissure, otic canal, and cavernous sinus (Figure 1). A craniotomy was carried out through a left frontotemporal approach, with resection of the intracranial component. Microscopic examination revealed a tumor composed of multinucleated giant cells, stromal cells, aggregates of foamy histiocytes, and hemorrhagic foci (Figure 2). Mitoses were scarce. A diagnosis of giant cell tumor (GCT) of the sphenoid bone was established. The patient presented recurrent disease at 2 months following the intervention. Imaging studies showed tumor growth toward the temporal lobe, invasion of the cavernous sinus, and persistence of the sphenoid and ethmoid mass. The patient underwent a second procedure with confirmation of the histopathological diagnosis. At the time of writing this article, he had sequelae of ophthalmoplegia of the left eye associated with palpebral ptosis and amaurosis.

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Figure 1.

Coronal (A) and axial (B) T2-weighted magnetic resonance imaging of the skull base. An expansive intradiploic lesion is seen (arrow), slightly hyperintense on T2 imaging, with bulging and displacement of the cortical bone of the major wing of the left sphenoid. The mass encompasses the optic nerve, whereas the interface between the posterior fossa and the orbit is preserved.

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Figure 2.

A and B, Histological study shows a tumor composed of multinucleated giant cells intermixed with ovoid stromal cells, hemorrhagic foci, and xanthomatous histiocytes (hematoxylin-eosin, original magnification ×100).

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