Destructive tophaceous calcium hydroxyapatite tumor of the infratemporal fossa

Case report and review of the literature

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✓ Tophaceous pseudogout is one of the rarest forms of crystal deposition disease, typically presenting as a destructive and invasive mass involving the temporomandibular joint or the infratemporal fossa region in the absence of any other articular manifestations. Previous cases have been assumed to be caused by calcium pyrophosphate dihydrate (CPPD) crystal deposition, based on finding weakly birefringent crystals in the involved tissues. The authors present the unique case of a 65-year-old woman with a destructive and invasive facial mass extending to the middle cranial fossa with microscopic and clinical features consistent with tophaceous pseudogout. High-resolution x-ray crystallographic powder diffraction and Fourier transformed infrared spectroscopy subsequently revealed that the crystals were composed of calcium hydroxyapatite without CPPD. The patient was later found to have primary hyperparathyroidism and mild hypercalcemia. This case demonstrates that tissue deposits of calcium hydroxyapatite can cause a destructive and invasive mass containing weakly birefringent crystals and raises the question of whether previous cases attributed to tophaceous pseudogout resulting from CPPD actually were composed of birefringent calcium hydroxyapatite.

Article Information

Address reprint requests to: Marc R. Mayberg, M.D., Department of Neurosurgery, S80, The Cleveland Clinic, 9500 Euclid Avenue, Cleveland, Ohio 44195.

© AANS, except where prohibited by US copyright law.

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Figures

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    Plain radiograph of the skull base and mandible demonstrating a large left TMJ soft-tissue mass (white arrow) with destruction of the adjacent mandibular ramus and condyle.

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    Gadolinium-enhanced axial (left) and coronal (right) T1-weighted MR images revealing a large enhancing mass filling the infratemporal fossa and extending superiorly into the middle cranial fossa.

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    Left: Photomicrograph of a representative section of the lesion showing a diffuse eosinophilic matrix containing numerous histiocytes that resemble chondrocytes. This appearance led to the initial erroneous diagnosis of chondrosarcoma. The correct diagnosis of tophaceous pseudogout was reached in part by identification of crystals (arrows) within the lesion. H & E, original magnification × 235. Right: Higher-power magnification provides further details of the needle-to-rhomboid—shaped crystals (large arrows) and associated multinucleated giant cell reaction (small arrow). H & E, original magnification × 470.

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    Photomicrographs showing high-power view of crystalline material within the tumorlike mass. Original magnification × 400. Left: H & E with transmitted light. Right: Compensated polarizing microscopy with first-order red plate. Birefringence is indicated by crystals in opposite orientations that appeared blue (black arrow) and yellow—orange (white arrow) against the red background. These crystals also appeared white against a black background when viewed with noncompensated crossed polarizers.

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