Myofibroblastoma in the suprasellar region

Case report

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✓ Myofibroblastoma is a rare type of benign mesenchymal tumor; only two cases of intracranial myofibroblastoma have been reported in the literature. The authors report on the case of a 34-year-old woman with a myofibroblastoma in the suprasellar region who presented with the complaint of sudden onset of headache followed within 2 weeks by progressively worsening visual disturbance. Computerized tomography scanning demonstrated a mixed low- and high-density mass in the suprasellar region and contrast-enhanced magnetic resonance imaging revealed the mass to be of mixed intensity with heterogeneous enhancement. The tumor was subtotally removed via a right frontobasal translamina—terminalis approach and her vision improved immediately. Histologically, the tumor was characterized by alternating areas of spindle-shaped and round cells that were separated by collagen fibers. The diagnosis of myofibroblastoma was based on the tumor's intense immunoreactivity for α—smooth-muscle actin and the ultrastructural identification of myofibroblasts. The tumor was thought to have originated from the meninges in the suprasellar region.

Article Information

Address reprint requests to: Naoki Shinojima, M.D., Department of Neurosurgery, Kumamoto University School of Medicine, 1–1–1 Honjo, Kumamoto 860-8556, Japan. email: 3008m9020@med.stud.kumamoto-u.ac.jp.

© AANS, except where prohibited by US copyright law.

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Figures

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    A CT scan revealing a mixed low- and high-density well-demarcated round mass in the suprasellar region.

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    Upper Left and Right: Magnetic resonance images revealing a mixed-intensity suprasellar mass lesion circumscribed by a capsule, which demonstrates isointensity on the T1-weighted image (left) and hypointensity on the T2-weighted image (right). Both T1- and T2-weighted images reveal a mixed-intensity pattern in the mass. Lower Left: Axial Gd-DTPA—enhanced T1-weighted MR image demonstrating heterogeneous contrast enhancement. Lower Right: Sagittal Gd-DTPA—enhanced T1-weighted MR image demonstrating the suprasellar cistern and the third ventricle, which are occupied by a mass that compresses the optic nerves upward. There is no evidence of a dural origin. There is no clear attachment to the diaphragma sellae.

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    Photomicrographs of the tumor specimen. A and B: Spindle cells forming fascicular clusters. H & E, original magnification × 200 (A) and × 400 (B). C: Abundant collagen bands separate the cells from each other. Masson trichrome, original magnification × 400. D and E: Diffuse, strongly positive staining of the cells. Stains used included α-SMA (D) and vimentin (E), original magnifications × 400. F: Focally positive staining of the cells. Desmin, original magnification × 400. G: Positive staining of endothelial cells and some of the tumor cells. CD34, original magnification × 400.

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    Electron micrographs demonstrating two types of cells. Upper and Center: Myofibroblastic cells manifest actinlike micro-filaments with dense bodies (arrowheads) and abundant rER. Note the extracellular layer of a basal lamina—like material parallel to the cell surface (arrow). Original magnification × 5600 (upper) and × 16,000 (center). Bars = 2 µm (lower) and 1 µm (center). Lower: Fibroblastic cells with abundant and dilated rER in the cytoplasm. Original magnification × 8000. Bar = 1 µm.

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