Extracerebral neural tissue mass in the middle cranial fossa extending into the oropharynx in a neonate

Case report

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✓ The authors report the case of a newborn baby girl who had a large extracerebral neural tissue mass in the right middle cranial fossa which extended into the oropharynx through an enlarged foramen ovale. The surgical specimen comprised various kinds of neural tissue, including primitive neuroepithelium, mature as well as immature neurons and glia, myelinated fibers, ependyma, choroid plexus, ocular pigmented epithelium, and a number of calcospherites. The mass was partially covered by its own leptomeninges. The question of whether this mass is a true neoplasm or a heterotopia is discussed.

Article Information

Address reprint requests to: Susumu Wakai, M.D., Department of Neurosurgery, Dokkyo University School of Medicine, Mibu-Machi, Tochigi, 321-02, Japan.

© AANS, except where prohibited by US copyright law.

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Figures

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    Coronal tomogram showing a large bone defect (arrows) in the right middle cranial fossa.

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    Contrast-enhanced computerized tomography scans taken when the patient was 6 months of age showing a cystic mass in the right frontotemporal region. A and B: The peripheral portion of the mass is slightly enhanced. C: A large bone defect (arrows) is apparent. D: The mass protrudes into the oropharynx (arrowhead).

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    Operative photographs. Upper: A mass with a black surface (arrowheads) is seen in the right middle fossa. The mass is partially covered by its own dura on the medial side (long arrow). The asterisk denotes the frontal lobe and the short arrow the tentorial edge. Center: After removal of the tumor, the oculomotor nerve and upper brain stem (arrow) were visualized. The enlarged foramen ovale (arrowheads) is packed with muscle and Oxycel gauze. Double asterisks indicate the middle cranial fossa, and the single asterisk marks the frontal lobe. Lower: The specimen removed at surgery.

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    Schematic drawing of the roentgenological and operative findings. Thick black line indicates the dura mater. The mass had its own dura on the medial side. The arachnoid membrane is indicated by the broken line. There is a cystic cavity in the center of the mass.

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    Postoperative computerized tomography scan. The relationship between the foramen ovale (arrows) and the remaining oropharyngeal mass (arrowhead) is clearly seen. Asterisk indicates the cavity that remained after removal of the mass.

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    Photomicrographs of the operative specimen. A: Primitive neuroepithelia with canal formation (arrows) are seen near leptomeninges in the upper right corner. Asterisks indicate large vessels. Bodian, × 74. B: Ocular pigmented epithelium is seen in the surface of the tumor (asterisk). There are many calcospherites beneath this structure. A number of immature neurons are observed, arranged in a laminar fashion (arrows). Bodian, × 74. C: A bundle of myelinated nerve fibers is seen on the left and a cluster of neurons (arrows) on the right of the picture. Asterisk indicates the cystic cavity. Klüver-Barrera, × 74. D: Higher magnification of a portion of the specimen seen in C, showing mature and immature neurons intermingled. Bodian, × 305.

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