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Best Cases from the AFIP

Congenital Intracranial Teratoma¹

¹ From the Departments of Radiology (B.A.S., C.E.D.), Pediatrics (M.A.A.), Pathology (M.A.A.), and Obstetrics and Gynecology (A.Z.A.), Eastern Virginia Medical School, 825 Fairfax Ave, Suite 541, Norfolk, VA 23507. Received July 14, 2003; revision requested August 28 and received October 14; accepted October 16. Address correspondence to B.A.S. (e-mail: bsandow@infionline.net).

View larger version (147K): [in a new window]   Figure 1.  Transverse US image of the fetal brain at 28 weeks gestation shows an ill-defined, echogenic, solid-appearing mass (arrows) at the level of the thalamus.

View larger version (148K): [in a new window]   Figure 2a.  T2-weighted MR images of the fetal brain at 30 weeks gestation. (a) Axial image shows that the mass (arrows) is iso- to hypointense and located in the region of the hypothalamus. The right temporal horn is partly effaced (arrowhead). (b) Sagittal image shows that the mass (arrows) is suprasellar or sellar. It effaces the third ventricle and extends anteriorly to the frontal cortex and posteriorly to the brainstem.

View larger version (141K): [in a new window]   Figure 2b.  T2-weighted MR images of the fetal brain at 30 weeks gestation. (a) Axial image shows that the mass (arrows) is iso- to hypointense and located in the region of the hypothalamus. The right temporal horn is partly effaced (arrowhead). (b) Sagittal image shows that the mass (arrows) is suprasellar or sellar. It effaces the third ventricle and extends anteriorly to the frontal cortex and posteriorly to the brainstem.

View larger version (114K): [in a new window]   Figure 3.  Postnatal coronal US image of the brain, obtained 1 day after birth, shows that the mass (arrows) is irregular and suprasellar. It is predominantly echogenic with cystic components and has shadowing echogenic foci, which are consistent with calcifications. Normal anatomic landmarks are replaced by the large mass, which occupies most of the intracranial compartment.

View larger version (155K): [in a new window]   Figure 4a.  Postnatal MR images of the brain obtained 1 day after birth. (a) Sagittal T1-weighted image shows that the mass (arrows) is heterogeneous and suprasellar. It is predominantly iso- to hyperintense and has multiple low-signal-intensity cystic regions as well as numerous punctate high-signal-intensity foci, which probably represent calcifications. The mass fills much of the supratentorial compartment, causing posterior displacement of the brainstem (arrowhead). The third ventricle is attenuated, resulting in obstructive hydrocephalus. (b) Sagittal T2-weighted image shows that the mass (arrows) is heterogeneous and predominantly iso- to hypointense with multiple hyperintense cystic areas of varying sizes. (c) Axial contrast material-enhanced T1-weighted image shows heterogeneous enhancement of the mass (arrows), which is in the midline and effaces the third ventricle, resulting in marked hydrocephalus.

View larger version (141K): [in a new window]   Figure 4b.  Postnatal MR images of the brain obtained 1 day after birth. (a) Sagittal T1-weighted image shows that the mass (arrows) is heterogeneous and suprasellar. It is predominantly iso- to hyperintense and has multiple low-signal-intensity cystic regions as well as numerous punctate high-signal-intensity foci, which probably represent calcifications. The mass fills much of the supratentorial compartment, causing posterior displacement of the brainstem (arrowhead). The third ventricle is attenuated, resulting in obstructive hydrocephalus. (b) Sagittal T2-weighted image shows that the mass (arrows) is heterogeneous and predominantly iso- to hypointense with multiple hyperintense cystic areas of varying sizes. (c) Axial contrast material-enhanced T1-weighted image shows heterogeneous enhancement of the mass (arrows), which is in the midline and effaces the third ventricle, resulting in marked hydrocephalus.

View larger version (147K): [in a new window]   Figure 4c.  Postnatal MR images of the brain obtained 1 day after birth. (a) Sagittal T1-weighted image shows that the mass (arrows) is heterogeneous and suprasellar. It is predominantly iso- to hyperintense and has multiple low-signal-intensity cystic regions as well as numerous punctate high-signal-intensity foci, which probably represent calcifications. The mass fills much of the supratentorial compartment, causing posterior displacement of the brainstem (arrowhead). The third ventricle is attenuated, resulting in obstructive hydrocephalus. (b) Sagittal T2-weighted image shows that the mass (arrows) is heterogeneous and predominantly iso- to hypointense with multiple hyperintense cystic areas of varying sizes. (c) Axial contrast material-enhanced T1-weighted image shows heterogeneous enhancement of the mass (arrows), which is in the midline and effaces the third ventricle, resulting in marked hydrocephalus.

View larger version (138K): [in a new window]   Figure 5a.  Histopathologic photographs of the postmortem specimen. (a) Gross photograph shows the right cerebral hemisphere (anterior is to the left), the mass (arrows), and marked hydrocephalus. The mass is lobulated, tan to cream colored, and located in the midline. The cut surface of the mass demonstrates solid and cystic areas as well as evidence of hemorrhage. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows a variety of incompletely differentiated tissue components, including endodermally derived respiratory epithelium (arrow) and enteric epithelium (arrowhead), which form cystic and tubular structures. Also seen are mesodermal elements, including moderately hypercellular cartilage (*), bundles of smooth muscle, and a surrounding hypercellular stroma. (c) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows ectodermally derived neuroepithelial rosettes (arrows), which mimic the developing neural tube. (d) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) shows primitive neuroblastlike cells forming a neuroepithelial rosette (arrows), which is embedded in a hypercellular stroma suggestive of embryonic mesenchyme.

View larger version (182K): [in a new window]   Figure 5b.  Histopathologic photographs of the postmortem specimen. (a) Gross photograph shows the right cerebral hemisphere (anterior is to the left), the mass (arrows), and marked hydrocephalus. The mass is lobulated, tan to cream colored, and located in the midline. The cut surface of the mass demonstrates solid and cystic areas as well as evidence of hemorrhage. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows a variety of incompletely differentiated tissue components, including endodermally derived respiratory epithelium (arrow) and enteric epithelium (arrowhead), which form cystic and tubular structures. Also seen are mesodermal elements, including moderately hypercellular cartilage (*), bundles of smooth muscle, and a surrounding hypercellular stroma. (c) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows ectodermally derived neuroepithelial rosettes (arrows), which mimic the developing neural tube. (d) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) shows primitive neuroblastlike cells forming a neuroepithelial rosette (arrows), which is embedded in a hypercellular stroma suggestive of embryonic mesenchyme.

View larger version (190K): [in a new window]   Figure 5c.  Histopathologic photographs of the postmortem specimen. (a) Gross photograph shows the right cerebral hemisphere (anterior is to the left), the mass (arrows), and marked hydrocephalus. The mass is lobulated, tan to cream colored, and located in the midline. The cut surface of the mass demonstrates solid and cystic areas as well as evidence of hemorrhage. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows a variety of incompletely differentiated tissue components, including endodermally derived respiratory epithelium (arrow) and enteric epithelium (arrowhead), which form cystic and tubular structures. Also seen are mesodermal elements, including moderately hypercellular cartilage (*), bundles of smooth muscle, and a surrounding hypercellular stroma. (c) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows ectodermally derived neuroepithelial rosettes (arrows), which mimic the developing neural tube. (d) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) shows primitive neuroblastlike cells forming a neuroepithelial rosette (arrows), which is embedded in a hypercellular stroma suggestive of embryonic mesenchyme.

View larger version (171K): [in a new window]   Figure 5d.  Histopathologic photographs of the postmortem specimen. (a) Gross photograph shows the right cerebral hemisphere (anterior is to the left), the mass (arrows), and marked hydrocephalus. The mass is lobulated, tan to cream colored, and located in the midline. The cut surface of the mass demonstrates solid and cystic areas as well as evidence of hemorrhage. (b) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows a variety of incompletely differentiated tissue components, including endodermally derived respiratory epithelium (arrow) and enteric epithelium (arrowhead), which form cystic and tubular structures. Also seen are mesodermal elements, including moderately hypercellular cartilage (*), bundles of smooth muscle, and a surrounding hypercellular stroma. (c) Photomicrograph (original magnification, x100; hematoxylin-eosin stain) shows ectodermally derived neuroepithelial rosettes (arrows), which mimic the developing neural tube. (d) High-power photomicrograph (original magnification, x400; hematoxylin-eosin stain) shows primitive neuroblastlike cells forming a neuroepithelial rosette (arrows), which is embedded in a hypercellular stroma suggestive of embryonic mesenchyme.