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Two Patients with Intraspinal Germinoma Associated with Klinefelter Syndrome: Case Report and Review of the Literature

Y. Nakataa, A. Yagishitaa and N. Araib

a Department of Neuroradiology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan
b Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Tokyo, Japan


Figure 1
Figure 1
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  		Fig 1. Patient 1. MR images of the thoracic spine and brain.

A, T2-weighted axial image of the thoracic spine in 1999 revealed an area of increased signal intensity changes in the spinal cord at the level of the T7 vertebral body (arrow). Enlargement of the spinal cord was not observed. Good sagittal MR images were not obtained, because of scoliosis.

B–G, Sagittal and coronal MR images of the thoracic spine in 2003 showed enlargement of the spinal cord between the level of T6 and T7 vertebral bodies, with hyperintensity on T2WI (B and E, arrows), isointensity on T1WI (C and F, arrows) and heterogeneous enhancement on T1WI obtained after an intravenous injection of contrast medium (D and G, arrows), which suggests an intramedullary tumor. Spinal cord atrophy was demonstrated at the upper and lower ends of the intramedullary tumor at the levels of T5 and T8 vertebral bodies on sagittal and coronal images (B–G, arrowheads). The tumor showed a "string of beads" appearance (B and E, asterisks).

H and I, Spinal cord atrophy was also confirmed on T2-weighted axial images at the levels of T5 and T8 vertebral bodies (arrowheads).

J, Photomicrograph of a specimen of the intraspinal tumor showed large round cells with clear cytoplasm and central nuclei (arrow) along fibrovascular septa. Small lymphocytes were not apparent (hematoxylin-eosin stain; original magnification, x66).

K, Photomicrograph of a specimen of the intraspinal tumor showed that the cell membrane (arrow), which stains brown, was immunoreactive for PLAP. Cell membrane expression of PLAP was characteristic of germinoma (placental alkaline phosphatase stain; original magnification, x132).

L and M, The left putamen was atrophic and showed high signal intensity change on T2WI (L, arrowhead). No contrast enhancement was observed on T1WI obtained after an intravenous injection of contrast medium (M, arrowhead). The presence of germinoma was suggested in the left putamen.


Figure 2
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  		Fig 2. Patient 2. MR images of the thoracic spine and brain. Because these images were obtained by using an old MR system with low magnetic field (0.5T), images were relatively poor in quality.

A, T1-weighted sagittal image of the brain in 1989 showed cerebellar atrophy (arrow).

B–D, No abnormal signal intensity changes suggesting the presence of tumors were detected on MR images, though contrast medium was not given.

E, T1-weighted sagittal image of the thoracic spine in 1989 demonstrated a slight enlargement of the spinal cord at the T8 level (arrow).

F and G, MR images of the thoracic spine in 1991 demonstrated further enlargement of the lesion in spinal cord between the level of T7 and T9 vertebral bodies, with hyperintensity on T2WI (not shown), isointensity on T1WI (F, arrow), and heterogeneous enhancement on T1WI obtained after an intravenous injection of contrast medium (G, arrow).

H, Photomicrograph of a specimen of the intraspinal tumor showed large round cells with clear cytoplasm and central nuclei (arrow). Small lymphocytes were not apparent (hematoxylin-eosin stain; original magnification, x66).

I, Photomicrograph of a specimen of the intraspinal tumor showed that the cell membrane (arrow), which was stained brown slightly, was immunoreactive for PLAP. Cell membrane expression of PLAP was characteristic of germinoma (placental alkaline phosphatase stain; original magnification, x132).

J, MR images of the brain in 1992 revealed slight contrast enhancement in the cerebellum on T1WI obtained after an intravenous injection of the contrast medium (arrowheads).

K and L, MR images of the brain in 1992 revealed lesions with increased signal intensity changes on T2WI in the corpus medullaris cerebelli (K, arrows), periventricular white matter around the anterior horn, and corticospinal tracts on both sides (L, arrowheads).

M and N, MR images of the brain in 1994 demonstrated multiple tumors in the cerebellum and the left lateral ventricle on T1WI after an intravenous injection of contrast medium (M and N, arrowheads).

O, MR images of the brain in 1994 demonstrated further enlargement of the lesions in the corpus medullaris cerebelli with increased signal intensity changes on T2WI (arrowheads). The lesions in the corpus medullaris cerebelli, periventricular white matter around anterior horn, and corticospinal tracts on both sides with increased signal intensity changes on T2WI remained stable.

P, Photomicrograph of a specimen of the tumor in the vermis showed 2 distinct cell types: large round cells with clear cytoplasm and central nuclei (arrow) were admixed with small lymphocytes (arrowhead) along fibrovascular septa, indicating a germinoma (hematoxylin-eosin stain; original magnification, x66).





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