Article Figures & Data
Figures
- 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, ×66).
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, ×132).
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.
- 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, ×66).
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, ×132).
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, ×66).
Tables
- TABLE 1:
Summary of previous cases of tumors in the central nervous system associated with Klinefelter syndrome
Patient No./Age (y)/Sex Series Tumor Location Surgery Histologic Diagnosis 1/16/M Rubenstein, 19729 Pineal region Not described Germinoma 2/20/M Ahagon et al, 198310 Suprasellar region Biopsy Germinoma 3/12/M Ellis et al, 198611 Posterior hypothalamus, left frontal lobe Radical excision Germinoma 4/15/M Arens et al, 198812 Pineal region Biopsy Germinoma 5/40/M Liang et al, 199013 Right frontoparietal region Partial excision Non-Hodgkin’s lymphoma 6/19/M Hashimoto, et al, 199214 Medulla oblongata Biopsy Germinoma 7/12/M Prall et al, 199515 Pineal region Subtotal resection Malignant mixed germ cell tumor 8/13/M Wysocka et al, 199616 Cerebellum Total resection Pilocytic astrocytoma 9/19/M Kaido et al, 20032 Hypothalamus–temporal region Partial removal Germinoma 10/29/M Ganslandt et al, 20006 Spinal cord (T12–L4) Partial resection Germinoma 11/35/M Our patient 1 Spinal cord (T6–T7), hypothalamic region Total resection Germinoma 12/27/M Our patient 2 Spinal cord (T7–T9), cerebellum, intraventricular region Partial resection Germinoma Patient No./ Age (y)/Sex Series Tumor Location Surgery Histologic Diagnosis Values of hCG in Serum/CSF 1/5/M Hisa et al, 198519 Th11–L3 Biopsy, amputation Germinoma with syncitiotrophoblastic giant cells High 2/31/F Matsuoka et al, 199120 Th12–L2 Biopsy Germinoma Normal 3/31/M Nagasawa et al, 199117 Midcervical Not performed Probably germinoma Not described 4/34/F Hanafusa et al, 199321 Th10–Th11 Gross total resection Germinoma Normal 5/16/F Slagel et al, 199522 Th11–L4 Partial resection Germinoma Not described 6/34/F Matsuyama et al, 199523 Th6–Th8 Partial resection Germinoma Normal 7/24/M Itoh et al, 19965 Th11–Th12 Gross total resection Germinoma Negative 8/24/M Miyauchi et al, 199624 Th12–L3 Partial resection Germinoma Negative 9/29/M Ganslandt et al, 20006 Th12–L4 partial resection Germinoma High 10/33/M Hata et al, 200218 Th7–Th9 Partial resection Germinoma Normal 11/7/M Zhu et al, 200225 Th12–L1 Not described Germinoma with syncitiotrophoblastic giant cells High 12/32/F Sasaki et al, 200226 Th3–Th4 Not performed s/o germinoma with syncitiotrophoblastic giant cells High 13/18/M Chute et al, 200327 Th6–Th8 Biopsy Germinoma High 14/18/M Huang et al, 200428 C3–C6 Partial resection Germinoma Not described 15/33/F Watanabe et al, 200529 Th1–Th3 Partial resection Germinoma Not described 16/35/M Our patient 1 Th6–Th7 Total resection Germinoma High 17/27/M Our patient 2 Th7–Th9 Partial resection Germioma High Note:—hCG indicates human chorionic gonadotropin; CSF, cerebrospinal fluid.
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