Primary Intracranial Atypical Teratoid/Rhabdoid Tumors of Infancy and Childhood: MRI Features and Patient Outcomes
S.P. Meyersa,
Z.P. Khademianc,
J.A. Biegeld,
S.H. Chuange,
D.N. Koronesb and
R.A. Zimmermanc
a Departments of Radiology, University of Rochester School of Medicine, Strong Memorial Hospital, Rochester, NY
b Department of Pediatrics, University of Rochester School of Medicine, Strong Memorial Hospital, Rochester, NY
c Departments of Radiology, Children’s Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, Pa
d Department of Pathology and Pediatrics, Children’s Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, Pa
e Department of Radiology, Hospital for Sick Children, Toronto, Ont, Canada
View larger version (59K):
[in a new window]
|
Fig 1. MR images of a 2-year-old girl with a germ-line mutation of the hSNF5/INI1 gene and AT/RT in the vermis extending into the fourth ventricle.
A, Sagittal (TR/TE, 520/10) MR image shows the tumor to have intermediate and low signal intensity. The tumor invades the dorsal brain stem.
B, Axial (TR/TE, 5700/90) MR image shows the tumor to have heterogeneous intermediate-to-slightly-high signal intensity that is slightly hyperintense relative to gray matter, as well as zones of high signal intensity representing cystic/necrotic regions. Abnormal high signal intensity is also seen in the dorsal pons and left middle cerebellar peduncle, consistent with tumor invasion.
C, Axial (TR/TE, 680/22) MR image shows the tumor to have solid-enhancing and nonenhancing cystic/necrotic components. Irregular enhancing margins of the lesion are seen at the dorsal pons and left middle cerebellar peduncle, consistent with tumor invasion. This patient died 1.6 years after diagnosis from locally recurrent and disseminated tumor.
D, Axial CT image shows the solid portion of the AT/RT to have attenuation slightly higher than that of gray matter. Low-attenuation cystic/necrotic zones and several tiny calcifications are also present.
| |
View larger version (66K):
[in a new window]
|
Fig 2. MR images of a 10-year-old boy with AT/RT in the vermis, extending into the fourth ventricle.
A, Axial (TR/TE, 600/11) MR image shows the tumor to have mixed intermediate signal intensity as well as irregular zones of high signal intensity secondary to hemorrhage (methemoglobin).
B, Axial (TR/TE, 3500/108) MR image shows the tumor to have heterogeneous mixed low, intermediate, and high signal intensity.
C, Axial (TR/TE, 500/29) MR image shows contrast enhancement in less than two thirds of the AT/RT. This patient is alive without evidence of disease 9 years after surgery/initial diagnosis.
| |
View larger version (65K):
[in a new window]
|
Fig 3. MR images of a 1.3-year-old boy with AT/RT in the right cerebral peduncle.
A, Axial (TR/TE, 600/10) MR image shows the tumor to have intermediate signal intensity.
B, Axial (TR/TE, 4000/105) MR image shows the tumor to have heterogeneous intermediate-to-slightly-high signal intensity, which includes zones that are isointense or slightly hyperintense to gray matter.
C, Axial (TR/TE, 700/22) fat-suppressed MR image shows prominent homogeneous contrast enhancement of the primary lesion as well as abnormal subarachnoid enhancement in the interpeduncular cistern and along the sulci adjacent to frontal lobes (arrows), representing disseminated leptomeningeal tumor. This patient died 4 months after diagnosis.
| |
View larger version (115K):
[in a new window]
|
Fig 4. MR images of 3.4-year-old boy with a hemorrhagic AT/RT in the frontal lobe.
A, Sagittal (TR/TE, 520/10) MR image shows a hemorrhagic lesion in the inferior left frontal lobe.
B, Sagittal (TR/TE, 600/22) MR image shows edge enhancement of the lesion.
C and D, Sagittal (C) (TR/TE, 520/10) and axial (D) (TR/TE, 520/10) MR images obtained 7 months after A, -B show marked interval enlargement of the lesion, which has heterogeneous mixed low, intermediate, and high signal intensity involving the inferior portions of both frontal lobes.
E, Axial (TR/TE, 5700/90) MR image shows the tumor to have mixed low, intermediate, and high signal intensity, with evidence of edema in the surrounding brain tissue.
F, Axial (TR/TE, 600/22) MR image shows prominent heterogeneous contrast enhancement of the tumor with irregular lobulated margins. This patient underwent partial resection of the AT/RT, subsequently had disseminated leptomeningeal tumor, and died 3.3 years after surgery/initial diagnosis.
G, Axial CT image shows the lesion to have intermediate attenuation with multiple calcifications and zones of low attenuation.
| |
View larger version (78K):
[in a new window]
|
Fig 5. MR images of a 4-year-old girl with a constitutional ring 22 chromosome and AT/RT involving the septum pellucidum, with extension into the lateral ventricles.
A, Axial (TR/TE, 450/18) MR image shows the tumor to have intermediate signal intensity with several small zones of low signal intensity.
B, Axial (TR/TE, 4000/105) MR image shows the tumor to have heterogeneous predominantly intermediate-to-slightly-high signal intensity that is isointense to gray matter, as well as small zones of high and low signal intensity.
C, Axial (TR/TE, 450/31) MR image shows irregular heterogeneous contrast enhancement of less than two thirds of the primary AT/RT. This patient had subtotal resection of the tumor and subsequently had locally recurrent disease and disseminated leptomeningeal tumor. She died 14 months after surgery/initial diagnosis.
| |
View larger version (61K):
[in a new window]
|
Fig 6. MR images of a 4-month-old female infant with a germ-line mutation of the hSNF5/INI1 gene with synchronous AT/RT, showing contiguous involvement of the pineal gland, dorsal midbrain, and upper cerebellum and a malignant rhabdoid tumor involving the left kidney.
A, Sagittal (TR/TE, 570/14) MR image shows the tumor to have predominantly intermediate signal intensity with several small zones of high signal intensity.
B, Sagittal (TR/TE, 750/22) MR image shows only minimal contrast enhancement in less than one third of the tumor.
C, Axial (TR/TE, 3710/105) MR image shows the tumor to have intermediate-to-slightly-high signal intensity that is isointense to gray matter, as well as a small zone of high signal intensity.
| |
View larger version (113K):
[in a new window]
|
Fig 7. MR images of a 10-month-old female infant with an extra-axial AT/RT in the right cerebellopontine angle cistern, extending into the right internal auditory canal (arrows).
A, Axial (TR/TE, 550/14) MR image shows the tumor to have intermediate signal intensity with a zone of low signal intensity medially.
B, Axial (TR/TE, 600/22) MR image shows prominent enhancement of the solid portion of the lesion. This patient had subtotal resection followed by chemotherapy and died 1 month after surgery from sepsis related to immunosuppression from chemotherapy.
| |
View larger version (77K):
[in a new window]
|
Fig 8. MR images of an 8-month-old female infant with AT/RT involving the pineal gland
A, Axial (TR/TE, 4000/105) MR image shows the tumor to have heterogeneous intermediate-to-slightly-high signal intensity with regions that are isointense and slightly hyperintense to gray matter, as well as a small zone of high signal intensity.
B, Axial FLAIR (TR/TE/TI, 9000/119/2200) MR image shows the tumor to have mostly intermediate signal intensity that is isointense to gray matter.
C, Axial (TR/TE, 800/20) fat-suppressed MR image with magnetization transfer shows no contrast enhancement of the primary lesion. This patient had subtotal resection of the primary lesion followed by chemotherapy. The patient had subsequent local recurrence and disseminated leptomeningeal tumor and died 7 months after initial diagnosis.
| |
View larger version (77K):
[in a new window]
|
Fig 9. MR images of 4-year-old boy with AT/RT involving the vermis
A, Axial FLAIR (TR/TE/TI, 9000/119/2200) MR image shows the tumor to have mostly intermediate signal intensity that is isointense to gray matter.
B, Axial (TR/TE, 6000/99) MR image shows the tumor to have mostly intermediate-to-slightly-high signal intensity that is slightly hyperintense to gray matter, as well as small zones of high signal intensity.
C, Axial (TR/TE, 800/20) fat-suppressed MR image with magnetization transfer shows prominent slightly heterogeneous contrast enhancement of the tumor. This patient had gross total resection of the primary lesion followed by chemotherapy and has been disease-free for 4 years.
D, Axial DWI (b = 1000) shows the lesion to have high signal intensity.
E, Axial ADC map shows low signal intensity in the lesion, indicating restricted diffusion of water within the AT/RT.
| |
