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Imaging findings of Atypical Teratoid /
Rhabdoid tumor in a 11-Year Old Boy
H Nakamura1, T Suzuki1, M Uchida2, M Doi3, M Takagi3, Y
Tanaka2, Y Nakajima2 ,
St. Marianna University of Medicine, Radiology1 and
Neurodsurgery 2 and Pahtology3 Kawasaki Japan.
Disclosures
The authors do not have a financial
relationship with a commercial
organization that may have a direct
or indirect interest in the content.
Purpose
Atypical teratoid / rhabdoid tumors (AT/RT) are
rare aggressive neoplasms occurring in
children younger than 2 years of age at the
infratentorial. We demonstrate an atypical
case of supratentorial AT/RT in a 11-year-old
boy.
History
. A 11-year-old boy came to our
hospital complaining of mild
headache and nausea, which had
been continued for a month.
CT shows a large
heterogeneous mass in
the left frontal lobe, which
contains two parts of iso
density on the ventral side
and low density on the
dorsal side.
Due to mass effect,
ventricle , especially on
the left side, is obliterated.
Black arrow indicates
small high density
suggesting calcification.
CT
DWI
FLAIR
Axi / Cor T2 WI
T1WI
Gd T1WI
Cor Gｄ T1WI
Cor T2*
MRS
MRI findings
Solid part (yellow arrow) - heterogeneous intensity on
DWI, iso and high heterogeneous intensity on T2 WI
and FLAIR , low and iso heterogeneous intensity on
T1 WI and mild to moderate contrast enhancement
on contrast images. MRS shows decreased NAA and
prominent choline peak, suggesting cell membrane
turn over.
Necrotic or cystic part - low intensity on DWI and T1 WI,
high signal on T2 WI, and no contrast enhancement
Calcification and hemorrhage ( white arrow on T2*)
Imaging findings
Large mass with solid and cystic
component.
Calcification and hemorrhage
High choline peak.
Differential diagnosis
Pilocytic astrocytoma
Pleomorphic xanthoastrocytoma
AT/RT
Primitive Neuroectodermal Tumor (PNET)
Glioma (glioblastoma, oligodendroglioma)
(anaplastic ) ependymoma
Desmoplastic infantile
ganglioglioma/astrocytoma
Surgical findings
Tumor shows well-defined margin
Solid and cystic tumor
Success of total removal
H&E stain
H&E stain on high power shows large pleomorphic cells with
abundant eosinophilic cell and cells with large eccentrically
located nucleus, which are characteristic of rhabdoid cells.
INI-1 stain
immunohistochemistry stain for INI-1 on high power shows
lack of staining (brown) in tumor cells with retention of
staining in non-tumor cells.
Final Diagnosis
Atypical Teratoid / Rhabdoid
tumor (AT / RT)
AT/RT
Highly malignant embryonal neoplasm
(grade 4 according to the World Health
Organization classification) that typically
occurs in children aged <3 years and
6.7% of CNS neoplasm children aged <
3years1
Most often reported as infratentorial2, 3
Age and localization - our case
Older than 10years -12%2, 9.1%3
Adult onset almost 30 cases were reported and
they favor the supratentorial4
Some reports show supratentorial is more
prevalent or comparative 2, 5
Pathology
Characteristic “rhabdoid” phenotype ( large immature
rhabdoid cells) are helpful for the diagnosis of AT/RT
if present, however are not always seen.
Pathologically , AT/RT has considerable overlap with
medulloblastoma/PNET
Cytogenetic evaluation of CNS AT/RT and renal AT/RT
has revealed deletions and mutations of the INI1
locus in chromosome 22
INI1 thought to represent a tumor suppressor gene
The immunohistochemical reagent for INI1 fails to stain
the nuclei of tumor cells with INI1 mutations
Differential diagnosis
Only imaging findings can not distinguish
from other high-grade tumors
Thick heterogeneous enhancing wall
surrounding a central cyst or cysts is the
key feature in differentiation from
others6
Discussion
The imaging feature is typical findings of
AT/RT
Supratentorial location and the age older
10 years are not typical, but not very
rare
Conclusion
When we see the supratentorial solid
and cystic tumor even aged >10
years , differential diagnosis
should include AT/RT
References
1. Buscariollo DL, Park HS, Roberts KB, et al. Survival outcomes in atypical
2.
3.
4.
5.
6.
teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance,
Epidemiology, and End Results analysis. Cancer 2012;118:4212-4219
Meyers SP, Khademian ZP, Biegel JA, et al. Primary intracranial atypical
teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient
outcomes. AJNR American journal of neuroradiology 2006;27:962-971
Warmuth-Metz M, Bison B, Dannemann-Stern E, et al. CT and MR imaging in
atypical teratoid/rhabdoid tumors of the central nervous system. Neuroradiology
2008;50:447-452
Han L, Qiu Y, Xie C, et al. Atypical teratoid/rhabdoid tumors in adult patients: CT
and MR imaging features. AJNR American journal of neuroradiology
2011;32:103-108
Jin B, Feng XY. MRI features of atypical teratoid/rhabdoid tumors in children.
Pediatric radiology 2013;43:1001-1008
Au Yong KJ, Jaremko JL, Jans L, et al. How specific is the MRI appearance of
supratentorial atypical teratoid rhabdoid tumors? Pediatric radiology
2013;43:347-354