<%server.execute "isdev.asp"%> Supratentorial atypical teratoid rhabdoid tumor: An uncommon childhood tumor Mondal D, Jana M, Julka PK - J Pediatr Neurosci
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Year : 2011  |  Volume : 6  |  Issue : 1  |  Page : 90-91

Supratentorial atypical teratoid rhabdoid tumor: An uncommon childhood tumor

1 Department of Radiation Oncology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
2 Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India

Date of Web Publication2-Sep-2011

Correspondence Address:
Manisha Jana
Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.84423

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How to cite this article:
Mondal D, Jana M, Julka PK. Supratentorial atypical teratoid rhabdoid tumor: An uncommon childhood tumor. J Pediatr Neurosci 2011;6:90-1

How to cite this URL:
Mondal D, Jana M, Julka PK. Supratentorial atypical teratoid rhabdoid tumor: An uncommon childhood tumor. J Pediatr Neurosci [serial online] 2011 [cited 2023 Dec 2];6:90-1. Available from: https://www.pediatricneurosciences.com/text.asp?2011/6/1/90/84423

Dear sir,

Atypical teratoid rhabdoid tumor (ATRT) is a rare neoplasm of childhood. Most common location of this tumor is infratentorial. Supratentorial ATRT is an uncommon occurrence. We present the imaging findings of a supratentorial ATRT in a child.

A 5-year-old girl presented to the pediatric casualty with sudden onset of headache and vomiting since last 15 days. She also developed right hemiparesis and deterioration of vision of right eye since last 5 days. Magnetic resonance imaging (MRI) of the brain revealed a large mass in left thalamic region with foci of hemorrhages and hydrocephalus due to mass effect on the third ventricle [Figure 1],[Figure 2],[Figure 3]. She underwent near total excision of the mass by transcortical transventricular approach under image guidance. On histopathology, the mass was diagnosed to be atypical teratoid rhabdoid tumor WHO grade IV. Six months follow-up imaging revealed no tumor recurrence.
Figure 1: Axial T2W MRI image of the brain reveals a large, well-defined mass lesion in left thalamic region, having heterogeneous hyperintense signal intensity

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Figure 2: Coronal T2W MR image of the brain reveals the mass causing mass effect and contralateral displacement of the third ventricle causing hydrocephalus

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Figure 3: Axial T2W gradient echo MR image shows areas of hemorrhage within the mass lesion seen as hypointense foci

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ATRT of the CNS has been recognized as a distinct disease entity as recently as in 1987. [1] Usual presentation is in the children of age group below 2 years. Most commonly (two-third cases), the tumor is located in posterior cranial fossa, [2] the common locations being cerebellopontine angle cistern and cerebellar hemispheres. Supratentorial location is relatively less common, though an incidence of 47% has been reported in some case series. [3] Supratentorial locations include the suprasellar, pineal and temporal locations. The age group of purely supratentorial tumors is higher compared to that of infratentorial tumors. [4] Clinical presentation is usually nonspecific and depends on the tumor location and age of the child. Congenital tumors may present with macrocrania. The tumor usually has a rapid progression; having an average survival of 6-11 months. [5] The lesions are hyperdense on imaging and heterogeneous on non-contrast magnetic resonance imaging (MRI) having areas of hemorrhage, necrosis and calcification, and show heterogeneous contrast enhancement. On T2W images, the tumor is isointense to hypointense to gray matter, owing to high nuclear-cytoplasmic ratio. Leptomeningeal spread of tumor is observed in one-third of the cases. There is no significant difference in imaging or prognosis in supratentorial and infratentorial tumors.

The differential diagnoses includes medulloblastoma, primitive neuroectodermal tumor (PNET), choroid plexus carcinoma, gemistocytic astrocytoma, lymphoma. [5] In infratentorial tumors, the main differential is medulloblastoma, whereas supratentorial tumors need to be differentiated from PNETs. There is significant overlap in imaging as well as histopathologic features of medulloblastoma and ATRT. [6] Both the tumors are hypercellular, can show CSF dissemination and drop metastases. ATRT is more heterogeneous and may show areas of hemorrhage. Medulloblastoma is almost always infratentorial, whereas ATRT can occur in supratentorial location. Differentiating ATRT from medulloblastoma is important as the prognosis and response to treatment is worse in ATRT. [7]

   References Top

1.Lefkowitz IB, Rorke JB, Packer R, Sutton LN, Siegel KR, Katnick RJ. Atypical teratoid tumor of infancy: Definition of an entity. Ann Neurol 1987;22:448-9.  Back to cited text no. 1
2.Oka H, Scheithauer BW. Clinicopathological characteristics of atypical teratoid/rhabdoid tumor. Neurol Med Chir 1999;39:510-7.  Back to cited text no. 2
3.Meyers SP, Khademian ZP, Biegel JA, Chuang SH, Korones DN, Zimmerman RA. Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am J Neuroradiol 2006;27:962-71.  Back to cited text no. 3
4.Warmuth-Metz M, Bison B, Dannemann-Stern E, Kortmann R, Rutkowski S, Pietsch T. CT and MR imaging in atypical teratoid/rhabdoid tumors of the central neurous system. Neuroradiology 2008;50:447-52.  Back to cited text no. 4
5.Parwani AV, Stelow EB, Pambuccian SE, Burger PC, Ali SZ. Atypical teratoid /rhabdoid tumor of the brain: Cytopathologic characteristics and differential diagnosis. Cancer Cytopathol 2005;105:65-70.   Back to cited text no. 5
6.Ho DM, Hsu C, Wong T, Ting LT, Chiang H. Atypical teratoid/rhabdoid tumor of the central nervous system: A comparison with primitive neuroectodermal tumor/medulloblastoma. Acta Neuropathol 2000;99:482-8.  Back to cited text no. 6
7.Koral K, Gargan L, Bowers DC, Gimi B, Timmons CF, Weprin B, et al. Imaging characteristics of atypical teratoid-rhabdoid tumor in children compared with medulloblastoma. AJR Am J Radiol 2008;190:809-14.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]

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