Journal of Pediatric Neurosciences
: 2011  |  Volume : 6  |  Issue : 1  |  Page : 90--91

Supratentorial atypical teratoid rhabdoid tumor: An uncommon childhood tumor

Dodul Mondal1, Manisha Jana2, Pramod K Julka1,  
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

Correspondence Address:
Manisha Jana
Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029

How to cite this article:
Mondal D, Jana M, Julka PK. Supratentorial atypical teratoid rhabdoid tumor: An uncommon childhood tumor.J Pediatr Neurosci 2011;6:90-91

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 2021 Aug 4 ];6:90-91
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Full Text

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}{Figure 2}{Figure 3}

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]


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