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Year : 2009  |  Volume : 4  |  Issue : 1  |  Page : 48-49

Primitive neuroectodermal tumor (MB) versus atypical teratoid/rhabdoid tumors, an imaging dilemma!

Department of Radiodiagnosis, KMC Hospital, Attavar, Mangalore 575001, Karnataka, India

Correspondence Address:
Santosh Rai
MD DNB FRCR, Shristi, 4-35/4(5), Sankeigudde, Bejai New Road, Bejai, Mangalore-575 004
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.49111

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How to cite this article:
Rai S. Primitive neuroectodermal tumor (MB) versus atypical teratoid/rhabdoid tumors, an imaging dilemma!. J Pediatr Neurosci 2009;4:48-9

How to cite this URL:
Rai S. Primitive neuroectodermal tumor (MB) versus atypical teratoid/rhabdoid tumors, an imaging dilemma!. J Pediatr Neurosci [serial online] 2009 [cited 2022 May 25];4:48-9. Available from: https://www.pediatricneurosciences.com/text.asp?2009/4/1/48/49111


We write to share our experience with the imaging of posterior fossa tumors in children and the common confusion between the imaging features of medulloblastoma and atypical teratoid/rhabdoid tumors (ATRT). We compared the imaging findings of three cases of a posterior fossa tumor in children of ages ranging from 2.5 to 3.5 years to the imaging findings in literature. We had reported all the three cases as medulloblastoma (PNET-MB), but the final pathological findings were of ATRT.

Over the past decade, atypical teratoid/rhabdoid tumors (ATRT) of the central nervous system have emerged as a distinct entity. [1] This tumor is typically misdiagnosed as a primitive neuroectodermal tumor (PNET)/medulloblastoma. The unique immunohistochemical profile of an ATRT helps to distinguish it from a PNET/medulloblastoma. This is of clinical importance because the prognosis of a patient with an ATRT is worse than that of a patient with PNET/medulloblastoma despite aggressive surgical treatment, with or without adjuvant chemotherapy and radiation therapy.

There are no specific imaging features for intracranial AT/RT. However, a high tendency towards large size, a hyperdense solid component on the CT scan with calcification, hemorrhage, necrosis, and subarachnoid spread suggest that this tumor should be considered in the differential diagnoses of large, pediatric, intracranial tumors. [2]

Infratentorial, atypical teratoid/rhabdoid tumors tend to present at a younger age than do medulloblastomas. The roof of the fourth ventricle is a common site of involvement. CPA involvement and intratumoral hemorrhage are more common in atypical teratoid-rhabdoid tumors. [3] Poor prognosis is associated with the presence of MR imaging evidence of a disseminated leptomeningeal tumor.

The striking heterogeneity shown by imaging studies of the atypical teratoid/rhabdoid tumor reflects the histopathological complexity of these tumors, and awareness of atypical teratoid/rhabdoid tumors is important in making the correct diagnosis of this uncommon, but probably underdiagnosed entity. [4]

We conclude that ATRT has to be the first diagnosis in a posterior fossa tumor involving the roof of the 4 th ventricle showing more heterogeneity on MRI (than medulloblastomas) and presenting in a younger age group (< 3 years).

   References Top

1.Danq T, Vassiyadi M, Michaud J, Jimenez C, Ventureyra EC. Atypical teratoid/rhabdoid tumors. Child Nerv Syst 2003;19:244-8.   Back to cited text no. 1    
2.Parmar H, Hawkins C, Bouffet E, Rutka J, Shroff M. Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors. Pediatr Radiol 2006;36:126-32.   Back to cited text no. 2    
3.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 Roentgenol 2008;190:809-14.   Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Arslanoglu A, Aygun N, Tekhtani D, Aronson L, Cohen K, Burger PC, et al . Imaging findings of CNS atypical teratoid/rhabdoid tumors. AJNR Am J Neuroradiol 2004;25:476-80.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]

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