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Extra-axial, dural-based atypical teratoid/rhabdoid tumor

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Abstract

Atypical teratoid/rhabdoid tumors (ATRTs) are malignant central nervous system tumors that affect early childhood (< 3 years), and mostly located in the infratentorial space. Owing to an infrequent occurrence, their radiological features have not been completely defined. Nevertheless, these are characteristically intra-axial except for few instances in the cerebellopontine angle region. We describe a case of a 10-year-old boy who harbored an extra-axial, dural-based ATRT in the right parietal region. The lesion was totally excised followed by adjuvant chemo-radiotherapy. At 10-month follow-up, he was well with no recurrence. The report intends to highlight an atypical imaging presentation of ATRT in an older child, and adds to the radiological spectrum. This uncommon pathology should be borne in mind, even in a supratentorial dural-based location.

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References

  1. Lee IH, Yoo SY, Kim JH, Eo H, Kim OH, Kim IO, Cheon JE, Jung AY, Yoon BJ (2009) Atypical teratoid/rhabdoid tumors of the central nervous system: imaging and clinical findings in 16 children. Clin Radiol 64(3):256–264

    Article  CAS  Google Scholar 

  2. Meyers SP, Khademian ZP, Biegel JA, Chuang SH, Korones DN, Zimmerman RA (2006) Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am J Neuroradiol 27(5):962–971

    CAS  PubMed  PubMed Central  Google Scholar 

  3. Yamasaki K, Kiyotani C, Terashima K, Watanabe Y, Kanamori M, Koga Y, Hata N, Iwasaki F, Goto H, Koh K, Kurihara J, Tokunaga S, Arakawa Y, Hasegawa D, Kosaka Y, Hara J (2019) Clinical characteristics, treatment, and survival outcome in pediatric patients with atypical teratoid/rhabdoid tumors: a retrospective study by the Japan Children’s Cancer Group. J Neurosurg Pediatr Nov 15:1–10. https://doi.org/10.3171/2019.9.PEDS19367 Epub ahead of print

    Article  Google Scholar 

  4. Chen ML, McComb JG, Krieger MD (2005) Atypical teratoid/rhabdoid tumors of the central nervous system: management and outcomes. Neurosurg Focus 18(6A):E8

    PubMed  Google Scholar 

  5. Gendle C, Karthigeyan M, Salunke P, Gupta K (2020) Pineal atypical teratoid rhabdoid tumor in a 5-month-old child. Pediatr Neurosurg Dec 10:1–5. https://doi.org/10.1159/000511995 Epub ahead of print

    Article  Google Scholar 

  6. Arslanoglu A, Aygun N, Tekhtani D, Aronson L, Cohen K, Burger PC, Yousem DM (2004) Imaging findings of CNS atypical teratoid/rhabdoid tumors. AJNR Am J Neuroradiol 25(3):476–480

    PubMed  PubMed Central  Google Scholar 

  7. Burger PC, Yu IT, Tihan T, Friedman HS, Strother DR, Kepner JL, Duffner PK, Kun LE, Perlman EJ (1998) Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol 22(9):1083–1092

    Article  CAS  Google Scholar 

  8. Ho DM, Hsu CY, Wong TT, Ting LT, Chiang H (2000) Atypical teratoid/rhabdoid tumor of the central nervous system: a comparative study with primitive neuroectodermal tumor/medulloblastoma. Acta Neuropathol 99(5):482–488

    Article  CAS  Google Scholar 

  9. Biegel JA, Rorke LB, Packer RJ, Emanuel BS (1990) Monosomy 22 in rhabdoid or atypical tumors of the brain. J Neurosurg 73(5):710–714

    Article  CAS  Google Scholar 

  10. Biegel JA, Tan L, Zhang F, Wainwright L, Russo P, Rorke LB (2002) Alterations of the hSNF5/INI1 gene in central nervous system atypical teratoid/rhabdoid tumors and renal and extrarenal rhabdoid tumors. Clin Cancer Res 8(11):3461–3467

    CAS  PubMed  Google Scholar 

  11. Bing F, Nugues F, Grand S, Bessou P, Salon C (2009) Primary intracranial extra-axial and supratentorial atypical rhabdoid tumor. Pediatr Neurol 41(6):453–456

    Article  Google Scholar 

  12. Gupta K, Karthigeyan M, Satapathy A, Salunke P (2018) Synchronous solitary calvarial yolk sac tumor metastasis as the initial presentation of mediastinal germ cell tumor. Childs Nerv Syst 34(2):363–366

    Article  Google Scholar 

  13. Perry A, Dehner LP (2003) Meningeal tumors of childhood and infancy. An update and literature review. Brain Pathol 13(3):386–408

    Article  Google Scholar 

  14. van den Munckhof P, Christiaans I, Kenter SB, Baas F, Hulsebos TJ (2012) Germline SMARCB1 mutation predisposes to multiple meningiomas and schwannomas with preferential location of cranial meningiomas at the falx cerebri. Neurogenetics 13(1):1–7

    Article  Google Scholar 

  15. Couce ME, Aker FV, Scheithauer BW (2000) Chordoid meningioma: a clinicopathologic study of 42 cases. Am J Surg Pathol 24(7):899–905

    Article  CAS  Google Scholar 

  16. Haberler C, Laggner U, Slavc I, Czech T, Ambros IM, Ambros PF, Budka H, Hainfellner JA (2006) Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype. Am J Surg Pathol 30(11):1462–1468

    Article  Google Scholar 

  17. Judkins AR, Mauger J, Ht A, Rorke LB, Biegel JA (2004) Immunohistochemical analysis of hSNF5/INI1 in pediatric CNS neoplasms. Am J Surg Pathol 28(5):644–650

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, Postgraduate Institute of Medical Education & Research (PGIMER), Sector 12, Chandigarh, 160012, India

    Madhivanan Karthigeyan, Parthasarathi Mondal, Pravin Salunke & Anshul Siroliya

  2. Department of Histopathology, PGIMER, Chandigarh, India

    Kirti Gupta

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  1. Madhivanan Karthigeyan
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  2. Parthasarathi Mondal
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  3. Pravin Salunke
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  4. Kirti Gupta
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  5. Anshul Siroliya
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Correspondence to Madhivanan Karthigeyan.

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Karthigeyan, M., Mondal, P., Salunke, P. et al. Extra-axial, dural-based atypical teratoid/rhabdoid tumor. Childs Nerv Syst 38, 655–658 (2022). https://doi.org/10.1007/s00381-021-05196-7

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  • DOI: https://doi.org/10.1007/s00381-021-05196-7

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