Skip to main content
SpringerLink
Log in

A case of atypical teratoid/rhabdoid tumor in an adult, with long survival

  • Case Report
  • Published:
Brain Tumor Pathology Aims and scope Submit manuscript

Abstract

Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant tumor that mostly occurs in early childhood and has poor prognosis despite aggressive therapy. Adult cases are rare and, as far as we are aware, only 30 cases have been reported to date. Here we present the case of a 27-year-old female with left parietal AT/RT with the chief complaint of numbness of the right superior limb. First, the tumor was surgically removed and the diagnosis was grade II glioma. With additional radiotherapy, the clinical course after surgery was favorable. After 6 years, she had an operation for recurrence and the diagnosis was grade III glioma. Temozolomide was prescribed, and a disease-free period of 2 years followed. Surgery was performed for a third time for second recurrence with histology of diffuse growth of rhabdoid cells. Immunohistochemistry was partially positive for vimentin and epithelial membrane antigen. Ki-67 labeling index was extremely high and tumor cells showed no staining of INI1 suggestive of diagnosis of AT/RT. We re-evaluated past specimens and none had immunoreactivity of INI1. Ki-67 labeling index and O-6 methylguanine DNA methyltransferase (MGMT) staining were also re-examined and both increased gradually. She is still alive without recurrence for more than 1 year. As far as we are aware, this is the second longest survival of an adult with AT/RT.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Lefkowitz IB, Rorke LB, Packer RJ (1987) Atypical teratoid tumor of infancy: definition of an entity. Ann Neurol 22:448–449

    Google Scholar 

  2. Rorke LB, Packer RJ, Biegel JA et al (1996) Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 85:56–65

    Article  CAS  PubMed  Google Scholar 

  3. Burger PC, Yu IT, Tihan T et al (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 22:1083–1092

    Article  CAS  PubMed  Google Scholar 

  4. Reddy AT (2005) Atypical teratoid/rhabdoid tumors of the central nervous system. J Neurooncol 75:309–313

    Article  CAS  PubMed  Google Scholar 

  5. Biswas A, Goyal S, Puri T et al (2009) Atypical teratoid rhabdoid tumor of the brain: case series and review of literature. Childs Nerv Syst 25:1495–1500

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  7. Versteege I, Sévenet N, Lange J et al (1998) Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer. Nature 394:203–206

    Article  CAS  PubMed  Google Scholar 

  8. Biegel JA, Zhou JY, Rorke LB et al (1999) Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res 59:74–79

    CAS  PubMed  Google Scholar 

  9. Rousseau-Merck MF, Versteege I, Legrand I et al (1999) hSNF5/INI1 inactivation is mainly associated with homozygous deletions and mitotic recombinations in rhabdoid tumors. Cancer Res 59:3152–3156

    CAS  PubMed  Google Scholar 

  10. Biegel JA, Kalpana G, Knudsen ES et al (2002) The role of INI1 and the SWI/SNF complex in the development of rhabdoid tumors: meeting summary from the workshop on childhood atypical teratoid/rhabdoid tumors. Cancer Res 62:323–328

    CAS  PubMed  Google Scholar 

  11. Judkins AR, Mauger J, Rorke LB et al (2004) Immunohistochemical analysis of hSNF5/INI1 in pediatric CNS neoplasms. Am J Surg Pathol 28:644–650

    PubMed  Google Scholar 

  12. Horn M, Schlote W, Lerch KD et al (1992) Malignant rhabdoid tumor: primary intracranial manifestation in an adult. Acta Neuropathol 83:445–448

    Article  CAS  PubMed  Google Scholar 

  13. Cossu A, Massarelli G, Manetto V et al (1993) Rhabdoid tumours of the central nervous system. Report of three cases with immunocytochemical and ultrastructural findings. Virchows Arch A Pathol Anat Histopathol 422:81–85

    Article  CAS  PubMed  Google Scholar 

  14. Fisher BJ, Siddiqui J, Macdonald D et al (1996) Malignant rhabdoid tumor of brain: an aggressive clinical entity. Can J Neurol Sci 23:257–263

    CAS  PubMed  Google Scholar 

  15. Ashraf R, Bentley RC, Awan AN et al (1997) Implantation metastasis of primary malignant rhabdoid tumor of the brain in an adult (one case report). Med Pediatr Oncol 28:223–227

    Article  CAS  PubMed  Google Scholar 

  16. Byram D et al (1998) (1999) Regarding Weiss, IJROBP 41: 103–109. Int J Radiat Oncol Biol Phys 45:247

    Google Scholar 

  17. Sugita Y, Takahashi Y, Hayashi I et al (1999) Pineal malignant rhabdoid tumor with chondroid formation in an adult. Pathol Int 49:1114–1118

    Article  CAS  PubMed  Google Scholar 

  18. Kuge A, Kayama T, Tsuchiya D et al (2000) Suprasellar primary malignant rhabdoid tumor in an adult: a case report (in Japanese). No Shinkei Geka 28:351–358

    CAS  PubMed  Google Scholar 

  19. Arrazola J, Pedrosa I, Méndez R et al (2000) Primary malignant rhabdoid tumour of the brain in an adult. Neuroradiology 42:363–367

    Article  CAS  PubMed  Google Scholar 

  20. Lutterbach J, Liegibel J, Koch D et al (2001) Atypical teratoid/rhabdoid tumors in adult patients: case report and review of the literature. J Neurooncol 52:49–56

    Article  CAS  PubMed  Google Scholar 

  21. Bruch LA, Hill DA, Cai DX et al (2001) A role for fluorescence in situ hybridization detection of chromosome 22q dosage in distinguishing atypical teratoid/rhabdoid tumors from medulloblastoma/central primitive neuroectodermal tumors. Hum Pathol 32:156–162

    Article  CAS  PubMed  Google Scholar 

  22. Pimentel J, Silva R, Pimentel T (2003) Primary malignant rhabdoid tumors of the central nervous system: considerations about two cases of adulthood presentation. J Neurooncol 61:121–126

    Article  PubMed  Google Scholar 

  23. Kachhara R, Retnam TM, Kumar S et al (2003) Rhabdoid tumor of the thalamus. Neurol India 51:273–274

    CAS  PubMed  Google Scholar 

  24. Kawaguchi T, Kumabe T, Watanabe M et al (2004) Atypical teratoid/rhabdoid tumour with leptomeningeal dissemination in an adult. Acta Neurochir (Wien) 146:1033–1038 (discussion 1038)

    Google Scholar 

  25. Erickson ML, Johnson R, Bannykh SI et al (2005) Malignant rhabdoid tumor in a pregnant adult female: literature review of central nervous system rhabdoid tumors. J Neurooncol 74:311–319

    Article  PubMed  Google Scholar 

  26. Raisanen J, Biegel JA, Hatanapaa KJ et al (2005) Chromosome 22q deletion in atypical teratoid/rhabdoid tumors in adults. Brain Pathol 15:23–28

    Article  CAS  PubMed  Google Scholar 

  27. Chen YW, Wong TT, Ho DM et al (2006) Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience). Int J Radiat Oncol Biol Phys 64:1038–1043

    Article  PubMed  Google Scholar 

  28. Rezanko T, Tunakan M, Kaharaman A et al (2006) Primary rhabdoid tumor of the brain in an adult. Neuropathology 26:57–61

    Article  PubMed  Google Scholar 

  29. Ingold B, Moschopulos M, Hutter G et al (2006) Abdominal seeding of an atypical teratoid/rhabdoid tumor of the pineal gland along a ventriculoperitoneal shunt catheter. Acta Neuropathol 111:56–59

    Article  PubMed  Google Scholar 

  30. Chacko G, Chacko AG, Dunham CP et al (2007) Atypical teratoid/rhabdoid tumor arising in the setting of a pleomorphic xanthoastrocytoma. J Neurooncol 84:217–222

    Article  PubMed  Google Scholar 

  31. Zarovnaya EL, Pallatroni HF, Hug EB et al (2007) Atypical teratoid/rhabdoid tumor of the spine in an adult: case report and review of the literature. J Neurooncol 84:49–55

    Article  PubMed  Google Scholar 

  32. Arita K, Sugiyama K, Sano T et al (2008) Atypical teratoid/rhabdoid tumor in sella turcica in an adult. Acta Neurochir (Wien) 150:491–495 (discussion 496)

    Google Scholar 

  33. Makuria AT, Rushiing EJ, McGrail KM et al (2008) Atypical teratoid rhabdoid tumor (AT/RT) in adults: review of four cases. J Neurooncol 88:321–330

    Article  PubMed  Google Scholar 

  34. Samaras V, Stamatelli A, Samaras E et al (2009) Atypical teratoid/rhabdoid tumor of the central nervous system in an 18-year-old patient. Clin Neuropathol 28:1–10

    CAS  PubMed  Google Scholar 

  35. Takei H, Adesina AM, Mehta V et al (2010) Atypical teratoid/rhabdoid tumor of the pineal region in an adult. J Neurosurg 113:374–379

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

We thank Eiko Aoyanagi, Miho Nodagashira, and Kazuko Shimizu for their excellent technique. This study is supported by the grant from Ministry of Education, Culture, Sports, Science, and Technology, Japan.

Author information

Authors and Affiliations

  1. Laboratory of Cancer Research, Department of Pathology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-Ku, Sapporo, Hokkaido, Japan

    Kenta Takahashi, Tomoaki Yoshinaga, Roshan Mahabir, Hiromi Kanno, Taichi Kimura, Mishie Tanino & Shinya Tanaka

  2. Department of Translational Pathology, Hokkaido University Graduate School of Medicine, Sapporo, Japan

    Hiroshi Nishihara & Shinya Tanaka

  3. Hokkaido Neurosurgical Memorial Hospital, Sapporo, Japan

    Masahito Katoh

  4. Sapporo IGRT Clinic, Sapporo, Japan

    Jun Ikeda

  5. Department of Neurosurgery, Hokkaido University Graduate School of Medicine, Sapporo, Japan

    Yutaka Sawamura

  6. Department of Pathology, Sapporo Higashi-Tokushukai Hospital, Sapporo, Japan

    Kazuo Nagashima

Authors
  1. Kenta Takahashi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Hiroshi Nishihara
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Masahito Katoh
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Tomoaki Yoshinaga
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Roshan Mahabir
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Hiromi Kanno
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Taichi Kimura
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Mishie Tanino
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Jun Ikeda
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Yutaka Sawamura
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Kazuo Nagashima
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Shinya Tanaka
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Shinya Tanaka.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Takahashi, K., Nishihara, H., Katoh, M. et al. A case of atypical teratoid/rhabdoid tumor in an adult, with long survival. Brain Tumor Pathol 28, 71–76 (2011). https://doi.org/10.1007/s10014-010-0008-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10014-010-0008-y

Keywords

Search

Navigation