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01-02-2011 | Case Report

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

Authors: Kenta Takahashi, Hiroshi Nishihara, Masahito Katoh, Tomoaki Yoshinaga, Roshan Mahabir, Hiromi Kanno, Taichi Kimura, Mishie Tanino, Jun Ikeda, Yutaka Sawamura, Kazuo Nagashima, Shinya Tanaka

Published in: Brain Tumor Pathology | Issue 1/2011

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.

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

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 85:56–65CrossRefPubMed

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 22:1083–1092CrossRefPubMed

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

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 25:1495–1500CrossRefPubMed

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

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

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

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 59:3152–3156PubMed

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 62:323–328PubMed

11.

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

12.

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

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 422:81–85CrossRefPubMed

14.

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

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 28:223–227CrossRefPubMed

16.

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

17.

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

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 28:351–358PubMed

19.

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

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 52:49–56CrossRefPubMed

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 32:156–162CrossRefPubMed

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 61:121–126CrossRefPubMed

23.

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

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)

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 74:311–319CrossRefPubMed

26.

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

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 64:1038–1043CrossRefPubMed

28.

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

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 111:56–59CrossRefPubMed

30.

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

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 84:49–55CrossRefPubMed

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)

33.

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

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 28:1–10PubMed

35.

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

Title: A case of atypical teratoid/rhabdoid tumor in an adult, with long survival
Authors: Kenta Takahashi
Hiroshi Nishihara
Masahito Katoh
Tomoaki Yoshinaga
Roshan Mahabir
Hiromi Kanno
Taichi Kimura
Mishie Tanino
Jun Ikeda
Yutaka Sawamura
Kazuo Nagashima
Shinya Tanaka
Publication date: 01-02-2011
Publisher: Springer Japan
Published in: Brain Tumor Pathology / Issue 1/2011
Print ISSN: 1433-7398
Electronic ISSN: 1861-387X
DOI: https://doi.org/10.1007/s10014-010-0008-y

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

Abstract

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