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Year : 2020  |  Volume : 16  |  Issue : 8  |  Page : 243-245

Brain atypical teratoid rhabdoid tumor in an adult with long-term survival: Case report and review of literature

1 Department of Radiotherapy, Abderrahmen Mami Hospital, Ariana, Faculty of Medicine of Tunis, University of Tunis-El Manar, Tunisia
2 Department of Neurosurgery, National Institute of Neurology, Tunis, Faculty of Medicine of Tunis, University of Tunis-El Manar, Tunisia
3 Department of Pathology, Rabta Hospital, Tunis, Faculty of Medicine of Tunis, University of Tunis-El Manar, Tunisia
4 Department of Pathology, Habib Bourguiba Hospital, Sfax, Faculty of Medicine of Sfax, University of Sfax, Tunisia
5 Department of Neuroradiology, National Institute of Neurology, Tunis, Faculty of Medicine of Tunis, University of Tunis-El Manar, Faculty of Medicine of Tunis, University of Tunis-El Manar, Tunis, Tunisia

Date of Submission22-Aug-2018
Date of Decision13-Nov-2018
Date of Acceptance24-Mar-2020
Date of Web Publication19-Aug-2020

Correspondence Address:
Khalil Ghedira
Department of Neurosurgery, National Institute of Neurology, Rabta, 1007, Tunis
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_554_18

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 > Abstract 

Atypical teratoid/rhabdoid tumor (AT/RT) represents a rare malignant embryonic tumor of infant and early childhood. Its prognosis remains dismal despite aggressive multimodal treatment. We report the case of a 24-year-old male who was diagnosed with left parietal AT/RT after total resection and who is still in good health and recurrence free 4 years after surgery and adjuvant chemotherapy and radiotherapy.

Keywords: Adult, atypical teratoid/rhabdoid tumor, brain, survival

How to cite this article:
Moujahed R, Ghedira K, Zehani A, Charfi S, Nagi S. Brain atypical teratoid rhabdoid tumor in an adult with long-term survival: Case report and review of literature. J Can Res Ther 2020;16, Suppl S1:243-5

How to cite this URL:
Moujahed R, Ghedira K, Zehani A, Charfi S, Nagi S. Brain atypical teratoid rhabdoid tumor in an adult with long-term survival: Case report and review of literature. J Can Res Ther [serial online] 2020 [cited 2021 Jan 25];16:243-5. Available from: https://www.cancerjournal.net/text.asp?2020/16/8/243/292707

 > Introduction Top

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare and malignant embryonic tumor of the central nervous system (CNS). It occurs mainly in children younger than 3 years.[1] This neoplasm is associated with an extremely poor prognosis, with survival seldom exceeding 1 year.[2] We describe a case of brain AT/RT in a young adult patient with an event-free survival of 4 years.

 > Case Report Top

A 24-year-old, right-handed young male presented with a 1-month history of headache with right hemi-body tingling and weakness. On admission, he had a right-sided hemiparesis with Gerstmann syndrome.

Brain computed tomography (CT) scan revealed a left parietal isodense mass including focal calcifications, with marked contrast enhancement and areas of necrosis. Magnetic resonance imaging (MRI) showed intra-axial heterogeneous lobulated tumor surrounded by peritumoral edema [Figure 1]a, [Figure 1]b, [Figure 1]c.
Figure 1: Supratentorial atypical teratoid/rhabdoid tumor: axial computed tomography without contrast (a). Parietal spontaneously isodense mass with nodular calcifications. Magnetic resonance imaging: axial fluid-attenuated inversion recovery (b) and axial T1 postcontrast (c) weighted images. Left parietal solid mass with lobulated margin. This lesion is iso and hyperintense on fluid-attenuated inversion recovery with vasogenic edema and mass effect on the lateral ventricle and midline. It is intense but with heterogeneous enhancement. Axial T1 postcontrast images after treatment (d). Remission with slight hypointensity without enhancement

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A left parietal craniotomy was achieved, and gross total removal of the tumor was performed. The lesion was described as grayish, fibrous, and hemorrhagic. The postoperative course was uneventful.

Pathological study revealed a highly dense tumor. The tumor cells were large, were plump and polyhedral or fusiform, and holding intracytoplasmic eosinophil inclusions. They harbored prominent nuclei with eccentric nucleoli, corresponding to rhabdoid morphology. Immunohistochemistry staining for vimentin and epithelial membrane antigen was positive, while INI 1 one was negative [Figure 2]. The Ki-67 labeling index was 10%. These findings were compatible with the diagnosis of AT/RT.
Figure 2: Photomicrographs of the pathological sample. Rhabdoid cells with abundant eosinophilic cytoplasm, vesicular chromatin, and prominent nucleoli (a, H and E, ×40). Tumor cells showing strong expression of vimentin (b) and diffuse positivity for epithelial membrane antigen (c). INI1 staining is negative (d)

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A lumbar puncture was performed at the 10th postoperative day to evaluate for CNS drop metastases, and no malignant cells were identified. The patient received chemotherapy according to the Medical University of Vienna-AT/RT regimen[3] consisting of three 9-week courses of a dose-dense regimen including doxorubicin, cyclophosphamide, vincristine, ifosfamide, cisplatin, etoposide, and methotrexate augmented with intrathecal therapy, followed by high-dose chemotherapy. Then, he underwent conformal three-dimensional radiotherapy to tumor bed at 54 Gy (fractions of 1.8 Gy). This chemoradiation adjuvant treatment was overall well tolerated.

At 1 year after treatment, the patient recovered normal motility, and control MRI showed no residual tumor or recurrence [Figure 1]d. At the last routine follow-up, 4 years after surgery, the patient remains symptom free.

 > Discussion Top

AT/RT is a rare Grade IV embryonic tumor according to the World Health Organization classification of CNS tumors.[4] Children under 3 years of age are the most involved population.[1]

To the best of our knowledge, about sixty cases of adult-onset AT/RT are reported in the literature, with only eight cases of brain AT/RT with survival for more than 3 years [Table 1].[5],[6],[7],[8]
Table 1: Brain atypical teratoid/rhabdoid tumor adult cases with survival >3 years

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Adult AT/RT involves mostly brain hemisphere or sellar and suprasellar region, while pediatric form is quite often infratentorial.[5]

AT/RT has no specific neuroimaging features. On CT scan, the lesion is iso to hyperdense with eventual calcifications and enhancing heterogeneously after contrast medium administration.[15] MRI scan shows classically a large mass, hypo-intense on T1-weighted images and iso to hyper-intense on T2-weighted images, with a thick and irregular enhancing wall surrounding a central cystic region.[16],[17]

The diagnosis of AT/RT is suspected on pathological study, revealing rhabdoid cell morphology with possible combination to primitive neuroepithelial, epithelial, and mesenchymal components. Confirmation is based on immunohistochemistry: tumor cells are negative for INI 1, or by the fluorescence in situ hybridization method.[18]

Due to the rarity of AT/RT, no consensual treatment has been established yet. The actual optimal treatment is based on gross total resection followed by adjuvant radiotherapy and intense chemotherapy.[5]

The prognosis of AT/RT is particularly poor because of the high incidence of local recurrence and leptomeningeal dissemination.[2] Adult-onset AT/RT has a better outcome than pediatric AT/RT, probably due to the ability to treat more aggressively. In the last literature review of adult AT/RT cases (54 cases), the median survival rate was 18 months (0.5–204 months).[5] Cases of brain AT/RT with survival >3 years [Table 1] were predominantly cases harboring brain hemisphere tumor. Among them, patients who experienced tumor recurrence did not receive optimal treatment initially: gross total resection plus chemotherapy and radiotherapy.

To date, no biological prognostic factor has been determined for AT/RT that could explain long-term survival cases.[19]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 > References Top

Ostrom QT, Chen Y, M de Blank P, Ondracek A, Farah P, Gittleman H, et al. The descriptive epidemiology of atypical teratoid/rhabdoid tumors in the United States, 2001-2010. Neuro Oncol 2014;16:1392-9.  Back to cited text no. 1
Buscariollo DL, Park HS, Roberts KB, Yu JB. Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a surveillance, epidemiology, and end results analysis. Cancer 2012;118:4212-9.  Back to cited text no. 2
Slavc I, Chocholous M, Leiss U, Haberler C, Peyrl A, Azizi AA, et al. Atypical teratoid rhabdoid tumor: Improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna experience 1992-2012. Cancer Med 2014;3:91-100.  Back to cited text no. 3
Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, et al. The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol 2002;61:215-25.  Back to cited text no. 4
Wu WW, Bi WL, Kang YJ, Ramkissoon SH, Prasad S, Shih HA, et al. Adult atypical teratoid/rhabdoid tumors. World Neurosurg 2016;85:197-204.  Back to cited text no. 5
Takahashi-Fujigasaki J, Matumoto M, Kan I, Oka H, Yasue M. Atypical teratoid/rhabdoid tumor with 26-year overall survival: Case report. J Neurosurg Pediatr 2012;9:400-5.  Back to cited text no. 6
Horiguchi H, Nakata S, Nobusawa S, Uyama S, Miyamoto T, Ueta H, et al. Adult-onset atypical teratoid/rhabdoid tumor featuring long spindle cells with nuclear palisading and perivascular pseudorosettes. Neuropathology 2017;37:52-7.  Back to cited text no. 7
Almalki MH, Alrogi A, Al-Rabie A, Al-Dandan S, Altwairgi A, Orz Y. Atypical teratoid/rhabdoid tumor of the sellar region in an adult with long survival: Case report and review of the literature. J Clin Med Res 2017;9:216-20.  Back to cited text no. 8
Horn M, Schlote W, Lerch KD, Steudel WI, Harms D, Thomas E. Malignant rhabdoid tumor: Primary intracranial manifestation in an adult. Acta Neuropathol 1992;83:445-8.  Back to cited text no. 9
Byram D. Regarding Weiss et al., IJROBP 41:103-109; 1998. Int J Radiat Oncol Biol Phys 1999;45:247.  Back to cited text no. 10
Chen YW, Wong TT, Ho DM, Huang PI, Chang KP, Shiau CY, et al. Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience). Int J Radiat Oncol Biol Phys 2006;64:1038-43.  Back to cited text no. 11
Makuria AT, Rushing EJ, McGrail KM, Hartmann DP, Azumi N, Ozdemirli M. Atypical teratoid rhabdoid tumor (AT/RT) in adults: Review of four cases. J Neurooncol 2008;88:321-30.  Back to cited text no. 12
Takahashi K, Nishihara H, Katoh M, Yoshinaga T, Mahabir R, Kanno H, et al. Case of atypical teratoid/rhabdoid tumor in an adult, with long survival. Brain Tumor Pathol 2011;28:71-6.  Back to cited text no. 13
Slemp SN, Martin SE, Zhang S, Ulbright TM, Cheng L, Hattab EM. Atypical teratoid/rhabdoid tumour in an adult with disseminated mediastinal germ cell tumour. Neuropathol Appl Neurobiol 2014;40:789-93.  Back to cited text no. 14
Han L, Qiu Y, Xie C, Zhang J, Lv X, Xiong W, et al. Atypical teratoid/rhabdoid tumors in adult patients: CT and MR imaging features. AJNR Am J Neuroradiol 2011;32:103-8.  Back to cited text no. 15
Kanoto M, Toyoguchi Y, Hosoya T, Kuchiki M, Sugai Y. Radiological image features of the atypical teratoid/rhabdoid tumor in adults: A systematic review. Clin Neuroradiol 2015;25:55-60.  Back to cited text no. 16
Au Yong KJ, Jaremko JL, Jans L, Bhargava R, Coleman LT, Mehta V, et al. How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors? Pediatr Radiol 2013;43:347-54.  Back to cited text no. 17
Güner G, Önder S, Söylemezoǧlu F. Cytomorphological features of atypical teratoid/rhabdoid tumor: An account of 12 years' experience. Diagn Cytopathol 2014;42:856-62.  Back to cited text no. 18
Sredni ST, Huang CC, Pundy T, Patel K, Halpern AL, Grupenmacher AT, et al. A gene signature for a long-term survivor of an atypical teratoid/rhabdoid tumor. Cancer Genet 2014;207:420-4.  Back to cited text no. 19


  [Figure 1], [Figure 2]

  [Table 1]


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