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Table of Contents    
Year : 2020  |  Volume : 68  |  Issue : 6  |  Page : 1465-1468

Lateral Ventricle Atypical Teratoid/Rhabdoid Tumor (AT/RT): Case Report and Review of Literature

1 Department of Neurosurgery, AIIMS, Bhubaneswar, Odisha, India
2 Department of Pathology, AIIMS, Bhubaneswar, Odisha, India
3 Department of Neurosurgery, PGI, Chandigarh, India

Date of Web Publication19-Dec-2020

Correspondence Address:
Dr. Chinmaya Dash
Assistant Professor, Neurosurgery, AIIMS, Bhubaneswar - 751 019, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.304127

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 » Abstract 

A 4-year-old male child presented with features of raised intracranial pressure due to tumor in the left lateral ventricle with shunt blockage. Ventriculoperitoneal shunt was done earlier (one month ago). Craniotomy and gross total excision of the tumor was achieved. Histopathological examination was suggestive of Atypical Teratoid/Rhabdoid tumor. Patient relatives were not compliant with the advice for adjuvant therapy and patient expired after three months of definitive surgery due to aggressive course of the disease. To the best of our knowledge only six cases of AT/RT of the lateral ventricle in pediatric population has been described in literature. The tumor may mimic radiologically with benign pathology and can have an aggressive course with poor outcome. Differential diagnosis of AT/RT must be kept in cases of lateral ventricle tumor in pediatric population.

Keywords: Atypical teratoid/rhabdoid tumor, intraventricular teratoid/rhabdoid tumor, pediatric intraventricular atypical teratoid/rhabdoid tumor
Key Messages: ATRT of lateral ventricle is a rare entity in pediatric population and can radiologically mimic benign tumors. Differential diagnosis of ATRT must be kept in cases of pediatric lateral ventricle tumors.

How to cite this article:
Sharma A, Sable MN, Singla R, Dash C, Sahu RN. Lateral Ventricle Atypical Teratoid/Rhabdoid Tumor (AT/RT): Case Report and Review of Literature. Neurol India 2020;68:1465-8

How to cite this URL:
Sharma A, Sable MN, Singla R, Dash C, Sahu RN. Lateral Ventricle Atypical Teratoid/Rhabdoid Tumor (AT/RT): Case Report and Review of Literature. Neurol India [serial online] 2020 [cited 2021 Jan 27];68:1465-8. Available from:

Atypical teratoid/rhabdoid tumor (AT/RT) occurs predominantly in children less than two years with a predilection for posterior fossa.[1] They are usually associated with early CSF spread[2] and a poor outcome. Radiologically they can be confused with relatively benign tumors.[3] We present a rare case of AT/RT in lateral ventricle of a 4-year-old child with a poor outcome. To the best of our knowledge, only six cases of pediatric lateral ventricle AT/RT tumors have been described in the literature [Table 1].
Table 1: Table enumerating cases of paediatric lateral ventricle atypical teratoid /rhabdoid tumor described in literature

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 » Case Report Top

A 4-year-old male child presented with complaints of mild-to-moderately intense dull aching headache for one month associated with projectile vomiting for five days and 1 episode of generalized tonic-clonic seizure. Ventriculoperitoneal shunt was done one month back due to features of raised intracranial pressure. On examination, the patient was drowsy, visual acuity was 6/6 in both eyes, and fundoscopy revealed papilledema. Non-contrast CT (NCCT) scan of the head showed a large hyperdense tumor arising in the lateral ventricle region and causing obstructive hydrocephalus with blockage of the foramen of Monro. MRI Brain showed a well-circumscribed lesion in the left lateral ventricle which was iso-intense on T1 weighted images (T1WI), iso to hyperintense on T2 with patchy enhancement on postcontrast images [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e. Preoperative hormone profile and serum Beta human chorionic gonadotropin (HCG) and alpha-fetoprotein (AFP) were within normal limits. Left frontal-parietal craniotomy with trans-cortical, trans-ventricular approach was taken. The tumor was greyish-white, soft, friable, mildly vascular, and attached to the lateral wall of the frontal horn of the left lateral ventricle. Gross total excision of the tumor was achieved [Figure 2]a. Histopathology showed a highly cellular tumor with intermittent areas of hemorrhage and necrosis. Tumor cells were arranged in nests, cords, trabeculae, short fascicles and in perivascular location. There was a mixed population of tumor cell morphology ranging from rhabdoid cells to spindle, oval, and round cells. A good proportion of cells with rhabdoid morphology had eccentrically placed nucleus with fine chromatin, prominent nucleoli, eosinophilic cytoplasm with well-defined cytoplasmic border. Most of the cells had cytoplastic clearing and mitotic activity was brisk. Tumor cells were negative for INI-1 expression and positive for EMA, CD99, Vimentin, SMA, and CK (focal) [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d, [Figure 3]e, [Figure 3]f.
Figure 1: (a) NCCT head showing hyperdense tumor in the left lateral ventricle with areas of calcification and obstructive hydrocephalus. (b) T1W Image showing isointense tumor in the left lateral ventricle. (c) Post contrast MRI scan showing patchy enhancement of the tumor in the left lateral ventricle. (d) T2WI showing iso to hyperintense tumor in the left lateral ventricle. (e) Diffusion Weighted (DWI) MRI showing diffuse restriction of the tumor

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Figure 2: (a) Immediate post operative NCCT head showing gross total excision of the tumor. (b) NCCT head at two months follow-up showing metastasis in the posterior fossa (arrow mark)

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Figure 3: (a) Hematoxylin and Eosin(H&E) staining showing large and pleomorphic rhabdoid cells with abundant eosinophilic cytoplasm, with filamentous cytoplasmic inclusions and vacuoles along with eccentric round nuclei and prominent nucleolus(low magnification). (b) High magnification image of [Figure 3]a. (c) Immuno histochemistry (IHC) showing tumor is negative for EMA. (d) IHC showing tumor is strongly positive for CD 99. (e) IHC showing tumor is positive for SMA. (f) IHC showing tumor is negative for INI1. The arrow is showing control cells(lymphocytes) positive for INI1

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In the postoperative period patient had right hemiparesis (3/5) which improved in follow-up period over one month with power 4/5 on the right side. CSF cytology was positive for malignant cells suggestive of leptomeningeal spread. Patient had developed transient diabetes mellitus in postoperative period. Patient was advised adjuvant chemotherapy, which the parents were non-compliant with. At two months follow-up, patient presented with vomiting and altered sensorium. NCCT head revealed multiple metastasis in the posterior fossa [Figure 2]b. The parents did not agree for any further treatment and subsequently, the patient expired after three months of definitive surgery.

 » Discussion Top

ATRT is highly malignant CNS tumor and usually occurs in the posterior fossa in children less than 2 years.[1] Male children have a slightly higher preponderance for this tumor.[2] These tumors have a high propensity of CSF spread due to their spread along the Virchow Robin space and nearly a third of these patients present with CNS dissemination.[2]

Radiological findings of these tumors are nonspecific. A large posterior fossa tumor with bleed and CSF spread is highly suggestive.[3] Rapid growth of tumor is generally seen on follow-up imaging.[3] Radiological differential diagnosis includes PNET, medulloblastoma, teratoma, astrocytoma, choroid plexus papilloma, and ependymoma.[3]

Light microscopic examination features for ATRT include a diffuse growth pattern of predominantly polygonal cells which are arranged in a focally trabecular or alveolar fashion, cells with vesicular nuclei and prominent nucleoli, and scattered cells with globular hyaline cytoplasmic inclusions in the vicinity of the nuclei. Immunohistochemistry reveals that the tumors are positive for Epithelial Membrane Antigen (EMA), Vimentin, Smooth muscle actin (SMA) and Cytokeratin (CK). Genetic bi-allelic loss and/or negative immunohistochemistry staining of SMARCB1 or SMARCA4 and their respective gene products hSNF5/INI1/BAF47 and BRG1 are accepted diagnostic criteria for AT/RT.[7]

Treatment includes surgical excision followed by chemotherapy and/or radiotherapy. High dose chemotherapy (HDCT) with autologous stem cell rescue has increasingly become a popular and mainstay of adjuvant therapy following surgery. High-dose methotrexate in the induction phase followed by 1–3 cycles of consolidation therapy with etoposide, carboplatin, and thiotepa has been used.[8] Darmoul et al. have used BBSFOP protocol for chemotherapy in the case of lateral ventricular ATRT in a 2-month-old male patient and have reported survival at 2 years follow-up in that patient.[9] Patient (of AT/RT) older than 3 years who have received postoperative craniospinal radiation have had better outcomes as compared to children less than 3 years.[10]

 » Conclusion Top

ATRT of the lateral ventricle is a rare entity in pediatric population. They can have an aggressive course with a tendency for early leptomeningeal spread as has been demonstrated in our case. They can be confused radiologically with relatively benign tumors in this age group. Differential diagnosis of ATRT must be kept in cases of pediatric lateral ventricle tumors.

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 » References Top

Donovan DJ, Smith AB, Petermann GW. Atypical teratoid/rhabdoid tumor of the velum interpositum presenting as a spontaneous intraventricular hemorrhage in an infant: Case report with long-term survival. Pediatr Neurosurg 2006;42:187-92.  Back to cited text no. 1
Singh J, Kharosekar H, Velho V, Survashe P. Atypical rhabdoid tumor of lateral ventricle: Report of an unusual tumor. J Pediatr Neurosci 2016;11:64-7.  Back to cited text no. 2
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Lee IH, Yoo S-Y, Kim JH, Eo H, Kim OH, Kim I-O, et al. Atypical teratoid/rhabdoid tumors of the central nervous system: Imaging and clinical findings in 16 children. Clin Radiol 2009;64:256-64.  Back to cited text no. 3
Ahmad FU, Suri A, Mahapatra AK, Mehta VS, Garg A, Sharma MC, et al. Intraventricular rhabdoid tumor. Indian J Pediatr 2005;72:693-6.  Back to cited text no. 4
Meyers SP, Khademian ZP, Biegel JA, Chuang SH, Korones DN, Zimmerman RA. Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am J Neuroradiol 2006;27:962-71.  Back to cited text no. 5
Li F, Gui Q, Piao Y. Primary supratentorial atypical teratoid/rhabdoid tumor in children: A report of two cases. J Child Neurol 2013;28:399-403.  Back to cited text no. 6
Biegel JA. Molecular genetics of atypical teratoid/rhabdoid tumor. Neurosurg Focus 2006;20:E11.  Back to cited text no. 7
Fangusaro J, Finlay J, Sposto R, Ji L, Saly M, Zacharoulis S, et al. Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNETs): Report of the Head Start I and II experience. Pediatr Blood Cancer 2008;50:312-8.  Back to cited text no. 8
Darmoul M, Ben Nsir A, Chabchoub I, Hattab MN. Atypical teratoid rhabdoid tumor of the lateral ventricle. J Pediatr Neurosci 2015;10:382-5.  Back to cited text no. 9
[PUBMED]  [Full text]  
Tekautz TM, Fuller CE, Blaney S, Fouladi M, Broniscer A, Merchant TE, et al. Atypical teratoid/rhabdoid tumors (ATRT): Improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy. J Clin Oncol 2005;23:1491-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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