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Year : 2005  |  Volume : 15  |  Issue : 4  |  Page : 529-530
Rhabdoid tumor of brain - an unusual entity


Departments of Radiodiagnosis and Pathology, Dayanand Medical College and Hospital, Ludhiana (Punjab), India

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Keywords: Rhabdoid tumor, CT, Brain

How to cite this article:
Ahluwalia A, Sagar K, Sandhu P, Malhotra V, Bajwa R. Rhabdoid tumor of brain - an unusual entity. Indian J Radiol Imaging 2005;15:529-30

How to cite this URL:
Ahluwalia A, Sagar K, Sandhu P, Malhotra V, Bajwa R. Rhabdoid tumor of brain - an unusual entity. Indian J Radiol Imaging [serial online] 2005 [cited 2020 Apr 6];15:529-30. Available from: http://www.ijri.org/text.asp?2005/15/4/529/28789

   Introduction Top


Malignant rhabdoid tumor (MRT) is an uncommon childhood neoplasm that usually arises within the kidney. Primary extra renal rhabdoid tumors are rare [1]. We report a case of primary MRT of the brain.


   Case report Top


A 12-year-old male child presented with history of headache, vomiting, fever and altered sensorium since 15 days.

On neurological examination, the child had decorticate posturing with GCS of E1V1M 5. The muscle tone was increased and plantar was extensor. Pupils were mildly dilated bilaterally and showed sluggish reaction to light.

CT showed a heterogenous predominantly hyperdense SOL in right parietal region adjacent to the atrium of right lateral ventricle. Perilesional white matter edema was present. A focus of calcification was seen at its periphery [Figure - 1]. No significant enhancement was seen on intravenous contrast administration [Figure - 2]. In view of the imaging appearance and age of the patient, a provisional diagnosis of primitive neuroectodermal tumor (PNET) was made. The tumor could be only partially resected on surgery. Histopathology revealed a cellular tumor with areas of hemorrhage. The tumor was predominantly composed of rhabdoid cells having round to oval nuclei and prominent nucleoli with abundant eosinophilic cytoplasm. No area of small cell embryonic component (PNET) was identified [Figure - 3].


   Discussion Top


Malignant rhabdoid tumor (MRT) is a rare, extremely aggressive childhood neoplasm, which typically arises in the kidney. It was initially reported as sarcomatoid variant of Wilm's tumor [2]. The term "rhabdoid" was coined due to similarity of these tumors to rhabdomyosarcoma under the light microscope. However, more studies using immunohistochemical and ultrastructural analysis have shown MRT to be unrelated to either Wilm's or rhabdomyosarcoma [3]. The exact etiology of MRT is unclear, although a possible neuroectodermal origin has been suggested [4]

Although MRT typically arise within the kidney, several cases of extra renal origin have also been reported. These include the paravertebral region, chest wall, liver, pelvis, prostate, soft tissues and brain [5].

Primary MRT of brain is common in infancy and early childhood. The average age of presentation is 2 years with a 3:1 male predominance. On histology, the tumor contains fields of rhabdoid cells having eccentric nuclei, "owl's eye" nucleoli, abundant eosinophilic cytoplasm and cytoplasmic inclusions. Mitosis is common [5],[6]. Approximately two-thirds of tumors have areas of classical PNET and remaining have neoplastic mesenchymal and/or epitheliod areas. On electron microscopy, cytoplasm show whorls of filaments in paranuclear areas corresponding to glassy hyaline inclusions visible under light microscope [6],[7]. Immunohistochemical staining shows these filaments to be positive for vimentin, cytokeratin and epithelial membrane antigen (EMA).

The imaging features of cranial MRT are nonspecific. CT shows an isodense to slightly hyperdense mass relative to the grey matter with perilesional edema. Necrosis and calcification may be seen [8]. On MR, the signal characteristic of solid component is similar to grey matter on T1 and T2 weighted images. Diffuse or patchy enhancement of solid component is seen following contrast administration [6].

Primary cerebral MRT does show a strong tendency to spread and relapse with leptomeningial involvement. The prognosis in childhood cases is poor. Chemotherapy is usually not effective although high dose radiotherapy may induce local remission. The differential diagnosis is from primitive neuroectodermal tumors (PNET), as the imaging findings are nonspecific. Histopathology and immunohistochemistry can effectively distinguish these two entities [9].

Although MRT is a rare tumor and imaging findings are not specific, it should be included in the differential diagnosis of childhood intracranial and spinal neoplasms.[10]

 
   References Top

1.Howlett DC, Jarosz JM, Stewart RAL, Bingham JB, Cox TCS. Imaging and pathological features of primary malignant rhabdoid tumors of the brain and spine. Neuroradiology 1997;39:719-23  Back to cited text no. 1    
2.Beckwith JB. Histopathology and prognosis of Wilm's tumor: results from the first national Wilm's tumor study. Cancer 1978;41:1937-48  Back to cited text no. 2    
3.Biggs PJ, Garen PD, Power JM, Gavin AJ. Malignant rhabdoid tumor of central nervous system. Human Pathology 1987;18:332-7  Back to cited text no. 3    
4.Haas JE, Palmer NF, Weinberg DG, Beckwith JB. Ultrastructure of malignant rhabdoid tumor of the kidney; a distinctive renal tumor of children. Human Pathology 1981;12:646-57  Back to cited text no. 4    
5.Hanna SL, Langston JW, Parham DM, Douglass EC. Primary malignant rhabdoid tumor of the brain: clinical, imaging and pathological findings. AJNR 1993;14:107-15  Back to cited text no. 5    
6.Arrazola J, Pedrosa I, Mendez R, Saldana C, Scheithauer BW, Martinez A. Primary malignant rhabdoid tumor of the brain in an adult. Neuroradiology 2000;42:363-7  Back to cited text no. 6    
7.Kumar R. Primary malignant rhabdoid tumor of brain, clinicoradiological findings of two cases. Neurology (India) 1999;47: 314-7  Back to cited text no. 7    
8.Ho PSP, Lee WH, Chen CY. Primary malignant rhabdoid tumor of the brain-CT characteristics. J Comput Assist Tomogr 1990;14:461-3  Back to cited text no. 8    
9.Caldemeyer S, Smith RR, Azzarelli B, Boaz JC. Primary central nervous system malignant rhabdoid tumor: CT and MR appearance simulates a primitive neuroectodermal tumor. Pediatr Neurosurg 1994;21:232-6  Back to cited text no. 9    
10.Ho DMT, Hsu CY, Ting LT, Chiang H. Rhabdoid tumor of central nervous system: a comparative study with primitive neuroectodermal tumor/medulloblastoma. Acta Neuropathol 2000;99:482-8.  Back to cited text no. 10    

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Correspondence Address:
A Ahluwalia
125-D, Kitchlu Nagar, Ludhiana-141001, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.28789

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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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