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CASE REPORT
Year : 2009  |  Volume : 5  |  Issue : 4  |  Page : 318-320

Giant lumbar paraspinal atypical teratoid/rhabdoid tumor in a child


1 Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha, India
2 Department of Pathology, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha, India

Date of Web Publication11-Feb-2010

Correspondence Address:
Amit Agrawal
Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha- 442 004, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.59906

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 > Abstract 

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly aggressive and uncommon tumor of the central nervous system, primarily affecting young children. AT/RT of the paraspinal region with involvement of the spine and spinal cord is extremely rare, with only few case reports in the literature. We report an unusual case of giant lumbar paraspinal AT/RT with intraspinal extension in a previously healthy 18-month-old female child. To the best of our knowledge, this kind of presentation has not been reported previously in the English literature.

Keywords: Atypical teratoid/rhabdoid tumor, spinal tumors, paraspinal tumors, computerized tomography, pediatric spinal tumor, rhabdoid tumor


How to cite this article:
Agrawal A, Bhake A, Cincu R. Giant lumbar paraspinal atypical teratoid/rhabdoid tumor in a child. J Can Res Ther 2009;5:318-20

How to cite this URL:
Agrawal A, Bhake A, Cincu R. Giant lumbar paraspinal atypical teratoid/rhabdoid tumor in a child. J Can Res Ther [serial online] 2009 [cited 2020 Jul 7];5:318-20. Available from: http://www.cancerjournal.net/text.asp?2009/5/4/318/59906


 > Introduction Top


Atypical teratoid/rhabdoid tumor (AT/RT) is a highly aggressive and uncommon tumor of the central nervous system, primarily affecting young children; it has a rapidly progressive course and a fatal outcome. [1],[2],[3],[4] AT/RT of the paraspinal region, the spine, and the spinal cord is extremely rare, with only few case reports in the literature. [1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11] We report an unusual case of a giant lumbar paraspinal AT/RT with intraspinal extension in a child. To the best of our knowledge, this kind of presentation has not been reported previously in the English literature.


 > Case Report Top


A previously healthy 18-month-old female child presented with a progressively increasing painful swelling over the left lumbar paraspinal region. There was no history of hematuria and fever. Her general and systemic examinations were normal. Local examination revealed a large, firm, tender lump over the left lumbar paraspinal region [Figure 1]. The lump was non-ballotable and was not palpable per abdomen. Plain computerized tomography (CT) scan showed a large, well-defined, isodense, lobulated mass in the left paraspinal area, with erosion of the transverse process of the 2 nd lumbar vertebra [Figure 2] A-C. The mass showed wavy contrast enhancement [Figure 2] B and C. The left kidney was seen well separated from the mass and was pushed anteriorly and laterally [Figure 2]B. The patient underwent surgery and near-total excision of the tumor was performed [Figure 3]A-F. Histopathological examination showed a tumor containing primitive neuroectodermal and rhabdoid cells. The rhabdoid component was characterized by medium-sized, round to oval cells with eccentric nuclei, prominent nucleoli, and eosinophilic cytoplasm. There were frequent aberrant mitotic activity [Figure 4] A and B. Based on all these histomorphological features, a diagnosis of atypical teratoid rhabdoid tumor (AT/RT WHO grade IV) was made. The patient was started on chemotherapy. Two months after surgery the patient presented with worsening of pain and weakness of both lower limbs. A repeat CT scan revealed extensive recurrence of the lesion.


 > Discussion Top


Rorke et al. termed these lesions as 'atypical teratoid/rhabdoid tumors' (AT/RT) to highlight the fact that these are distinct central nervous system (CNS) neoplasms with a disparate combination of rhabdoid, primitive neuroepithelial, epithelial, and mesenchymal components. [1],[12] AT/RT arising from the paraspinal region and involving the spine is rare and is usually seen in young children (<3 years), with no predilection for either sex. [1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11] Imaging findings in intracranial AT/RT have been well documented; [1],[2],[3],[4] however, the imaging findings in paraspinal and spinal AT/RT are nonspecific. [1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11] On imaging, these lesions appears as a large-sized, heterogeneous masses, which correlates well with their histopathologic complexity and aggressive nature. [5],[7] Warmuth-Metz et al. have described a distinct and unusual pattern of a wavy, band-like, enhancement surrounding a central hypointensity [Figure 2] B and C, a typical feature of AT/RTs; [3] this pattern, although uncommon, was seen in the present case also. It has been recommended that, despite their nonspecific imaging features, rhabdoid tumor be included in the differential diagnosis of childhood intracranial and paraspinal region neoplasms. [2] Histologically, AT/RTs contain nests or sheets of rhabdoid tumor cells. In addition, they show the multiple-lineage developmental characteristics of malignant teratomas of neuroectoderm, mesoderm, and endoderm. AT/RT also express a wide range of immunohistochemical markers, including vimentin, epithelial membrane antigen, cytokeratin, synaptophysin, glial fibrillary acidic protein, and smooth muscle actin. [1, 2, 6, 11, 12] It has been suggested that recognition of the rhabdoid element is critical because this phenotype correlates with a significantly worse prognosis than the classic primitive neuroectodermal (PNET)/medulloblastoma (MB). [13],[14] The treatment of patients with AT/RT is complex and many current treatment regimens for AT/RTs are derived from the strategies used for management of PNET/ MB (i.e., radical surgery, chemotherapy, and radiotherapy). [4],[13],[14],[15] The outcome of patients with AT/RT is uniformly poor despite aggressive surgical and adjuvant radiochemotherapy, [1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[13],[14],[15] and this was true in the present case also.

 
 > References Top

1.Rorke LB, Packer RJ, Biegel JA. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: Definition of an entity. J Neurosurg 1996;85:56-65  Back to cited text no. 1
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2.Howlett DC, King AP, Jarosz JM, Stewart RA, al-Sarraj ST, Bingham JB, et al. Imaging and pathological features of primary malignant rhabdoid tumours of the brain and spine. Neuroradiology 1997;39:719-23.   Back to cited text no. 2
    
3.Warmuth-Metz M, Bison B, Dannemann-Stern E, Kortmann R, Rutkowski S, Pietsch T. CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system. Neuroradiology 2008;50:447-52.   Back to cited text no. 3
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4.Yano S, Hida K, Kobayashi H, Iwasaki Y. Successful multimodal therapies for a primary atypical teratoid/rhabdoid tumor in the cervical spine. Pediatr Neurosurg 2008;44:406-13.   Back to cited text no. 4
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5.Cheng YC, Lirng JF, Chang FC, Guo WY, Teng MM, Chang CY, et al. Neuroradiological findings in atypical teratoid/rhabdoid tumor of the central nervous system. Acta Radiol 2005;46:89-96.  Back to cited text no. 5
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6.Bambakidis NC, Robinson S, Cohen M, Cohen AR. Atypical teratoid/rhabdoid tumors of the central nervous system: Clinical, radiographic and pathologic features. Pediatr Neurosurg 2002;37:64-70.   Back to cited text no. 6
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7.Tamiya T, Nakashima H, Ono Y, Kawada S, Hamazaki S, Furuta T, et al. Spinal atypical teratoid/rhabdoid tumor in an infant. Pediatr Neurosurg 2000;32:145-9.   Back to cited text no. 7
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8.Rosemberg S, Menezes Y, Sousa MR, Plese P, Ciquini O. Primary malignant rhabdoid tumor of the spinal dura. Clin Neuropathol 1994;13:221-4.  Back to cited text no. 8
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9.Moeller KK, Coventry S, Jernigan S, Moriarty TM. Atypical Teratoid/Rhabdoid tumor of the spine. AJNR Am J Neuroradiol 2007;28:593-5.   Back to cited text no. 9
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10.Tanizaki Y, Oka H, Utsuki S, Shimizu S, Suzuki S, Fujii K. Atypical teratoid/rhabdoid tumor arising from the spinal cord: Case report and review of the literature. Clin Neuropathol 2006;25:81-5.  Back to cited text no. 10
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11.Kodama H, Maeda M, Imai H, Matsubara T, Taki W, Takeda K. MRI of primary spinal atypical teratoid/rhabdoid tumor: A case report and literature review. J Neurooncol 2007;84:213-6.   Back to cited text no. 11
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12.Rorke LB, Biegel JA: Atypical teratoid/rhabdoid tumor. In: Kleihues P, Cavenee WK editors. World Health Organization classification of tumours in pathology and genetics. tumours of the nervous system. Lyon: IARC Press; 2000. p. 145-8.  Back to cited text no. 12
    
13.Weiss E, Behring B, Behnke J, Christen HJ, Pekrun A, Hess CF. Treatment of primary malignant rhabdoid tumor of the brain: Report of three cases and review of the literature. Int J Radiat Oncol Biol Phys 1998;411013-9.   Back to cited text no. 13
    
14.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. 14
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15.Madigan CE, Armenian SH, Malogolowkin MH, Mascarenhas L. Extracranial malignant rhabdoid tumors in childhood the childrens hospital los angeles experience. Cancer 2007;110:2061-6.  Back to cited text no. 15
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    Figures

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


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