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A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child
Dipanker Singh Mankotia1, Vivek Tandon1, Bhawani Shankar Sharma1
1Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
Abstract:
Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system neoplasm affecting children, and isolated primary spinal involvement is extremely rare. Authors describe a case of spinal AT/RT in a 5-year-old male child presenting with rapidly progressing quadriparesis diagnosed and managed surgically and medically. Biopsy revealed large, rhabdoid cells with prominent nucleoli in nest and immunohistochemistry further showed loss of integrase integrator 1 expression considered to be gold standard for diagnosis. AT/RT has extremely poor prognosis with median survival being 6 months.
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