A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child.
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.
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
Dipanker Singh
DS
Mankotia
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
Vivek
V
Tandon
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.
Bhawani Shankar
BS
Sharma
Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India.
Madhu
M
Rajeshwari
Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India.
Mehar Chand
MC
Sharma
Pediatric spine; rhabdoid tumor; spinal tumor; teratoid tumor