Open AccessA case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child
Author(s) -
Dipanker Singh Mankotia,
Vivek Tandon,
Bhawani Shankar Sharma,
Madhu Rajeshwari,
Mehar Chand Sharma
Publication year - 2016
Publication title -
journal of pediatric neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.247
H-Index - 18
eISSN - 1998-3948
pISSN - 1817-1745
DOI - 10.4103/1817-1745.187634
Subject(s) - medicine , atypical teratoid rhabdoid tumor , pathology , immunohistochemistry , biopsy
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.