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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...

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Detalles Bibliográficos
Autores principales: Mankotia, Dipanker Singh, Tandon, Vivek, Sharma, Bhawani Shankar, Rajeshwari, Madhu, Sharma, Mehar Chand
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4991152/
https://www.ncbi.nlm.nih.gov/pubmed/27606020
http://dx.doi.org/10.4103/1817-1745.187634
Descripción
Sumario: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.