Atypical Teratoid/Rhabdoid Tumor of the Spinal Wire in a Little one: Case Report and Complete Evaluation of the Literature.
Pediatr Neurosurg. 2018 Might 22;:1-9
Authors: Babgi M, Samkari A, Al-Mehdar A, Abdullah S
INTRODUCTION: Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system is characterised by SMARCB1/INI deletion or mutation within the lengthy arm of chromosome 22 11(22q11.2), additionally leading to lack of nuclear expression of INI1 protein immunohistochemically. AT/RT tumors often happen in kids under three years. The tumor is often seen within the cerebellum or the cerebrum, with a particularly uncommon incidence within the spinal twine.
MATERIALS AND METHODS: We report a uncommon case of AT/RT in a 6-year-old boy who had a main spinal twine lesion within the thoracolumbar junction. Pathology revealed lack of nuclear staining of INI1 immunohistochemically. That is the primary case reported with combined intraspinal lesion (intra- and extramedullary). The affected person underwent two surgical procedures and acquired radiotherapy and chemotherapy; nonetheless, he died 16 months after the preliminary presentation.
RESULTS AND DISCUSSION: We reviewed the literature on all kids with spinal twine AT/RT. The evaluate confirmed that the cervical area is the commonest location of origin, particularly in youthful kids. Reported instances had been handled with a mixture of surgical procedure, systemic and intrathecal chemotherapy, and radiation remedy, and a survival time of 18 months represented the perfect end result. General imply survival time was 10 months.
PMID: 29788028 [PubMed – as supplied by publisher]