Abstract
Pediatric spinal tumors are rare and account for 10% of all central nervous system tumors in children. Onset usually occurs with chronic nonspecific symptoms and may depend on the intra- or extradural neoplastic location. Meningiomas, schwannomas, and neurofibromas are the most common intradural-extramedullary lesions, while astrocytomas and ependymomas represent the majority of intramedullary tumors. The new molecular discoveries regarding pediatric spinal cancer currently contribute to the diagnostic and therapeutic processes. Moreover, some familial genetic syndromes can be associated with the development of spinal tumors. Currently, magnetic resonance imaging (MRI) is the standard reference for the evaluation of pediatric spinal tumors. Our aim in this review was to describe the imaging of the most frequent intradural intra/extramedullary pediatric spinal tumors and to investigate the latest molecular findings and genetic syndromes.
Highlights
Pediatric spinal tumors are relatively rare and account for 10% of all central nervous system tumors in children [1,2,3]
Among these, it is useful to describe the atypical teratoid/rhabdoid tumors and embryonal tumors with multilayered rosettes, which are rarely found in children
Atypical teratoid/rhabdoid tumor (ATRT) is a rare brain tumor that is more common in children under 2 years of age and accounts for 1–2% of all pediatric brain neoplasms [51,52,53] with aggressive development and poor prognosis [53,54]
Summary
Pediatric spinal tumors are relatively rare and account for 10% of all central nervous system tumors in children [1,2,3]. The advancement of molecular findings in pediatric central nervous system tumors (CNS) provides additional information regarding the tumor subtypes and their biological behaviors and patient outcomes These novel findings help to reach a detailed diagnosis, obtaining new therapeutic options by targeted drugs. IInnffiillttrraattiivvee ggrroowwtthh,, nnoonn--ddeemmaarrccaatteedd mmaarrggiinnss,, aanndd ddiisssseemmiinnaattiioonn tthhrroouugghh tthhee cceerree-bbrroossppiinnaall fflluuiidd ((CCSSFF)) aarree mmoorree ttyyppiiccaall iinn hhiigghh--ggrraaddee ttuummoorrss.. OHwoewveevr,ert,hteheaassssoocciaiattioionn bbeettwweeeenn KKIIAAAA11554499--BBRRAAFF ffuussiioonn aanndd oouuttccoommee hhaass nnoott yyeett bbeeeenn vvaalliiddaatteedd,, aass aaccccoorrddiinngg ttoo ssoommee ssttuuddiieess,, tthhiiss mmoolleeccuullaarr aalltteerraattiioonn ccoouulldd nnoott pprreeddiicctt tthhee pprrooggnnoossiiss [[1188]]. MMeeningiomas, rare in cchhiildren, mmaake uupp oonnllyy 33%% ooffppeeddiiaattrriicc SSNNCC ttuummoorrss [[4455]]aanndd aarree cclloosseellyy rreellaatteedd ttoo tthhee ddiiaaggnnoossiiss ooff NNFF22;; iitt iiss eessttiimmaatteedd tthhaatt 2200%% ooff NNFF22 ppaattiieennttss hhaarrbboorr ssppiinal meningiomaass. BBeeyyoonndd tthhee NNFF22,, tthheeSSMMAARRCCEE11(S(SWWI/I/SSNNFF--rreellaatteedd,, mmaattrriixx--aassssoocciiaatteedd,, aaccttiinn--ddeeppeennddeenntt rreegguullaattoorr ooff cchhrroommaattiinn,,susubbfafammiliylyE,Em, emmebmebre1r) g1e)ngeemneutmatuiotantiwoanswfoausnfdoiunnydouinngyoaduunlgtsa(d16u–lt2s4 (y1e6a–r2s4) wyeitahrsm) wenitinhgmioemnian.gHioemtear.oHzyegteoruoszlyogsos-uosf-lfousns-cotfio-fnunmcutitoantiomnustiantiothnes SinWtIh/eSSNWFIc/hSNroFmcahtrino-rmematoind-erleinmgocdoemlinpglecxosmupbulenxitsugbenueniStMgeAnReCSEM1ApRlaCyEa1kpelyayroalekeiny trhoelepianththoegpenaethsiosgoefnsepsins aolf mspeinnianlgmioemnainsgwioitmh acslewaritchellclheiasrtocloelglyh[i5s1to].loFguyrth[5e1r]m. oFruer,tihnerymouonreg,pineoypoleu(n1g–1p5eyoepalres)(,1t–h1e5 germline LZTR1-mutation is associated with the development of schwannomas, as well as that of SMARCB1, which, in the older age group, accounts for 25% of schwannomatosis disease [42]
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