2024/12/31 by Boutrif, M., Kebaili R, Khalifa MA +2
#Tumeur-Nerf-Neurolipofibrome-Dia- gnostic-Traitement-Enfant #Tumor-Nerve-Fibrolipomatous hamar- toma-Diagnosis-Treatment-child
paper · doi:10.71566/pist-rmp-194763
"Introduction : Fibrolipohamartoma (Fibrolipoma- tous hamartoma) is a benign, rare, childhood and young adult tumor of unknown etiology, charac- terized by a proliferation of fatty and fibrous ele- ments surrounding the nerve, infiltrating the epi- neuriumandtheperineuriumandwhichfitsintothe framework of neural lipomatosis. Its elective loca- tion is the distal part ofthe median nerve. The as- sociation ofFibrolipohamartomawith Macrodactyly achieves Lipomatous Macro-dystrophy. Throughtwoobservationswewillseethespecificitiesofthis benignnervetumoraswellontheclinical,diagnostic and therapeutic level. Observation1:Wereportthecaseofa4-year-old patient,whoconsultsforanasymptomaticswelling ofthepalmofthehandandtheanterioraspectof therightwrist,ofasoft,painlessconsistencyevol- ving from a very young age. Magnetic resonance imagingshowedthatthismasshadafat-likesignal and was not enhanced by the contrast medium. Thisswellingpassedthroughthecarpaltunnelto thethenarregion,producinga""cable""appearance ofthenervefibersinaxialsectionanda""bowtie""appearanceinsagittalsections. Wedecidedtoap- proachthistumortheannularligamentwasopened showingamacroscopicappearanceofahugehour- glass swelling of the median nerve with the pre- senceofaproximalstrictionofthelatter. Asimple neurolysisofthetumorconsistingofanopeningof theepineuriumandasuperficialdegreasingwithout interfasciculardissectionaswellasabiopsysample wascarriedoutunderanoperatingmicroscopewi- thouttouchingthenervefibers. Theanatomopa- thological study revealed benign hamartomata’s infiltrationofthenerveenvelopes,fibro-lipomatous innature,dissociatingthefascicleswithoutinvading thenervefiberitself,withoutsignsofmalignancies. Observation2:Thisisa17-year-oldpatientwith no particularpathological historywho presentswith amajorhypertrophyoftherighthandfallingwithin theframeworkofmacrodactylyprogressinggradual- lyfrombirthandbecomingmoreandmorepainful. Ultrasoundfoundaspindle-shapedenlargementof the median nervewith fattyproliferation dissociating thenervefascicles. TheMRIobjectifiedtheincrease involumeofthevariousconstituentsofthehandin- cludingthemediannervewhichpresenteditselfin hypointenseinT1andT2withfasciclesdissociated byafattyinfiltratewith asinuous aspect and proxi- malextensionofthefattyinfiltration. Therapeutically, the patient had benefited from li- berationoftherightmediannerveatthelevelofthe carpaltunnelassociatedwithneurolysisoftheinter- digitalnerveofthesecondspaceandremodelingof the soft tissues. Conclusion : Fibro-lipomatous hamartoma of the median nerve is a rare, benign tumor, dissociating the fascicles without invading them, of congenital origin, of very slow evolution and difficult clinical diagnosis. In 1/3 of cases this tumor can be asso- ciated with macrodactyly of one or more fingers, anditiscalledmacrodactylylipomatousdystrophy. Nuclear magnetic resonance imaging can make the diagnosis and avoid a surgical biopsy for diagnostic purposes. The treatment of Fibro-lipomatous ha- martomaisverycontroversial, it must be earlyand must remain conservative with decompression of the nerve and possible tumor reduction by careful intra-neuro-dissection. Because oftheir infiltrating nature,nerveresectioniscontraindicated. Theseare, in fact, inextricable tumors. "