CASE REPORTS   

Rivista Italiana di Chirurgia Maxillo-Facciale 2001 August;12(2):103-6

Copyright © 2002 EDIZIONI MINERVA MEDICA

language: English

Desmoplastic fibroma of the mandible. Report of a case

Maremonti P., Califano L. *, Mangone G. M. *, Friscia M. *, Longo F. *, Insabato L. **

From the Operative Unit Maxillofacial Surgery, Bellaria Hospital of Bologna, Italy * Maxillofacial Surgery Department and ** Department of Pathology, ”Federico II“ University of Naples, Italy

Desmoid ­tumour of ­the ­soft tis­sues ­first report­ed by MacFarlane in 1932, ­occurs ­most fre­quent­ly in ­the abdom­i­nal cav­ity, ­but ­could ­also be locat­ed in ­the extrem­ities. Desmoplastic fibro­ma is ­one of ­the rar­est of ­bone ­tumours, it ­can be con­sid­ered as ­the intra­os­seous coun­ter­part of ­the ­much ­more com­mon ­desmoid ­tumour of ­soft tis­sue, ­with ­only ­about 150 report­ed cas­es. The des­mo­plas­tic fibro­ma ­has ­been report­ed in dif­fer­ent ­bones, ­the man­dible is ­the ­most com­mon ­site of dis­ease, fol­lowed by ­the met­a­phy­sis of ­the ­femur, tib­ia or humer­us. The aetio­lo­gy is ­unknown, ­although trau­ma, endo­crine ­and genet­ic fac­tors ­have ­been sug­gest­ed. The ­jaws ­are affect­ed ­more fre­quent­ly ­between 10 to 45 ­years of ­age ­with a ­mean of 17.2 ­years ­and a ­male to ­female ­ratio of 1.5 to 1. Symptoms ­are usu­al­ly relat­ed to ­the expan­sion of ­the ­tumour; ­pain ten­der­ness or par­a­es­the­sia ­are uncom­mon clin­i­cal find­ings. Pathological frac­tures ­have ­been report­ed in asso­ci­a­tion ­with ­lesions of ­the ­long ­bones. Radiographically ­the ­lesions ­appear as ­well delin­eat­ed radio­lu­cen­cies ­with uni­loc­u­lar or mul­ti­loc­u­lar ­aspect. The radio­graph­ic dif­fe­ren­tial diag­no­sis ­often ­includes amel­o­blas­to­ma, odon­tog­e­nous myx­o­ma, cen­tral hae­man­gio­ma, aneu­rys­mal ­bone ­cyst, chon­dro­myx­oid fibro­ma ­and eosin­o­phil­ic gra­nu­lo­ma. Macroscopically, des­mo­plas­tic ­tumours ­appear as a ­firm, rub­bery, ­white ­non-encap­su­lat­ed ­mass. Local recur­rence is obvi­ous­ly strong­ly cor­re­lat­ed ­with pos­i­tive sur­gi­cal mar­gins. The ­authors ­report a ­case of a 45-­year ­old ­white ­male ­with a pain­less ­lesion of ­the ­left man­dib­u­lar ­angle. Resection of ­the ­left man­dib­u­lar ­angle ­through a cer­vi­cal ­approach ­was per­formed ­under gen­er­al anaesthe­sia with­out recon­struc­tion. The ­patient ­has ­been fol­lowed-up ­for 12 ­months ­with no evi­dence of recur­rence. We ­are cur­rent­ly plan­ning to per­form sec­on­dary recon­struc­tion.