Malignant Fibrous Histiocytoma of the Brain in the Pediatric Patient: A Case Report and Literature Review

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1 Mlignnt Firous Histioytom of the Brin in the Peditri Ptient: A Cse Report nd Literture Review Histioitom firoso mligno ererl en piente pediátrio: Presentión de un so y revisión de l litertur Key words (MeSH) Brin neoplsms Histioytom mlignnt firous Mgneti resonne imging Plrs lve (DeCS) Neoplsis enefális Histioitom firoso mligno Imgen por resonni mgnéti 1 Rdiology nd dignosti imging resident. Fundión Universitri Snits. Bogotá, Colomi. Drío Leonrdo Blguer Suárez 1 Ctlin Wilhes Vnegs 2 Crolin Mntill Grosso 3 Germán Arturo Beltrán 3 Ctherine Alvrdo Heine 3 Summry Brin tumours re the seond use of mlignnt neoplsms in hildren while primry sroms in the entrl nervous system (SNC), re extremely rre. For this reson, we present the results of the study of se of girl nd revision on the pthophysiology, linil mnifesttions, nd findings on rin MRI of this pthology. We report the se of sevenyer-old ptient dmitted to the emergeny deprtment with hedhe nd symptoms suggestive of intrrnil hypertension, nd deresed strength of the right side of the ody. In rin MRI, we found left temporoprietl extr-xil mss, displing the midline. The ptient ws exmined y the neurosurgeon who performed left frontoprietl rniotomy, with omplete tumor resetion. The pthology study showed high-grde spindle ell srom, lssified s mlignnt firous histioytom. Resumen Los tumores ererles son l segund us de neoplsis mligns en niños y los sroms primrios en el sistem nervioso entrl son extremdmente rros. Por est rzón, presentmos los resultdos del estudio de un so en un menor de edd y l revisión relizd sore l fisioptologí, mnifestiones línis y hllzgos en ls imágenes de resonni mgnéti ererl de est ptologí. Se trt de un niñ de 7 ños de edd que ingres l serviio de urgenis on efle, sintomtologí sugerente de hipertensión endornen y disminuión de l fuerz del hemiuerpo dereho, por lo que se le prti resonni mgnéti ererl (RM) on medio de ontrste, on hllzgo de ms extrxil temporoprietl izquierd, que desplz l líne medi. L piente es vlord por neuroirugí y remitid rneotomí frontoprietl izquierd, on reseión omplet del tumor. El informe de ptologí muestr srom fusoelulr de lto grdo, omptile on histioitom firoso mligno. 2 Rdiologist, Clíni Rein Sofí. Rdiologist professor, Fundión Universitri Snits. Bogotá, Colomi. 3 Pthologist, Clíni Universitri Colomi. Orgniztion Snits Interntionl. Bogotá, Colomi. Introdution Brin tumors ount for etween 16% nd 23% of ll tumors in the peditri ge (1) nd re the seond use of hildhood ner, with homogeneous distriution from irth to 14 yers of ge (2). Sroms represent pproximtely 6% of ll ners in this ge group (1); however, primry rin sroms re extremely rre neoplsms, with n estimted inidene of 0.1-4% of ll intrrnil neoplsms (1). The term mlignnt firous histio- 4678

2 ytom (MFH) ws first oined in 1963 y Ozzello. These tumors ompromise usully the lims nd the retroperitoneum, nd intrrnil presenttion is very rre, with very few ses desried in the literture (3). Mgneti resonne imging (MRI) is the study of hoie for the dignosis of intrrnil tumors, surgil plnning nd post-tretment ontrol (4, 5). Histopthologilly there re more thn fifty soft tissue sroms sutypes, ording to the lssifition of the World Helth Orgniztion (WHO) (6), for whih it is essentil n dequte tretment to lerly identify the type of lesion, through the use of iomoleulr tehniques nd the identifition of the tumor genome (7). Clinil history Seven-yer-old girl who entered the emergeny deprtment due to multiple episodes of emesis with week of evolution, frontl hedhe nd loss of strength of the right hemife, ipsilterl epiphor, previously medited with ntiemetis nd nlgesis without improvement. Physil exmintion reveled devition of the left orner of the lips, deresed strength of the right hemife nd limittion for ipsilterl plperl olusion. We lso found derese in strength in the right hemiody 4/5, whih limited stnding nd wndering. Negtive Romerg sign. MRI of the rin with ontrst medium showed left temporoprietl extr xil mss nd suphline herni tht displed the midline to the right (figures 1, 2, 3 nd 4). By endornil hypertension lini nd Neurosurgery deision, left frontoprietl rniotomy ws performed with omplete resetion of the lesion, whih resulted in prtil improvement of the symptomtology in the immedite postopertive period. The pthology report revels mlignnt neoplsm of fusoellulr pttern with elongted hyperhromti nulei, some rounded, multiloulted, with frequent mitoti figures (up to 50 per 10 CAP) nd foi of nerosis (figure 5). Adjent gry mtter rejeted y the lesion is srse nd dequte irumsription etween the tumor nd the djent prenhym is oserved (figure 5). Cells re rrnged in undles nd some foi show ells with lrge pleomorphi eosinophil ytoplsm (figure 6). Positivity of tumor ells to vimentin (figure 7), folly to desmin in pleomorphi ells (figure 7) nd negtivity for ute myeloid leukemi (AML), epithelil memrne ntigen (EMA), myogenin nd glil firillry idi protein (GFAP). The ell prolifertion index (Ki 67) is of 90% (figure 7). The INI1 is preserved. This immunohistohemil profile together with the rhiteture of the lesion in whih mrked ellulr typi is oserved, with nerosis nd high mitoti ount llows to dignose high-grde fusoellulr neoplsi with pleomorphi srom type omptile with mlignnt firous histioytom. In the ontrol MRI, postopertive hnges re evidened with tumor residue, whih is why the hemotherpy yle egins with vinristine, phosphmide, ispltin, etoposide, nd rdition therpy. Clinilly, the ptient mnifests omplete reovery of the neurologil stte. In the perfusion ontrol nd MRI spetrosopy t 2, 9, 12 nd 19 months post-tretment there is no tumor reurrene oserved. Rev. Colom. Rdiol. 2017; 28(2): Figure 1. MRI with T1, xil informtion. In the left frontoprietl region the extr-xil mss of defined ontours is oserved, predominntly low signl nd some high signl foi tht orrespond to intrtumorl leeding, displing the djent prenhym, it is ssoited with suphline hernition to the right with olitertion of the frontl horns of the lterl ventriles. Figure 2. ) Axil; ) oronl nd ) xil FLAIR sequene. Mss of heterogeneous signl with multiple ysti foi nd importnt ssoited vsogeni edem. 4679

3 Figure 5. ) Axil sequenes with T1 informtion with ontrst medium, nd ) oronl. Enhnement intensity of the lesion with the ontrst medium. Figure 3. ) Diffusion sequene nd ) ADC Mp. High signl of the diffusion lesion, with low signl of the sme in the ADC mp, whih orresponds to restrition y high ellulrity. Figure 4. Eho grdient sequene (mgneti suseptiility). Ares of low intrtumorl signl, whih orrespond to res of hemorrhge. Figure 6. ) Trnsition etween tumor nd djent norml ererl prenhym (irumsried edge, hollow rrows). ) Frequent mitoti figures (rrows). ) Zoom (40X), ell is oserved with pleomorphi nuleus nd typil (rrow with noth) Mlignnt Firous Histioytom of the Brin in the Peditri Ptient: A Cse Report nd Literture Review. Blguer D., Wilhes C., Mntill C., Beltrán G., Alvrdo C.

4 d Figure 7. ) Vimentin, ) BCL2, ) desmin, d) Ki67. Disussion Soft tissue sroms re mesenhyml tumors, originting from mesoderm ells (5). Its primry origin in the entrl nervous system is extremely rre, with few se studies over the pst 50 yers, ut with vrying histology nd derivtives of the meninges minly, hene it ppers s n extr-xil lesion (8). Desriptions hve een found of MFH s: pleomorphi srom or srom without other speifition (NOS), rhdomyosrom, hondrosrom, myxoid liposrom nd mlignnt mesenhyml tumor (1). Suprtentoril tumors re more ommon neontes nd infnts, while in hildren of more thn 2 yers of ge infrtentoril involvement is more ommon (5). Aording to the histologil type, the tumor of this ptient is lssified s pleomorphi srom. Initil desriptions of this tumor were mde in the 1960s nd 1970s: lesions tht ompromised the Turkish sddle nd the temporl one with intrrnil extension s result of rdition therpy in pituitry tumors (9, 10). This type of tumor hs two peks of inidene, elow 10 nd t 40 yers of ge, with predominne in men (11). Some predisposing ftors hve een proposed for its development, suh s trum, rdiotherpy or intrrnil surgery for other uses (11). Computed xil tomogrphy (CT) my show n extr-xil lesion rejeting the djent ererl prenhym, with heterogeneous density, defined ontours, with some res of high signl due to intrtumorl hemorrhge nd low ysti res nd nerosis, s well s peritumorl vsogeni edem nd enhnement Rev. Colom. Rdiol. 2017; 28(2): fter dministrtion of ontrst medium (12). In MRI, the lesion shows regulr ontours, rod durl se nd low signl in the white mtter in T1, nd intermedite signl to slightly high in T2 with some ysti res nd surrounding vsogeni edem. It reflets high diffusion signl with low ADC vlues, intense ontrst enhnement nd loss of intrtumorl signl in the sequene of suseptiility due to hemorrhge. Due to its mss effet, it my oliterte the ventriulr system nd e ssoited with suphline hernition (8,12). CT-fused positron emission tomogrphy (PET-CT) shows high uptke of fluorodeoxygluose (FDG) solely y mss; however, this finding is not speifi nd my our in other types of entrl nervous system (CNS) mlignnt tumors (12). The tretment is minly imed t the surgil resetion of the tumor with removl of wide mrgins, to void lol reurrene of the tumor, nd rdium nd hemo s djuvnt therpies. Chemotherpy, on its own, hs no definite effetiveness. The prognosis depends on the ffeted re, tumor extension nd ellulr typiity (3,12). In the se studied, solid left frontoprietl mss ws oserved, with ysti res, homogeneous enhnement nd surrounding vsogeni edem, whih y its hrteristis nd histologil study ws omptile with high-grde mlignnt firohistioytom (9). The differentil dignosis should e done with meningiom, gliosrom, Shwnnom, pleomorphi xnthostroitom, mlignnt glioms, lymphom, nd metstsis (3,10,12), hene the importne of the histologil orreltion nd iomrkers for lssifition. 4681

5 Conlusion Brin sroms re rre entity in the generl popultion nd more in the peditri ge, for whih there re few series of ses pulished in this ge group. Mlignnt firous histioytom or primry pleomorphi srom in hildren is even rrer, so there re few desriptions in the literture nd, lthough their long term prognosis is d, in the ptient in this se there hs een no oserved tumor reurrene so fr. Referenes 1. Benesh M, Von Bueren AO, Dntonello T, Von Hoff K, Pietsh T, Leushner I, et l. Primry intrrnil soft tissue srom in hildren nd dolesents: A oopertive nlysis of the Europen CWS nd HIT study groups. J Neuroonol. 2013;111: González J, Grí A, Grizr C. Tumores ererles infntiles: dignóstio y semiologí neurológi [internet]. AEP [itdo 10 ene. 2016]. Disponile en: 3. Ozdemir M, Ozgurl O, Bozkurt M, Torun FM, Oku Heper A, Tun H. Primry intrererl mlignnt firous histioytom mimiking meningiom. Turk Neurosurg. 2012;22: Al-Okili RN, Krejz J, Wng S, Woo JH, Melhem ER. Advned MR imging tehniques in the dignosis of intrxil rin tumors in dults. Rdiogrphis. 2006;26(Suppl 1):S Borj MJ, Plz MJ, Altmn N, Sigl G. Conventionl nd dvned MRI fetures of peditri intrrnil tumors: Suprtentoril tumors. Am J Roentgenol. 2013;200: Lewis VO, Mdewell JE. Onologi Imging: A Multidisiplinry Approh [internet]. Onologi Imging: A Multidisiplinry Approh [itdo 10 ene 2016]. Disponile en: Tshoep K, Kohlmnn A, Shlemmer M, Hferlh T, Issels RD. Gene expression profiling in sroms. Crit Rev Onol Hemtol. 2007;63: Oliveir AM, Sheithuer BW, Slomo DR, Prisi JE, Burger PC, Nsimento AG. Primry sroms of the rin nd spinl ord: study of 18 ses. Am J Surg Pthol. 2002;26: Koşuu P, Yvuz AA, Yvuz MN, Reis A, Ahmetoğlu A. Primry intrrnil mlignnt firous histioytom. Eur J Rdiol Extr. 2003;47: Romero FJ, Orteg A, Irr B, Piquers J, Rovir M. post-rdition rnil mlignnt firous histioytom studied y CT. Comput Med Img Grphis. 1989;13: Gelert-González M, Fernández-Vill JM, Reyes-Sntís R. Histioitom firoso mligno de durmdre. Neuroirugí. 2003;14: Yoo RE, Choi SH, Prk SH, Jung HW, Kim JH, Sohn CH, et l. Primry intrererl mlignnt firous histioytom: CT, MRI, nd PET-CT findings. J Neuroimging. 2013;23: Correspondene Ctlin Wilhes Vnegs Deprtment of Rdiology nd dignosti imging Clini Rein Sofí Bogotá, Colomi wilhes30@hotmil.om Reeived for evlution: Jnury 25, 2016 Aepted for pulition: Novemer 29, Mlignnt Firous Histioytom of the Brin in the Peditri Ptient: A Cse Report nd Literture Review. Blguer D., Wilhes C., Mntill C., Beltrán G., Alvrdo C.