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Ewing's Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases

Ewing's Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases Introdu ction an d purpose . Primary E wing’ s s arcoma a rising fr om t he b one s o f the h ead a nd n e ck r egion i s e xtreme ly r are representing o nly 1 ± 4% of all E wing’ s s arcoma c ases. P revious r eports s ug gest a b etter p rogno sis f or that p articul ar a natomi c site. T he pur pose o f this s tud y w as t o a na lyze t he c linico- epidem i ol ogic c haracte ristics o f that r are clini cal p resenta tion, as well a s i ts p atterns o f failur e and p rognos is fo llowing treatme nt. M a terials an d me thods . This s tud y i nclude d a r etros pective r eview of the m e dical r ecor ds o f patients w ith t he d iagno sis o f Ewing ’ s s arcoma o f the h ead a nd n eck r egion t reated a t K i ng F aisal S peciali st H ospital a nd R e search C enter between 1 975 and 1996. Results. Out o f a t otal n um b er of 24 cases a na lyzed, there were 17 ma les a nd 7 females w ith a r atio o f 2.4:1. The me di an age a t d iagnos is w as 1 6.5 years. A p ainful s welling w as t he m o st c omm o n c linical p resent ation. T he ma xilla w as t he m o st comm o n s ite o f present ation ( 9/2 4 cases). T here were 3/2 4 cases w ho p resente d w ith m e tastati c d isease at d iagnos is. T he ma j or ity o f pa tient s ( 16/2 4 cas es) h ad a t um o r si ze >10 cm. Mo s t p a tient s w ere treated w ith s ys temi c c hemo t herap y p lus loc alized i rradi ation f ollowi ng an i ni tial b i op s y. W ith a m e an f ollow up o f 3.4 years , t h e 5-year ac tua r ial o verall s u r vival (OS) f or the who l e g rou p w as 5 3%, whi le t he 5-year a c tua r ial d i seas e-f ree sur vi val ( DFS ) w as 3 0%. These ® gur es w ere hi gher tha n t ho se repor ted f rom o u r ins titut ion f or you n g patient s ( £ 14 year s t reated f or Ewing ’ s s a rcom a i n o t her an a tom i c l oc ations ( 30% v 1 5%). The respo n s e t o c hemo t herapy w as t he o n l y p r og no st ic f actor that a ffected b ot h t he OS and DFS. Conclusion. The prognos is o f Ewing’ s s arcoma o f the h ead a nd n e ck r egion i s s lightly b etter t han t hat o f other a na tomi c sites.T he respons e t o s ystemi c c hemo therapy i s o ne of the m o st i mp or tant p rognos tic f actor s a ffecting bot h D FS a nd O S o f Ewing ’ s s arcoma o f the h ead a nd n e ck. M u ltimo d ality t herapy c ons isting of an i nitial b iopsy, a ggress ive c om b ina tion chemo therapy a nd l ocalize d r adiot herapy i s t he t reatme nt o f choice fo r Ewing ’ s s arcoma o f the h ead a nd n e ck r egion a nd ma y r esult i n l on g-term s ur vival. Int roduct ion loc ation. Th e d e t ails of trea tme nt i nc l ud i ng s urge r y, ch emo t her apy and r adiation t her apy, as wel l a s the Pr imary Ew ing ’ s sarcoma arising i n t he h ea d and pattern s of failure a re present ed. Also, the v arious ne c k r egi on i s ex t rem e ly r are, co mp rising 1 ± 4% of all potent ial p rogn o stic f actors affecting b oth the d i sease- 1,2 ca ses of Ew ing ’ s sarcoma . Mo s t authors claim a free ( D F S ) a nd t h e o v er all sur viv al (O S ) a re better p rogn o s is for Ew ing’ s sarcoma of the h ead a nd high ligh te d . ne c k r egi on a s co mp ared to t ha t a rising i n o t her 1± 5 anatom i c l oca tion s. Th e m o s t co m m o n l y affected bone s in t he h ead a nd n ec k r egi on a re the s kull, the Ma terials and me tho d s ma ndi ble a nd t he m a xilla. Th ere h as also been c a se reports of loc alized E w ing ’ s sarcoma affecting t he Th e f ollowi ng s tud y inc lude d a retros pect ive a nalysis 3 4 5 of da ta available f rom the m e di ca l rec or ds of 24 c a ses or bital r oof, the r et rop harynx and t he n a sal cavity. Th e a im of the p resent s tud y was to r ev iew the c ases of prima ry Ew ing’ s sarcoma arising i n t he h ead a nd of Ew ing ’ s sarcoma of the h ea d and n e c k r egi on ne c k r eg i on, treated a t t he K ing F a isal Spec i alist treated a t o u r i ns titut ion d u ri ng t he p ast 20 y ears, Hos pital and R esea rch Cen ter d ur ing t he p er iod 1 97 5 aimi ng a t a bet t er un d e r standi ng o f the c l ini co - to 1 9 9 6 . Th e K ing F aisal Speci alist Hos pital and ep idem i ol ogi c c h aracter istics of tha t r are anatom i c Research Cent er i s a tert iary care c en t er s erving t he Co rrespon de nce t o: Dr H enri k S chultz, De part ment o f R adiation O n cology, M B C 3 4, Ki ng F aisal S pecialis t Hospital & R C, PO B ox 3354, Riyadh 1 1211, Ki ngdom o f S audi A rabia. F ax: 9661-442 4566. *This paper h as b een a ccepted a s a n o ral p resentat ion i n t he 17th Annual E STRO meeting, Edinbur gh, 20± 24 September 1 998. 1357-714X/ 9 9/010011-05 $9.00 ½ 1999 Taylor & F rancis L td 12 A. Allam et al. Table 1. Char ac teri stics o f eval uab le p atient s whole k ing d o m of Saudi A rabia. It i s the l arge st onc o logy refer ral ce nt er at p res en t r ece iving p atien t s Patients’ characte ristics N umb er of cases ( %) from all over the k ing do m . Th e a nalysis incl ud e d r eview ing v ariou s progno s tic 1. Sex Male 17/24 (71%) factors that c o ul d a ffect t he t reatme nt o ut com e i n Female 7/24 (29%) term s of DF S as wel l a s OS .Th e c h arts wer e r ev iew ed 2. Symptoms for age , s ex, presenting s ymptoms and s ign s and d ur a- Swelling 23/24 (96%) ti on o f sympto m s. Th e p rim a ry tu m o r site w as Pain a nd T ende rness 21/24 (87.5%) de ter mined f or each patient . T h e t um o r size a s wel l Vi sual p roblems 8/24 (33%) Anemia 8/24 (33%) as the i nitial s tage o f present ation w ere d e t er mi ne d Fever 3/2 4 (12.5%) for all patient s. Stagi ng w ork-up of patient s inc ludi ng 3. Tumo r size com p uteri ze d t om o gr aphy (CT ) s ca n of the h ead 5± 10 cm 8/24 (33%) and n e c k, che st X -ray, CT s can of the c he st, b one >10 cm 16/24 (67%) 4. Initial s tage sca n, bone m a rrow biopsy and s erum lactic d e h ydro- Localized 21/24 (87.5%) ge na se (LD H ) l ev els were r eview ed . Metastatic 3/2 4 (12.5%) T h e d i f fer en t t r ea tm en t m od a li t i e s ado p te d 5. Tumo r site inc l ud i ng s urge r y, radiot he r apy and c h em ot he r apy Maxilla 9/2 4 (37.5%) wer e r ev iew ed i n d e ta il.Th e r adiation t he r apy ch arts Mandible 6/24 (25%) Orbit 4/24 (17%) wer e r ev iew ed f or the t ot a l do s e gi ven , th e n u m b er Skull 3/2 4 (12.5%) of fract ion s , th e o v er all tr ea tm e n t t ime , th e e n e r gy Nasal C avity 2/24 (8%) us ed a nd t h e u s e of CT p laning . Chem ot he r apy de t a ils inc l ud e d t imi ng o f system ic c h em ot he r apy, cases ); while t he r em a ining h ad ei ther inco mp lete age n t s us ed, nu m b er of cy cl es a s well a s the r espon s e exc ision ( 6/ 2 4 c a ses) or com p lete s urgi cal e xc ision to c h em ot he r apy trea tme n t . (2/ 2 4 c ases ). Pa tien t s wer e f ollowe d u p reg ul arly ever y 3 m o n ths Th e m o st c o mm o n t rea tme n t m o da lity was ini tial for the ® rst yea r, ever y 6 m o nt hs in t he s ubseq uent 2 biopsy followed b y co mb ine d s ystemi c c hem o the rapy yea rs and t hen y early ther eafter . Patien ts w ho wer e plus radioth er apy to t he p rimary site i nvolved . Th is los t to fo llow- up were c e ns ored f rom the s urvival mo da lity was adop ted i n 1 4 / 2 4 c a ses (58 %). T h er e analysis at t he t ime o f last follow-up. No ne o f tho se wer e v ariou s ot her t reatme nt m o d alities , the m o st patient s partici pated i n c l ini ca l trials. com m o n o f which was surgi cal e x ci sion f ollowed b y Th e p attern s of trea tme nt f ailure, whet her loca l pos t-oper ative c h em o- radi ot he r apy in 5 / 2 4 c a ses recu rr en c e ( LR ) o r di stant m e tastas is (DM ) , were (21%). studi ed i n d etail. D a tes o f loca l recur rence a nd d istant System i c c he m o t her apy was de l ivered i n 2 2 / 2 4 me tastas is, as wel l a s the i ni tial s ite o f DM w er e cases (92 % ). T wo p atient s refused s ystemic c he mo - de t e r m ine d . Bo t h act u a rial O S and D F S wer e the r apy tr ea tm e n t , 1 w as tr ea ted w ith co m plet e analyzed u s ing t he K aplan± Me ier me t ho d. surgi ca l exc i sion followed b y post-oper ative radio- the rapy while t he o the r w as trea ted w ith i nco mp lete exc ision a nd r efused p ost-operative i rradiation. Th e Results mo st c om m o n r eg i me n g i ven w as VAC (vinc r istine , Ou t o f 24 e v aluable patient s, the re w er e 1 7 m a les adriamycin, cy clophosphamide ; 1 0 p atien ts) o r VAC and 7 f emales with a ma le t o f ema le r atio of 2. 4: 1. alter na ti ng w ith IEP ( i fosfamide , et o p os ide a nd Th e m e d i an age o f the p atien t s was 16 . 5 y ea rs (rang e cisplatinu m ) g i ven i n 7 p atient s and VA IA (vinc ri s- 2± 33 y ears). A painful swelling w as the m o s t co mm o n tine , a driamyci n, ifosfamide a nd a ctino m y cin D ) i n 5 present ation f ound i n m o r e t ha n 90 % of patien t s as pati en t s . Th e c h oi ce o f ch em ot h er apy reg i m en shown i n T able 1. Th e m e a n du ration o f symptom s dep en d ed o n t he p rotoco l a dopted d u r ing a gi ven was 5. 5 m o n t hs (rang e 2 ± 12 m o n t hs ). period o f time , i.e. patient s treated f rom 19 7 5 t o Th e m a xilla w as the m o s t co mm o n s ite o f present a- 19 8 0 r ec ei ved V AC alone, while t hos e trea ted f rom tion ( 9/ 2 4 c ases ). Me tastas is at i ni tial p resent ation 19 8 0 t o 1 9 9 0 r ec ei ved V AC/I EP. A ll p atien t s treated was foun d i n o nl y 3/ 2 4 c ases. Mo s t of ou r patien t s from 19 9 0 o n w ards rece ived V AIA. Th er e w er e o n l y had tum o r size > 10 c m in d i ameter (16 / 2 4 c ases). 13 / 2 2 p atien ts ( 59 % ) w ho c o mp leted s ix o r mo r e A bone m a rrow co re b iopsy from the i liac c r es t was cy cles of systemi c c he mo t he rapy. Comp lete r em i s- per forme d i n 1 9 p atien t s; and w as foun d t o b e sion w as ob taine d i n 5 7 % of patient s, while p artial in® ltrated b y ma lign a nt c el ls in 1 p atient o n l y. Th e rem i ssion o cc ur red i n 4 3 % of patien t s. No ne o f the LD H was assayed in 1 9 p atien ts a nd w as el ev ated i n patient s had di sease progr es sion o n c he mo t herapy. 12 / 1 9 ( 63 % ). A bone s ca n was do ne i n 2 2 /2 4 p atien t s Radiot he rapy was de l iver ed i n 2 0 /2 4 c ases (83 % ), (92 % ). I t s howed l oca lized i nc reased u p take o p posite with a radica l int en t i n 1 9 / 2 0 p atient s (95 % ). T h e the i ni tial s ite o f bony involvem e n t i n a ll 22 p atient s tot al r adiation d o se r ange d f rom 30 0 0 t o 5 6 0 0 c Gy with n o e v idenc e o f me t astatic s pread. with a me di an dos e of 50 4 0 c G y. Th er e w ere 3 / 1 9 Th e m a jority of patient s had biop sy onl y (16 / 2 4 patients w ho received a total r adiation d ose £ 4000 cGy. Ewing’ s sar coma o f t he he ad an d neck 13 Th e t otal d o se o f radiation g i ven d epend ed o n t he for Ew ing ’ s sarcoma , a di sease affecting p rimarily treating p hysician. It d id c hange t o a mo re r adical dose patient s <20 y ears of age . (5400 c Gy ) over time . T h e m a jority of the p atients Th e m a xi lla was the m o s t comm o n s ite o f present a- (13/2 0 ) w ere t reated w ith 6 M V e nergy p hotons. Six tion i n t his study (9/ 2 4 c ases). Although t his represen t s patients w ere t reated w ith C obalt 60 a nd t he r ema ining a rare site o f primary present ation , yet t her e h as bee n patient w ith 8 M V e nergy p hotons. CT p laning w as a tot al o f 22 c ases of prima ry Ew ing ’ s sarcoma of the 8,9 use d i n 1 0 /2 0 c ases (50%) . ma xi lla repor ted i n t he l iteratu r e. Th ere w ere 7 /2 4 c ases who de vel op ed L R (29 % ), In t he p res en t s eries , mos t pati en t s wer e t re a ted and 1 1/ 2 4 c ases (46 % ) w ho develop ed D M . Th e l ung s w it h in i t i a l b io p sy fol l o w ed b y syste m ic were t he m o s t com m o n s ingle s ite o f DM ( 27 % ); ch em ot he r apy plus radi ca l r adiot he r apy.Th is is du e the re w er e 4 / 1 1 p atien t s (36 % ) w ho had mo r e t ha n to t h e f act t h at m o s t of ou r patien t s have large on e s ite o f DM . Fi ve o ut o f 24 p atient s de velop ed les ions (>10 c m ) o f the h ea d and n e ck , wher e r adical both L R and D M . surge r y wou l d b e m ut i lati ng . Radi ca l s urge r y ca n, With a me an fo llow- up time o f 3. 4 y ears, the 5 - year how ev er , play an imp or tan t r ole i n a ch iev ing l oc a l act uari al OS for the w hole g r oup of patient s was co n t rol i n c e r tai n a rea s (e . g. ma nd i ble a nd s kul l), 53 % ; while t h e a ct u a ria l 5 - year DF S was 30 % es pec i ally in t um o r s <5 c m in d i amet er wher e t h e (Fi g. 1a and b ). Th ere w as no s tatisti ca lly sign i® c a nt tr ea tm en t r el a te d m or bid i t y wou l d n o t b e 5,10,11 di fferen c e i n 5 - year OS and D F S betwe en m a les and increased. fema les (54 % vs 50 % and 2 6% vs 36 % , res pectively) . Th e l oca l co nt rol r ate i n t he p res en t s tud y was Tum o r size d i d n o t s ee m t o h ave any imp act o n O S 71 %.Th is ® gur e i s lower t han the 9 0 % ® gu re r eported and D F S .Th e a ct ua rial 5- year DF S for patien t s with in o t h er ser ie s . Th er e a re t w o m ain f act o r s tum o r size o f 5± 10 c m was 30 % com p ared to 2 9 % affecting t he l oca l co nt rol r ate, the se a re the i ni tial for thos e with t um o rs >10 c m in d iameter ( p = 0.93). tum o r size a nd t h e t ot al r adiation d o s e d e l iver ed . Ana lysis of the s urvival res ults (both D F S and O S ) Th e m ed i a n d o s e i n t h is stu d y (5 0 4 0 c G y) i s acc or di ng t o t he p rimary tum o r site d id n o t s how any co ns idered a deq ua te t o a chiev e l oc al c o n t rol b y mo s t 12,13 sign i ® c ant i mp act o f tha t f actor on t he t reatme n t inv es tiga to r s. Of th e 3 p ati en t s who r ec e i ved a ou t com e . T h e m a ndible h ad the h igh est 5 - year OS tot al r adi ati on d o s e £ 40 0 0 c G y, on l y 1 d e v el op ed (80 % ), f ollowe d b y the m a xi lla, skull a nd o r bit ( 71 % , loc a l r ec u r ren c e . Th e l ow er loc a l co n t rol r ate ® gu r e 50 % and 3 8 % , respect ivel y). Th is di fferen c e w as no t ob tai ne d i n t h e p res ent s tud y seem s to b e a ttr ibuted statisti cally sign i ® c ant . to t h e l arge t um o r size ( >10 c m ) i n t h e m ajority o f Th e r espons e to c he mo t herapy treatme n t w as the ou r patien t s (1 6 / 2 4 c a ses). M o s t of ou r pati en t s on l y progno s tic f actor which affected b oth t he O S pres ent r el ativel y late i n t h e c o u r se of the i r di sea se and D F S . Th e 5 - year OS for patien t s with c o mp lete du e t o l on g d i stance s , cu l tur al r ea sons and l ack of remi ssion (CR ) w as 68 % comp ared to 0 % for patient s ef ficien t p rima ry ca re h os pital s in r em ot e a rea s of with p artial r emi ssion (PR ) t o c hem o the rapy ( p = the k ing d o m . 0. 01 5 ) ; a lso the 5 - year DF S for CR patien t s was Th e 5 - year actua rial DF S as wel l a s OS in t he sign i ® c ant ly h ighe r t han that o f PR patient s (27 % vs present s tud y (30 % and 5 3% , respect ively) a re lower 0%; p = 0.02). than the ® gur es reported b y IESS (80 % survival at 3 yea rs). Aga in we a ttri bute t ho se l ower ® gur es ma inly to t w o f act o r s: th e l a rge r tu m or siz e a t i ni t i a l Discussion pres ent at ion a s wel l a s the l ow c o m p lianc e r ate t o ch em ot he r apy tre a tme n t , with o n l y 59 % of patien t s Ew ing ’ s sarcoma s arising f rom the b one s of the h ea d co m plet ing m or e t ha n six c y cl es . and n e ck regi on a re ex treme l y rare. Mo s t ca ses in t he De spite o ur lowe r s urvival rates , yet t hos e are still literatu r e a re rep orted s poradica lly as case r ep orts. high er tha n the 1 5 % 5- year rel apse free s urvival Th e l arge st r eported s eries u n t il n o w i s that o f the repor ted f or a gr oup of young p atient s ( £ 14 years) Int ergr oup Ew ing’ s Sarcoma Stud y (IES S) i n 1 9 8 7 . with the d i agn o s is of Ew ing ’ s sarco m a in o t he r Siegal et al. rep orted i n t hat s eries o n a total o f 29 anatom i c l oca tion s trea ted i n o ur ins titut e b etwee n ca ses of Ew ing ’ s sarcoma of the h ea d and n e c k r eg i on 19 8 0 a nd 1 9 9 3 ( persona l com m u n icatio n) . S imi larly, which repres en t ed 4 % of all Ew ing ’ s sarcoma cases the I ESS reported a highe r m e d ian s urvival for head reported b y the I ESS gr oup . In t he p resent s eries , and n e ck loc ation i n c om p arison t o E w ing ’ s sarcoma Ew ing ’ s sarcoma of head a nd n e ck co ns titut ed 9 % of in o ther anatom i c s ites . all Ew ing ’ s ca ses trea ted i n t his ins titut e ( 2 4 / 2 5 9 Th e o nl y progn os tic f actor sign i® c a ntly a ffecting ca ses). Th is rel atively high ® gur e m i gh t b e a ttribu ted to t he f act that o u r hospital is the m a in ref erral both D F S and O S in t his stud y was the r es pons e to on c ol og y c e nt er in t he r eg i on . Th e m a le t o f ema le ch emo t her apy. Th is ® nd i ng i s in a gr ee me n t w ith t he ratio was 2.4: 1, this is in a ccor da nce w ith t he l iteratur e rece nt a rticles t hat e mp hasize t he i mp ortanc e o f the being a di sease wher e m ales are mo re f reque nt ly ini tial r es pons e to c he mo t herapy treatme nt a s the affected. Th e m e di an age a t d i agn o sis for thi s stud y on ly signi ® c a nt i nd e pen d e nt p redi ctor o f survival for was 16 . 5 y ears, which rep resent s an exp ec ted ® gu re Ew ing ’ s sarcoma patient s. It s houl d b e n o t ed , 14 A. Allam et al. Fig. 1. (a) O v eral l sur vival for t he whole group. Fig. 1. (b) D isease-free sur vival for t he whole group. however , that t he p ower o f subanalysis in t his study altern a ting c he mo t he rapy com b ination r egi me n h as was low d u e t o t he s mall nu m b er of patien t s. already yiel de d a statisti ca lly sign i ® c ant i mp roveme nt In t he e arly phase of the p res en t s tud y, patien t s in D F S when c om p ared t o t he s tandard VACA alone wer e t r ea te d w it h th e u s ua l V AC co m bin a ti o n in a phase III rando m i zed s tudy . In c on c lus ion, reg i me n s , howe v er , starting i n 1 9 9 5 , with th e Ew ing ’ s sarcoma of the h ea d and n e ck reg i on c a rries int rod u c tion o f mo re a gg r es sive che mo t herapy, we a slightly b et ter progn o sis than that i n o ther sites. are no w us ing V ACA alter na ting w ith I /E (ifosfa- Mu ltimo da lity the rapy co ns isting o f an ini tial b iopsy, mi de , e t opos ide) f or a total o f 52 w eek s. Th is ne w aggr essive com b ination c he mo t he rapy and l oc alized Ewing’ s sar coma o f t he he ad an d neck 15 8 Posnick J C, Loui e G , Zuk er R, Weitzma n S . E wing ’ s radiothe rapy is the u s ual trea tme n t o f cho ice a nd sarcoma : p rima ry i nvolveme nt o f the z ygoma u nde r- co uld r esul t i n l ong -term d isea se-free survival. Radical going r esection a nd i mm e di ate r econs tructio n. Plast surge r y ma y be p erforme d f or loc al c on t rol i n c er tain Re constr Surg 1992; 89:956± 61. anatomi c l ocation s (e.g. ma ndib ular and s kull les ions), 9 Fi orillo A , Tranfa F, C ana le G , et al. Primary E wing’ s howev er, thi s ne ed s to b e f ollowe d b y an adeq uate sarcoma o f the m a xilla, a r are and c urable l oc alizatio n: recon struc tive s urge ry .Th e r esponse to c h emo therap y repor t o f tw o n e w cas e s, su c cess f ul l y t r eate d b y radi otherapy a nd s ystemi c c hemo t herapy. Ca ncer Le tters tr ea tm en t r em ain s on e o f th e m os t i m por ta nt 1996; 103:177± 82. prog n o s tic f act o r s affect i ng t h e D F S as wel l a s the 10 Zenk e K , Hatake yam a T , Has himo t o H , Sa kak i S , OS of patient s with E w ing’ s sarcoma of the h ea d and Ma na be K . Primary E wing’ s s arcoma o f the o cciptal ne c k r egi on. bon eÐ C ase r epor t. Neurol M e d C h ir 1994; 34:246± 50. 11 Mi shra H B, Haran R P , Joseph T , Chand i S M. Primary Ewing ’ s s arcoma o f the s ku ll. A r eport o f two c ases. B r References J Neurosurg 1993; 7:683± 6. 12 Ra zek A , Perez CA, Tefft M, et al. Intergr oup E wing ’ s 1 Seigal G P , OliverWR, R e inusW R, et al. Primary E wing’ s Sa rcoma S t udy . L ocal c ont rol r elated t o r adiatio n d o se, sarcoma i nvol ving t he b on es o f the h ead a nd n e ck, volume , and s ite o f prima ry l esion i n E wing’ s s arcoma . Cancer 1987; 60:2829± 40. Cancer 1980; 46:516± 21. 2 Watana be H , Tsubokaw a T , Ka tayama Y , K o yama S , 13 Bu r ne t N G, Bl iss J M, Harme r CL. The imp act o f Nakamu ra S . P rimary E wing ’ s s arcoma o f the t emp oral radi otherap y d o se o n l ocal c on trol o f Ewing ’ s s arcoma bone. Surg Neurol 1992; 37:54± 8. of bone. Sarcoma 1997; 1:31± 8. 3 Al varez A , Schut L , Br uce D. Localize d p rima ry i nt rac- 14 Delepi ne HC, Al ka l laf S, Br un B , Ma rko w ska B , ranial E wing ’ s s arcoma o f the o r bital r oof. Case r epor t. Desbois J C. Is a ge a p r ogno stic f actor in l ocalise d J Neurosurg 1979; 50:811± 3. Ewing ’ s s arcom a t reated b y m u ltidrug regime ns a nd 4 O’ Connell J E, Calder C , Ra afat F, P roops D. Ewing ’ s systema tic s urgery ? Pr oceedings of t he Am e ri can Society sarcoma o f the r etrop harynx . The Journal of L a r y n- of C l inical On cology (ASCO ) , 3 3 rd A n nual M e eting , gology and O t ology 1994; 108:363± 6. abstrac t # 1890, p. 525a, 1997. 5 Had® e ld M G , Luo V Y , W illiams R L , Ward J D, Ru sso 15 Gr ier HE, Kr ailo M , et al. Imp r oved o u t com e in CP. Ewing’ s s arcoma o f the s ku ll i n a n i nf ant . Pediatr Neurosurg 1996; 25:100± 4. no n- me tastati c E wing’ s s arcoma ( EWS) a nd P NET of 6 Falk S , A l pert M . Five y ear s ur vival o f patient s w ith bon e w ith t he a ddi tion o f ifosfami de a nd e toposi de to Ewing ’ s s arcom a . Surg Gy necol Ob s t 1967; 124:319± 24. vinc ristine , adr iamyci n, c ycloph ospham i de and a ctino- 7 Dah l in D C, Coven t ry M B , Sc an l on P W. Ewing ’ s my cin: A C hildr en’ s C ancer Gr oup ( CCG) a nd Pe di- sarcoma : a c ritical a na lysis o f 165 cases. J B one Joint atric O ncolog y G r oup ( POG) R e port. Proc ASCO 1994; Surg 1961; 43A:185± 92. 13:421. 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Ewing's Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases

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Hindawi Publishing Corporation
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Copyright © 1999 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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1357-714X
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1369-1643
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10.1080/13577149977811
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Abstract

Introdu ction an d purpose . Primary E wing’ s s arcoma a rising fr om t he b one s o f the h ead a nd n e ck r egion i s e xtreme ly r are representing o nly 1 ± 4% of all E wing’ s s arcoma c ases. P revious r eports s ug gest a b etter p rogno sis f or that p articul ar a natomi c site. T he pur pose o f this s tud y w as t o a na lyze t he c linico- epidem i ol ogic c haracte ristics o f that r are clini cal p resenta tion, as well a s i ts p atterns o f failur e and p rognos is fo llowing treatme nt. M a terials an d me thods . This s tud y i nclude d a r etros pective r eview of the m e dical r ecor ds o f patients w ith t he d iagno sis o f Ewing ’ s s arcoma o f the h ead a nd n eck r egion t reated a t K i ng F aisal S peciali st H ospital a nd R e search C enter between 1 975 and 1996. Results. Out o f a t otal n um b er of 24 cases a na lyzed, there were 17 ma les a nd 7 females w ith a r atio o f 2.4:1. The me di an age a t d iagnos is w as 1 6.5 years. A p ainful s welling w as t he m o st c omm o n c linical p resent ation. T he ma xilla w as t he m o st comm o n s ite o f present ation ( 9/2 4 cases). T here were 3/2 4 cases w ho p resente d w ith m e tastati c d isease at d iagnos is. T he ma j or ity o f pa tient s ( 16/2 4 cas es) h ad a t um o r si ze >10 cm. Mo s t p a tient s w ere treated w ith s ys temi c c hemo t herap y p lus loc alized i rradi ation f ollowi ng an i ni tial b i op s y. W ith a m e an f ollow up o f 3.4 years , t h e 5-year ac tua r ial o verall s u r vival (OS) f or the who l e g rou p w as 5 3%, whi le t he 5-year a c tua r ial d i seas e-f ree sur vi val ( DFS ) w as 3 0%. These ® gur es w ere hi gher tha n t ho se repor ted f rom o u r ins titut ion f or you n g patient s ( £ 14 year s t reated f or Ewing ’ s s a rcom a i n o t her an a tom i c l oc ations ( 30% v 1 5%). The respo n s e t o c hemo t herapy w as t he o n l y p r og no st ic f actor that a ffected b ot h t he OS and DFS. Conclusion. The prognos is o f Ewing’ s s arcoma o f the h ead a nd n e ck r egion i s s lightly b etter t han t hat o f other a na tomi c sites.T he respons e t o s ystemi c c hemo therapy i s o ne of the m o st i mp or tant p rognos tic f actor s a ffecting bot h D FS a nd O S o f Ewing ’ s s arcoma o f the h ead a nd n e ck. M u ltimo d ality t herapy c ons isting of an i nitial b iopsy, a ggress ive c om b ina tion chemo therapy a nd l ocalize d r adiot herapy i s t he t reatme nt o f choice fo r Ewing ’ s s arcoma o f the h ead a nd n e ck r egion a nd ma y r esult i n l on g-term s ur vival. Int roduct ion loc ation. Th e d e t ails of trea tme nt i nc l ud i ng s urge r y, ch emo t her apy and r adiation t her apy, as wel l a s the Pr imary Ew ing ’ s sarcoma arising i n t he h ea d and pattern s of failure a re present ed. Also, the v arious ne c k r egi on i s ex t rem e ly r are, co mp rising 1 ± 4% of all potent ial p rogn o stic f actors affecting b oth the d i sease- 1,2 ca ses of Ew ing ’ s sarcoma . Mo s t authors claim a free ( D F S ) a nd t h e o v er all sur viv al (O S ) a re better p rogn o s is for Ew ing’ s sarcoma of the h ead a nd high ligh te d . ne c k r egi on a s co mp ared to t ha t a rising i n o t her 1± 5 anatom i c l oca tion s. Th e m o s t co m m o n l y affected bone s in t he h ead a nd n ec k r egi on a re the s kull, the Ma terials and me tho d s ma ndi ble a nd t he m a xilla. Th ere h as also been c a se reports of loc alized E w ing ’ s sarcoma affecting t he Th e f ollowi ng s tud y inc lude d a retros pect ive a nalysis 3 4 5 of da ta available f rom the m e di ca l rec or ds of 24 c a ses or bital r oof, the r et rop harynx and t he n a sal cavity. Th e a im of the p resent s tud y was to r ev iew the c ases of prima ry Ew ing’ s sarcoma arising i n t he h ead a nd of Ew ing ’ s sarcoma of the h ea d and n e c k r egi on ne c k r eg i on, treated a t t he K ing F a isal Spec i alist treated a t o u r i ns titut ion d u ri ng t he p ast 20 y ears, Hos pital and R esea rch Cen ter d ur ing t he p er iod 1 97 5 aimi ng a t a bet t er un d e r standi ng o f the c l ini co - to 1 9 9 6 . Th e K ing F aisal Speci alist Hos pital and ep idem i ol ogi c c h aracter istics of tha t r are anatom i c Research Cent er i s a tert iary care c en t er s erving t he Co rrespon de nce t o: Dr H enri k S chultz, De part ment o f R adiation O n cology, M B C 3 4, Ki ng F aisal S pecialis t Hospital & R C, PO B ox 3354, Riyadh 1 1211, Ki ngdom o f S audi A rabia. F ax: 9661-442 4566. *This paper h as b een a ccepted a s a n o ral p resentat ion i n t he 17th Annual E STRO meeting, Edinbur gh, 20± 24 September 1 998. 1357-714X/ 9 9/010011-05 $9.00 ½ 1999 Taylor & F rancis L td 12 A. Allam et al. Table 1. Char ac teri stics o f eval uab le p atient s whole k ing d o m of Saudi A rabia. It i s the l arge st onc o logy refer ral ce nt er at p res en t r ece iving p atien t s Patients’ characte ristics N umb er of cases ( %) from all over the k ing do m . Th e a nalysis incl ud e d r eview ing v ariou s progno s tic 1. Sex Male 17/24 (71%) factors that c o ul d a ffect t he t reatme nt o ut com e i n Female 7/24 (29%) term s of DF S as wel l a s OS .Th e c h arts wer e r ev iew ed 2. Symptoms for age , s ex, presenting s ymptoms and s ign s and d ur a- Swelling 23/24 (96%) ti on o f sympto m s. Th e p rim a ry tu m o r site w as Pain a nd T ende rness 21/24 (87.5%) de ter mined f or each patient . T h e t um o r size a s wel l Vi sual p roblems 8/24 (33%) Anemia 8/24 (33%) as the i nitial s tage o f present ation w ere d e t er mi ne d Fever 3/2 4 (12.5%) for all patient s. Stagi ng w ork-up of patient s inc ludi ng 3. Tumo r size com p uteri ze d t om o gr aphy (CT ) s ca n of the h ead 5± 10 cm 8/24 (33%) and n e c k, che st X -ray, CT s can of the c he st, b one >10 cm 16/24 (67%) 4. Initial s tage sca n, bone m a rrow biopsy and s erum lactic d e h ydro- Localized 21/24 (87.5%) ge na se (LD H ) l ev els were r eview ed . Metastatic 3/2 4 (12.5%) T h e d i f fer en t t r ea tm en t m od a li t i e s ado p te d 5. Tumo r site inc l ud i ng s urge r y, radiot he r apy and c h em ot he r apy Maxilla 9/2 4 (37.5%) wer e r ev iew ed i n d e ta il.Th e r adiation t he r apy ch arts Mandible 6/24 (25%) Orbit 4/24 (17%) wer e r ev iew ed f or the t ot a l do s e gi ven , th e n u m b er Skull 3/2 4 (12.5%) of fract ion s , th e o v er all tr ea tm e n t t ime , th e e n e r gy Nasal C avity 2/24 (8%) us ed a nd t h e u s e of CT p laning . Chem ot he r apy de t a ils inc l ud e d t imi ng o f system ic c h em ot he r apy, cases ); while t he r em a ining h ad ei ther inco mp lete age n t s us ed, nu m b er of cy cl es a s well a s the r espon s e exc ision ( 6/ 2 4 c a ses) or com p lete s urgi cal e xc ision to c h em ot he r apy trea tme n t . (2/ 2 4 c ases ). Pa tien t s wer e f ollowe d u p reg ul arly ever y 3 m o n ths Th e m o st c o mm o n t rea tme n t m o da lity was ini tial for the ® rst yea r, ever y 6 m o nt hs in t he s ubseq uent 2 biopsy followed b y co mb ine d s ystemi c c hem o the rapy yea rs and t hen y early ther eafter . Patien ts w ho wer e plus radioth er apy to t he p rimary site i nvolved . Th is los t to fo llow- up were c e ns ored f rom the s urvival mo da lity was adop ted i n 1 4 / 2 4 c a ses (58 %). T h er e analysis at t he t ime o f last follow-up. No ne o f tho se wer e v ariou s ot her t reatme nt m o d alities , the m o st patient s partici pated i n c l ini ca l trials. com m o n o f which was surgi cal e x ci sion f ollowed b y Th e p attern s of trea tme nt f ailure, whet her loca l pos t-oper ative c h em o- radi ot he r apy in 5 / 2 4 c a ses recu rr en c e ( LR ) o r di stant m e tastas is (DM ) , were (21%). studi ed i n d etail. D a tes o f loca l recur rence a nd d istant System i c c he m o t her apy was de l ivered i n 2 2 / 2 4 me tastas is, as wel l a s the i ni tial s ite o f DM w er e cases (92 % ). T wo p atient s refused s ystemic c he mo - de t e r m ine d . Bo t h act u a rial O S and D F S wer e the r apy tr ea tm e n t , 1 w as tr ea ted w ith co m plet e analyzed u s ing t he K aplan± Me ier me t ho d. surgi ca l exc i sion followed b y post-oper ative radio- the rapy while t he o the r w as trea ted w ith i nco mp lete exc ision a nd r efused p ost-operative i rradiation. Th e Results mo st c om m o n r eg i me n g i ven w as VAC (vinc r istine , Ou t o f 24 e v aluable patient s, the re w er e 1 7 m a les adriamycin, cy clophosphamide ; 1 0 p atien ts) o r VAC and 7 f emales with a ma le t o f ema le r atio of 2. 4: 1. alter na ti ng w ith IEP ( i fosfamide , et o p os ide a nd Th e m e d i an age o f the p atien t s was 16 . 5 y ea rs (rang e cisplatinu m ) g i ven i n 7 p atient s and VA IA (vinc ri s- 2± 33 y ears). A painful swelling w as the m o s t co mm o n tine , a driamyci n, ifosfamide a nd a ctino m y cin D ) i n 5 present ation f ound i n m o r e t ha n 90 % of patien t s as pati en t s . Th e c h oi ce o f ch em ot h er apy reg i m en shown i n T able 1. Th e m e a n du ration o f symptom s dep en d ed o n t he p rotoco l a dopted d u r ing a gi ven was 5. 5 m o n t hs (rang e 2 ± 12 m o n t hs ). period o f time , i.e. patient s treated f rom 19 7 5 t o Th e m a xilla w as the m o s t co mm o n s ite o f present a- 19 8 0 r ec ei ved V AC alone, while t hos e trea ted f rom tion ( 9/ 2 4 c ases ). Me tastas is at i ni tial p resent ation 19 8 0 t o 1 9 9 0 r ec ei ved V AC/I EP. A ll p atien t s treated was foun d i n o nl y 3/ 2 4 c ases. Mo s t of ou r patien t s from 19 9 0 o n w ards rece ived V AIA. Th er e w er e o n l y had tum o r size > 10 c m in d i ameter (16 / 2 4 c ases). 13 / 2 2 p atien ts ( 59 % ) w ho c o mp leted s ix o r mo r e A bone m a rrow co re b iopsy from the i liac c r es t was cy cles of systemi c c he mo t he rapy. Comp lete r em i s- per forme d i n 1 9 p atien t s; and w as foun d t o b e sion w as ob taine d i n 5 7 % of patient s, while p artial in® ltrated b y ma lign a nt c el ls in 1 p atient o n l y. Th e rem i ssion o cc ur red i n 4 3 % of patien t s. No ne o f the LD H was assayed in 1 9 p atien ts a nd w as el ev ated i n patient s had di sease progr es sion o n c he mo t herapy. 12 / 1 9 ( 63 % ). A bone s ca n was do ne i n 2 2 /2 4 p atien t s Radiot he rapy was de l iver ed i n 2 0 /2 4 c ases (83 % ), (92 % ). I t s howed l oca lized i nc reased u p take o p posite with a radica l int en t i n 1 9 / 2 0 p atient s (95 % ). T h e the i ni tial s ite o f bony involvem e n t i n a ll 22 p atient s tot al r adiation d o se r ange d f rom 30 0 0 t o 5 6 0 0 c Gy with n o e v idenc e o f me t astatic s pread. with a me di an dos e of 50 4 0 c G y. Th er e w ere 3 / 1 9 Th e m a jority of patient s had biop sy onl y (16 / 2 4 patients w ho received a total r adiation d ose £ 4000 cGy. Ewing’ s sar coma o f t he he ad an d neck 13 Th e t otal d o se o f radiation g i ven d epend ed o n t he for Ew ing ’ s sarcoma , a di sease affecting p rimarily treating p hysician. It d id c hange t o a mo re r adical dose patient s <20 y ears of age . (5400 c Gy ) over time . T h e m a jority of the p atients Th e m a xi lla was the m o s t comm o n s ite o f present a- (13/2 0 ) w ere t reated w ith 6 M V e nergy p hotons. Six tion i n t his study (9/ 2 4 c ases). Although t his represen t s patients w ere t reated w ith C obalt 60 a nd t he r ema ining a rare site o f primary present ation , yet t her e h as bee n patient w ith 8 M V e nergy p hotons. CT p laning w as a tot al o f 22 c ases of prima ry Ew ing ’ s sarcoma of the 8,9 use d i n 1 0 /2 0 c ases (50%) . ma xi lla repor ted i n t he l iteratu r e. Th ere w ere 7 /2 4 c ases who de vel op ed L R (29 % ), In t he p res en t s eries , mos t pati en t s wer e t re a ted and 1 1/ 2 4 c ases (46 % ) w ho develop ed D M . Th e l ung s w it h in i t i a l b io p sy fol l o w ed b y syste m ic were t he m o s t com m o n s ingle s ite o f DM ( 27 % ); ch em ot he r apy plus radi ca l r adiot he r apy.Th is is du e the re w er e 4 / 1 1 p atien t s (36 % ) w ho had mo r e t ha n to t h e f act t h at m o s t of ou r patien t s have large on e s ite o f DM . Fi ve o ut o f 24 p atient s de velop ed les ions (>10 c m ) o f the h ea d and n e ck , wher e r adical both L R and D M . surge r y wou l d b e m ut i lati ng . Radi ca l s urge r y ca n, With a me an fo llow- up time o f 3. 4 y ears, the 5 - year how ev er , play an imp or tan t r ole i n a ch iev ing l oc a l act uari al OS for the w hole g r oup of patient s was co n t rol i n c e r tai n a rea s (e . g. ma nd i ble a nd s kul l), 53 % ; while t h e a ct u a ria l 5 - year DF S was 30 % es pec i ally in t um o r s <5 c m in d i amet er wher e t h e (Fi g. 1a and b ). Th ere w as no s tatisti ca lly sign i® c a nt tr ea tm en t r el a te d m or bid i t y wou l d n o t b e 5,10,11 di fferen c e i n 5 - year OS and D F S betwe en m a les and increased. fema les (54 % vs 50 % and 2 6% vs 36 % , res pectively) . Th e l oca l co nt rol r ate i n t he p res en t s tud y was Tum o r size d i d n o t s ee m t o h ave any imp act o n O S 71 %.Th is ® gur e i s lower t han the 9 0 % ® gu re r eported and D F S .Th e a ct ua rial 5- year DF S for patien t s with in o t h er ser ie s . Th er e a re t w o m ain f act o r s tum o r size o f 5± 10 c m was 30 % com p ared to 2 9 % affecting t he l oca l co nt rol r ate, the se a re the i ni tial for thos e with t um o rs >10 c m in d iameter ( p = 0.93). tum o r size a nd t h e t ot al r adiation d o s e d e l iver ed . Ana lysis of the s urvival res ults (both D F S and O S ) Th e m ed i a n d o s e i n t h is stu d y (5 0 4 0 c G y) i s acc or di ng t o t he p rimary tum o r site d id n o t s how any co ns idered a deq ua te t o a chiev e l oc al c o n t rol b y mo s t 12,13 sign i ® c ant i mp act o f tha t f actor on t he t reatme n t inv es tiga to r s. Of th e 3 p ati en t s who r ec e i ved a ou t com e . T h e m a ndible h ad the h igh est 5 - year OS tot al r adi ati on d o s e £ 40 0 0 c G y, on l y 1 d e v el op ed (80 % ), f ollowe d b y the m a xi lla, skull a nd o r bit ( 71 % , loc a l r ec u r ren c e . Th e l ow er loc a l co n t rol r ate ® gu r e 50 % and 3 8 % , respect ivel y). Th is di fferen c e w as no t ob tai ne d i n t h e p res ent s tud y seem s to b e a ttr ibuted statisti cally sign i ® c ant . to t h e l arge t um o r size ( >10 c m ) i n t h e m ajority o f Th e r espons e to c he mo t herapy treatme n t w as the ou r patien t s (1 6 / 2 4 c a ses). M o s t of ou r pati en t s on l y progno s tic f actor which affected b oth t he O S pres ent r el ativel y late i n t h e c o u r se of the i r di sea se and D F S . Th e 5 - year OS for patien t s with c o mp lete du e t o l on g d i stance s , cu l tur al r ea sons and l ack of remi ssion (CR ) w as 68 % comp ared to 0 % for patient s ef ficien t p rima ry ca re h os pital s in r em ot e a rea s of with p artial r emi ssion (PR ) t o c hem o the rapy ( p = the k ing d o m . 0. 01 5 ) ; a lso the 5 - year DF S for CR patien t s was Th e 5 - year actua rial DF S as wel l a s OS in t he sign i ® c ant ly h ighe r t han that o f PR patient s (27 % vs present s tud y (30 % and 5 3% , respect ively) a re lower 0%; p = 0.02). than the ® gur es reported b y IESS (80 % survival at 3 yea rs). Aga in we a ttri bute t ho se l ower ® gur es ma inly to t w o f act o r s: th e l a rge r tu m or siz e a t i ni t i a l Discussion pres ent at ion a s wel l a s the l ow c o m p lianc e r ate t o ch em ot he r apy tre a tme n t , with o n l y 59 % of patien t s Ew ing ’ s sarcoma s arising f rom the b one s of the h ea d co m plet ing m or e t ha n six c y cl es . and n e ck regi on a re ex treme l y rare. Mo s t ca ses in t he De spite o ur lowe r s urvival rates , yet t hos e are still literatu r e a re rep orted s poradica lly as case r ep orts. high er tha n the 1 5 % 5- year rel apse free s urvival Th e l arge st r eported s eries u n t il n o w i s that o f the repor ted f or a gr oup of young p atient s ( £ 14 years) Int ergr oup Ew ing’ s Sarcoma Stud y (IES S) i n 1 9 8 7 . with the d i agn o s is of Ew ing ’ s sarco m a in o t he r Siegal et al. rep orted i n t hat s eries o n a total o f 29 anatom i c l oca tion s trea ted i n o ur ins titut e b etwee n ca ses of Ew ing ’ s sarcoma of the h ea d and n e c k r eg i on 19 8 0 a nd 1 9 9 3 ( persona l com m u n icatio n) . S imi larly, which repres en t ed 4 % of all Ew ing ’ s sarcoma cases the I ESS reported a highe r m e d ian s urvival for head reported b y the I ESS gr oup . In t he p resent s eries , and n e ck loc ation i n c om p arison t o E w ing ’ s sarcoma Ew ing ’ s sarcoma of head a nd n e ck co ns titut ed 9 % of in o ther anatom i c s ites . all Ew ing ’ s ca ses trea ted i n t his ins titut e ( 2 4 / 2 5 9 Th e o nl y progn os tic f actor sign i® c a ntly a ffecting ca ses). Th is rel atively high ® gur e m i gh t b e a ttribu ted to t he f act that o u r hospital is the m a in ref erral both D F S and O S in t his stud y was the r es pons e to on c ol og y c e nt er in t he r eg i on . Th e m a le t o f ema le ch emo t her apy. Th is ® nd i ng i s in a gr ee me n t w ith t he ratio was 2.4: 1, this is in a ccor da nce w ith t he l iteratur e rece nt a rticles t hat e mp hasize t he i mp ortanc e o f the being a di sease wher e m ales are mo re f reque nt ly ini tial r es pons e to c he mo t herapy treatme nt a s the affected. Th e m e di an age a t d i agn o sis for thi s stud y on ly signi ® c a nt i nd e pen d e nt p redi ctor o f survival for was 16 . 5 y ears, which rep resent s an exp ec ted ® gu re Ew ing ’ s sarcoma patient s. It s houl d b e n o t ed , 14 A. Allam et al. Fig. 1. (a) O v eral l sur vival for t he whole group. Fig. 1. (b) D isease-free sur vival for t he whole group. however , that t he p ower o f subanalysis in t his study altern a ting c he mo t he rapy com b ination r egi me n h as was low d u e t o t he s mall nu m b er of patien t s. already yiel de d a statisti ca lly sign i ® c ant i mp roveme nt In t he e arly phase of the p res en t s tud y, patien t s in D F S when c om p ared t o t he s tandard VACA alone wer e t r ea te d w it h th e u s ua l V AC co m bin a ti o n in a phase III rando m i zed s tudy . In c on c lus ion, reg i me n s , howe v er , starting i n 1 9 9 5 , with th e Ew ing ’ s sarcoma of the h ea d and n e ck reg i on c a rries int rod u c tion o f mo re a gg r es sive che mo t herapy, we a slightly b et ter progn o sis than that i n o ther sites. are no w us ing V ACA alter na ting w ith I /E (ifosfa- Mu ltimo da lity the rapy co ns isting o f an ini tial b iopsy, mi de , e t opos ide) f or a total o f 52 w eek s. Th is ne w aggr essive com b ination c he mo t he rapy and l oc alized Ewing’ s sar coma o f t he he ad an d neck 15 8 Posnick J C, Loui e G , Zuk er R, Weitzma n S . E wing ’ s radiothe rapy is the u s ual trea tme n t o f cho ice a nd sarcoma : p rima ry i nvolveme nt o f the z ygoma u nde r- co uld r esul t i n l ong -term d isea se-free survival. Radical going r esection a nd i mm e di ate r econs tructio n. Plast surge r y ma y be p erforme d f or loc al c on t rol i n c er tain Re constr Surg 1992; 89:956± 61. anatomi c l ocation s (e.g. ma ndib ular and s kull les ions), 9 Fi orillo A , Tranfa F, C ana le G , et al. Primary E wing’ s howev er, thi s ne ed s to b e f ollowe d b y an adeq uate sarcoma o f the m a xilla, a r are and c urable l oc alizatio n: recon struc tive s urge ry .Th e r esponse to c h emo therap y repor t o f tw o n e w cas e s, su c cess f ul l y t r eate d b y radi otherapy a nd s ystemi c c hemo t herapy. Ca ncer Le tters tr ea tm en t r em ain s on e o f th e m os t i m por ta nt 1996; 103:177± 82. prog n o s tic f act o r s affect i ng t h e D F S as wel l a s the 10 Zenk e K , Hatake yam a T , Has himo t o H , Sa kak i S , OS of patient s with E w ing’ s sarcoma of the h ea d and Ma na be K . Primary E wing’ s s arcoma o f the o cciptal ne c k r egi on. bon eÐ C ase r epor t. Neurol M e d C h ir 1994; 34:246± 50. 11 Mi shra H B, Haran R P , Joseph T , Chand i S M. Primary Ewing ’ s s arcoma o f the s ku ll. A r eport o f two c ases. B r References J Neurosurg 1993; 7:683± 6. 12 Ra zek A , Perez CA, Tefft M, et al. Intergr oup E wing ’ s 1 Seigal G P , OliverWR, R e inusW R, et al. Primary E wing’ s Sa rcoma S t udy . L ocal c ont rol r elated t o r adiatio n d o se, sarcoma i nvol ving t he b on es o f the h ead a nd n e ck, volume , and s ite o f prima ry l esion i n E wing’ s s arcoma . Cancer 1987; 60:2829± 40. Cancer 1980; 46:516± 21. 2 Watana be H , Tsubokaw a T , Ka tayama Y , K o yama S , 13 Bu r ne t N G, Bl iss J M, Harme r CL. The imp act o f Nakamu ra S . P rimary E wing ’ s s arcoma o f the t emp oral radi otherap y d o se o n l ocal c on trol o f Ewing ’ s s arcoma bone. Surg Neurol 1992; 37:54± 8. of bone. Sarcoma 1997; 1:31± 8. 3 Al varez A , Schut L , Br uce D. Localize d p rima ry i nt rac- 14 Delepi ne HC, Al ka l laf S, Br un B , Ma rko w ska B , ranial E wing ’ s s arcoma o f the o r bital r oof. Case r epor t. Desbois J C. Is a ge a p r ogno stic f actor in l ocalise d J Neurosurg 1979; 50:811± 3. Ewing ’ s s arcom a t reated b y m u ltidrug regime ns a nd 4 O’ Connell J E, Calder C , Ra afat F, P roops D. Ewing ’ s systema tic s urgery ? Pr oceedings of t he Am e ri can Society sarcoma o f the r etrop harynx . The Journal of L a r y n- of C l inical On cology (ASCO ) , 3 3 rd A n nual M e eting , gology and O t ology 1994; 108:363± 6. abstrac t # 1890, p. 525a, 1997. 5 Had® e ld M G , Luo V Y , W illiams R L , Ward J D, Ru sso 15 Gr ier HE, Kr ailo M , et al. Imp r oved o u t com e in CP. Ewing’ s s arcoma o f the s ku ll i n a n i nf ant . Pediatr Neurosurg 1996; 25:100± 4. no n- me tastati c E wing’ s s arcoma ( EWS) a nd P NET of 6 Falk S , A l pert M . Five y ear s ur vival o f patient s w ith bon e w ith t he a ddi tion o f ifosfami de a nd e toposi de to Ewing ’ s s arcom a . Surg Gy necol Ob s t 1967; 124:319± 24. vinc ristine , adr iamyci n, c ycloph ospham i de and a ctino- 7 Dah l in D C, Coven t ry M B , Sc an l on P W. Ewing ’ s my cin: A C hildr en’ s C ancer Gr oup ( CCG) a nd Pe di- sarcoma : a c ritical a na lysis o f 165 cases. J B one Joint atric O ncolog y G r oup ( POG) R e port. Proc ASCO 1994; Surg 1961; 43A:185± 92. 13:421. 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