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Letters to the Editor

Letters to the Editor Sarcoma (1998) 2, 193± 196 : Ma ny thanks for asking m e t o c om m e n t o n t he protoc ol w ith p re-oper ative RT fo r retrope r itone al Sir Du t ch c o ns ens us do cu m e nt o n `D i agn o sis and tumours. Tr eatme nt o f Soft Ti ssue T u m o ur s’ . Chem o ther apy is a mo s t co nf used t opic. R eading Th e D u t ch c o ns ens us do c um e nt o n s oft tissue the l iterat ur e, adjuvant c he mo t he rapy is not i nd i - sarcoma (STS ) i s well d on e , c o mp rehens ible a nd ca ted o u tside c linica l trials. Th e m e t a-analysis stim- apart from mi no r rema rks it c o ul d b e b asically ul ates e thica l questions and i n t he n e ar futur e o ther acc ep ted a s a standa rd. inv es tiga tors will p robably state t hat a djuvant Ne ver thel es s, the d oc um e n t g i ves the i mp ression ch emo t herapy is ma ndator y in s elect ed c ases. tha t a gr ee me n t e x i sts amon g s peci alists in t he t rea t- A mi no r rem a rk is tha t t he t er m of `com p art- me n t o f STS , and t ha t b y simply following t hes e me n t’ , so frequent ly a dop ted s ince t he e arly 19 8 0 s , gu i de line s, ev eryone i s allowed t o t rea t t hes e lesions . is no w ob solet e. In f act the o n l y us ef ul co mp artmen t In p ractic e t his is far from true . that d e termi ne s the q uality of surge r y is the m u s cu- In o ur ma jor Italian ca nce r c e nt re, a nd i t h appens lar fascia. Th e d e ® n i tion o f int ramus cul ar or ext ra- in m a ny ot he r E u r opea n institu t ions , 60 % of STS mu s cu l ar lesion s hould b e p referred. are seen fo r res cue t rea tme n t a fter ina dequa te t he ra- In c on c lus ion sarcoma s are rare, the d a ta con- pies p erforme d e lsew her e. After imp ortant p rogr es s fused a nd p hysicians should n o t h ave the p rivilege t o in s urgi ca l cul tur e a nd p roce du r es , the l ikel ihood o f treat p atien ts b y thei r own f eeling . It i s mo r e e thi cal ach iev ing a goo d l oca l con t rol i mp roved, but o nl y in for any eligi ble p atien t t o b e e nt ered i nto a clini cal ma jor ins tituti on s . trial, and t hat f or any simple o r co mp licate d c a se of Results o f treatme nt r ema in very poor. Sur geo ns STS , or wher e i t i s suspected, the p hysician no t and r adioth er apists ins ist on s tressing t hei r imp ort- familiar with t his di sea se should c o nt act a speci ® c ant r ole w hile u p to 5 0 % of patien ts w ith h igh -grade centre. tum o ur s di e f rom me tastat ic d i sea se waiting f or a A. AZZARELLI mi ssing e ffect ive m e d i cal t rea tme n t. I n t hes e ci r- Istituto Na zi onale Tumori cu m s tance s not hing u p to n o w can b e s tanda rdized Mi lan o, Italy or imposed. Surge ry is also no t o p tima l even w her e s kill and facilities are bes t, but t he o v erall survival for me t astatic s pread di sco ur age s us from mo re h ea vy, Sir: Ma ny thanks for asking m e t o c o mm e n t o n t he mu t ilating o r da nge rous proce du r es . Th e d o cu m e nt Du t ch n a tionw ide a cce pt ed c on s en sus do c um e nt o n di scus ses the p ossible d ang e rous imp act o f the u ne x - `Di agn o sis and T r eatme nt o f Soft Ti ssue T u m o ur s’ . pect ed ® nd i ng o f sarcoma , typica lly after mi no r Th ere i s no d o ub t t ha t t he m e cha nism by which op er ations performed i n n on - oncol og ical d ep art- this con s ens us doc um e nt w as dr awn u p has in i tself me n t s. De s pite e x p ect ations , these p atient s have, in bee n v er y ther apeut ic. I t a ppears to h ave bee n d e m- ou r series , a bet ter progn o sis (da ta reported a t t he oc ratica lly de r ived a fter a total o f ni ne m e et ing s and EC CO C onf erenc e , H amburg 1 99 7 ) . T h is fact i s cl early mu c h t hou gh t a nd e f fort h as gon e i nto i ts probably du e t o a favourable sel ect ion o f patien ts produc t ion. pres en ting w ith s mall super ® cial l es ions with a slow It i s a ver y prescr iptive d o cu me n t , d e ® n i ng w hat gr owing p atter n. should b e d on e i n v irtua lly ev er y ci rcu m s tance Radiothe r apy (RT) i s acc ep ted a s a ne ce ssary dr awing o n s tanda rd principl es o f sarcoma care. I proced ur e c o mp lem e nt ary to s urge ry, but i t i s com - have no d o ub t t hat t he c l ini ci ans who attend e d t he mo n e x pe rienc e t ha t i ts effect i s proportion a l to t he me e ting s and w ho w ere i nvolved i n t he p roduc tion quant ity o f tum o ur tissue l ef t behi nd ; the t rea tme nt of thes e gu i de line s wer e a ll better i nformed a nd is acc ep table if on ly a mi crosco pic r esidu al is lef t bet ter e q uipped t o d ea l with t he c h alleng es posed b y after an adequa te m a croscopic c o mp let e r es ec tion . soft tissue s arcoma s at t he c o mp letion o f the e xe r - It i s ine ffect ive i f ma cr osco pic r esidua l tum o u r is cise. left, ev en i f mi ni ma l. It s hould b e s tres sed, and i t i s Bu t t her e a re three p roblems posed by do c um e nt s no t c lea r in t he d o cu me n t , t ha t R T i s no t a rem e d y such as this: for inc o mp lete o r imp rovable s urge ry. Again t he timi ng o f RT i s no t y et s tanda rdized . Ma ny cen tres 1. Th e g ui de l ine s are ver y preci se a nd a llow for have a satisfactory e x p erien ce w ith p re-oper ative little l atitud e . N o n- adher en ce t o t hes e RT, and i n o ur Ins titut e w e a re performing a ne w gui de l ine s ma y now be u n a cce ptableÐ b oth 1357-714X/ 9 8/030193± 04 $9.00 Ó 1998 Ca rfax P ublishing L t d 194 Letters t o the E d itor clini cally and m e d ico l eg a lly. Any aberration How mi gh t g u i de line s such as this be u s ed i n t he from the d e ® ned p athway ma y lay that c l ini - Uni ted K ingd o m? cian o pen t o c laims of an un a cc ep table or It i s an un f ortuna te f act tha t i n t he U ni ted K ing- imp roper s tanda rd of ca re for tha t p atien t . do m s oft tissue s arcoma s con t inu e t o b e m a nage d Th is ma y be t he c ase b ut e qu ally it d o e s not by a variety o f surgeo ns us ing a whole h ost of allow for latitud e o f inn ov ation, particu l arly treatme nt s ma ny of which cou l d n o t b e c on s ider ed for the e x pe rienc e d c l ini ci an. Fo r the op tima l in l igh t o f cu r rent k no w led ge . Th ere i s occ asional surge o n, howe v er, thes e gu idel i- acc ept anc e t ha t t rea tme n t a nd o u t com e s tend t o b e ne s rep resent a mi ni mu m standard of ca re bet ter i f patien t s are treated i n s pecialist c en tres f or from which he o r she varies at h is/her peril. all forms of canc er but t he d a ta con ® rming t his is 2. Th ese g u idel ine s ma ke tur gi d r eadi ng . H ow simply no t a vailable f or soft tissue s arcoma s. ma ny clini cians who w ere n o t a ctivel y Int rod u ct ion o f a do c um e nt s uch as this wou ld inv olved i n d rawing u p the o r igi na l do cu - hel p to s tanda rdize t reatme nt i n t he b igge r ce nt res me nt w ill h ave rea d them or even b e a ware (wh ere t rea tme n t w ill a lready follow acce pt ed g u i de - of them . How ma ny will g o a nd l oo k it u p lines ) but w ill p robably no t i n¯ u e n ce i n t he s ligh test when a nd i f they get a patien t w ith a soft the o c ca siona l surge on w ho is unl ikely to r ea d let tissue s arcoma ? Fo r gu idel ine s to b e e f fec- alone a ck no wl edg e a do c um e nt s uch as thi s. He w ill tive t her e n e ed s to b e a na tion al cons ens us righ tly ins ist that t her e a re no d ata showing t ha t of no t o nl y how the p atien t s houl d b e treatme nt i n a speci alist cen tre i s bet ter f or the trea ted b ut a lso by whom the p atient s houl d ind i vidua l. Unt il t his is forthco mi ng I ® rmly believe be t reated . Shoul d a ll patient s be t reated a t that t he m o s t that c an b e d o ne i s to s et a mi ni mu m a na tiona l cen tre o r is ther e s till r oom f or the standa rd of care a nd t o w idel y circul ate t his in a `occ asional’ surge on i n t his ® eld o f brief and s ucci nc t d o cu m e nt ( see T a ble 1) . onc o log y . What a re the o ut com e s, do es it Na tiona l gu i de lines shoul d i nd ee d b e r each ed b y ma tter ?Ð I believe t hat t hes e are still q ue s- co ns en s us but s houl d i dea lly follow the r eco gn i tion tions withou t c om p let e a nswer s. of the s treng t hs and w ea knesses of a cou nt ry’ s cu r - 3. Any doc um e n t s uch a s thi s mu st h ave an rent o u tco me s in a n attemp t t o i mp rove thes e. ackno w led ge d t ime l imi t o n i t s uch t hat t he gu idel ine s are review ed a nd m o di ® e d a ppro- R. J. GRIMER priatel y in l ight o f cha ngi ng ® nd ing s a nd Bi rmingham Or thopaedi c O n cology fashions. Bi rmingham, UK. Letters t o the E d itor 195 Table 1. Document describi ng mi nimum standard o f c are in treatme nt of s oft tissue sarcoma s MA NA GEME NT G UI D ELINE S FOR B ONE AND SOFT T I SSUE SARC O MA S. Introd u ction Bon e a nd s oft t issue sarcoma s a re a r are and h eteroge ne ous g roup o f tumo u rs. M a ny c linician s w ill n e ver see a c ase d u ring their p rofessi ona l c areer. Their recog ni tion i s i mp ortant h owever , because time ly i nvesti gation a nd t reatme nt c an r esul t i n cure. Their ma na geme nt r equires c lose c ol laborat ion b etween d esigna ted s peciali sts i n a m u ltidisc iplinar y t eam a nd e arly referral to s uch a r egional o r s upra-r egiona l s ervice w ill l ead t o t he b est c lini cal a nd c ost e ffective care. There gui delines h ave b een p rod uced i n l ine with c ur rent a ccepted p ractice not o n ly i n t his c ou ntry b ut w ithin E ur ope a nd the U n i ted S tates. T hey s tem f rom c ons ensus c onferences i n t hese c oun tries a nd f rom p ub lished g ui delines w hich a re circulat ed t o a ll i ntereste d p arties i n t hese c ount ries. T here guide lines w ill b e submi tted f or agreeme nt t o t he R o yal C ollege of Surgeon s, T he Br itish O rthopaed ic A s soc iation, The Me dical R e search C ounc il. Pr esen tation Primary b one tum o ur s c an o c cur a t a ny a ge. W hereas o t eosarco ma a nd E wing’ s s arcom a h ave t heir p eak i ncidenc e in ado lescenc e and e arly a dul thood , c hondr osarcom a a nd o steosa rcoma s s econd ary t o r adiatio n d ama ge a nd P aget’ s d i sease increase in f requenc y w ith a ge. P ain i s t he c om m o ne st p resenti ng s ymp tom s i n p atients w ith p rima ry b on e t um o ur s. Un fo rtun ately, t his i s a c omm o n c omp laint a fter mi nor t rauma a nd s porting injuries, and a h igh i nd ex o f suspicio n i s r equired. Fe atures s uggest ive o f bony m a lignanc y a re: · night pain · non- me chani cal p ain ( ie. c ont inu ous p ain n o t a ggravat ed b y e xercise ) · swe lling t hat i s n ot a ssocia ted w ith a j oint The changes s een o n p lain r adiog raphs c an b e subtle, s uc h as p erios teal r eaction , bon e d estruct ion, new b one forma tion (calci® c ation ) a nd s oft t issue s welling . These ® nd ing s a re not s peci® c of a b one tumo ur b ut a re suggest ive a nd a lways w arrant further investig ation. Soft t issue sarcom a s i ncrease in f requenc y w ith a ge. S ome (particul arly i n y oung er patients ) m a y b e associat ed w ith f ami lial syndr ome s s uch a s n e ur o® b roma tosis a nd L i-Fr aume ni s yndr ome . They u sua lly p resent a s a p ainl ess m a ss. F e atures o f a s oft tissue l ump s uggest ive o f ma lignanc y a re: · size . 5 cm d iame ter enlarging · painful deep t o f ascia sol id m a ss ( not c ystic) recurrence at s ite o f previous e xcision ( whatever t he p revious h istolog y) An y s uch l esions s houl d i mm e diately b e referred to a s peciali st s arcoma s ervice. Investigat ions The biopsy s houl d i de ally b e unde rtaken b y t he s peciali st s urgeon w ho w ill b e respons ible fo r the d e ® nitive o peratio n b ecause the b iopsy t ract w ill n eed t o b e resected a t t hat p roc edur e. An e xcision b iop sy o r `s hell-ou t ’ is n ever a ppropr iate b ecause sarcoma s t ypicall y fo rm a p seudo capsul e t hat i s n ot a b arrier to s pread. The increasi ng u se o f cytog enetic a nalysi s i n d iagnos is and m o nitorin g of treatme nt r equi res f resh tissue f rom t he i nitial b iopsy. When a s arcom a i s s uspect ed o r demo ns trated, staging investi gations s houl d b e performed b y t he s peciali st t eam b efore biopsy, becaus e h aema toma , o ede ma a nd s car t issue reduc e the y ield o f di agno stic i nfo rma tion a nd d eline ation o f the t umo ur e xtent, particul arly i n r elation t o n e ur ovascul ar s tructur es. Films o f suspect ed s arcoma s s hould b e reviewed b y R a diolog ists t rained and e xperienced i n t his w or k. The followin g a re required b efore biopsy t o d e® n e the p rima ry t umo u r: high d e® n ition p lain r adiogra phs i n t wo p lanes · C.T. or p referably M . R. I scan o f the t umo u r The following a re required b efore de® n i tive s urgery t o e xclud e m e tastase s: · CXR and C .T scan o f thorax · bone s can ( for prima ry b one t umo u rs) · FBC, U1 E, LFT, LDH · in s elected c ases, b one ma rrow biopsy Close c o-op eration i s r equired b etwe en t he r adiolog ists, s urgeon , p atholo gist a nd o ncolog ists. S arcom a s a re not o n ly a heteroge ne ous g roup o f tumo urs b ut a w ide r ang e of appearan c es c an o c cur w ithin i ndividu al t um o ur s. S pecial t raining and me mb ership o f na tiona l b on e o r soft t issue sarcom a p atholog y p anels i s r equi red t o g ain e xperienc e in t he i nt erpretat ion o f these r are tum o ur s. I n o rder t o b e able t o c omm e nt o n g rade, n ecrosis , m i totic r ate a nd a de qua cy o f the m a rgins, t he p atholog ist mu st b e able t o o rientat e t he s pecime n a ccurate ly b efore tissue r etractio n o c curs. T rojani g radi ng s hould b e us ed f or soft t issue sarcoma s. P atholog y r eview by a s arcoma p anel i s o b ligator y fo r ent ry i nto c linical t rials a nd l eads t o r evision o f the d iagnos is in 1 0± 30% of cases. 196 Letters t o the E d itor De® nitive Surgery Surgery fo r prima ry b on e t um o ur s a nd m o st s oft tissue sarcoma s s houl d o n ly b e und ertaken b y a pprop riately t raine d s urgical on cologi sts. T his w ill u s ua lly i mp ly a n o rthopa edi c o ncolog ist f or bone tum o ur s o r a s urgeon w orking in a r ecognis ed s arcoma un it w ith a ppropr iate t raining and e xperience. I t i s e ssentia l t hey w ork w ithin a m u l tidiscip linary t eam w ith a ppropr iate s up port facilities . T here is n o p lace fo r the ª o ccasion a l o perator º fo r these t umo u rs. Surgery i s d e® n e d a s:- IncompleteÐ when a ny m a croscop ic t umo ur i s l eft in s itu. T his r equi res r e-excisi on . M arginalÐ when t he p seudoc apsule i s v isible o r a ny c learanc e ma rgin i s , 1 cm w ithou t fa scia W ide - w hen t here is a c uff of no rma l t issue a t l east 1 cm l aterally ( or a fa scial p lane) a nd 3 cm l on gitud ina lly RadicalÐ when t he e ntire m u scular comp artme nt Contami natedÐ when r up tur e of the p seudo capsul e r esults i n t umo ur s pillage Adj uvant Tr eatme n t For prima ry b one tumo u rs, p re and p os t o perativ e c hemo therapy s hould b e given i n a ccorda nce w ith t he c urrent M R C /EOI prot ocols. R e ferral to a r egion al o nc ol ogy u ni t i s e ssent ial f or this. For soft tissue s arcoma s, t he r ole o f adj uvant c hemo therapy i s u ncertai n. P atients w ith h igh g rade t umo urs s hould b e cons ide red f or enr olme nt i nto t he c ur rent E ORTC soft t issue s arcoma s tud y. T his r equires a n e arly d ecision r egarding chemo therapy a nd a ll c ases s hould b e di scus sed a t a m u ltidisc iplina ry m e eting f ollowin g d e® n itive h istol ogy o f the r esected tumo ur. A d juvant r adiothe rapy i s r equi red f or mo s t s oft t issue s arcoma s a nd c lose l inks b etween t he o peratin g surgeon , s urgical un it a nd t he l ocal r adi otherap ist a re essentia l. Follow u p a nd Re c urrence Patient s c an p resent w ith l ocal r ecurrence of any s arcoma m a ny y ears a fter prima ry s urgery. They s houl d b e promp tly r eferred back t o t he s peciali st s arcoma t eam f or conside ration o f de® n itive s ur gery. Some patients w ill h ave m e tastase s a t p resenta tion a nd m a ny o t hers w ill d e velop t hem a t a l ater d ate. S ome of these, particul arly p ul mo n ary m e tastase s, c an b e treated b y s urgery. For this r eason, sarcoma p atient s s hou ld b e followed u p b y t he speciali st s arcoma t eam, with r egular C XRs. W idesprea d m e tastase s a re incurabl e, but c an b e palliated b y c hemo therapy i n a p roporti on o f case. T reatme nt s hou ld b e given b y a m e dical o nc ol ogist i n t he s peciali st t eam u s ing c ur rent p rot ocols o f the MR C and E ORTC grou ps. PR O MP T R E FERR A L OF ANY S US PECT E D MU S CU L OSKELETA L MA LIGN A NC Y T O A RE GION A L/ SUP R A RE GION A L CE NT R E IS S TR O NG LY R E CO MM E ND ED. 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Letters to the Editor

Sarcoma , Volume 2 – Jan 1, 1998
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Publisher
Hindawi Publishing Corporation
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Copyright © 1998 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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10.1080/13577149877966
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Abstract

Sarcoma (1998) 2, 193± 196 : Ma ny thanks for asking m e t o c om m e n t o n t he protoc ol w ith p re-oper ative RT fo r retrope r itone al Sir Du t ch c o ns ens us do cu m e nt o n `D i agn o sis and tumours. Tr eatme nt o f Soft Ti ssue T u m o ur s’ . Chem o ther apy is a mo s t co nf used t opic. R eading Th e D u t ch c o ns ens us do c um e nt o n s oft tissue the l iterat ur e, adjuvant c he mo t he rapy is not i nd i - sarcoma (STS ) i s well d on e , c o mp rehens ible a nd ca ted o u tside c linica l trials. Th e m e t a-analysis stim- apart from mi no r rema rks it c o ul d b e b asically ul ates e thica l questions and i n t he n e ar futur e o ther acc ep ted a s a standa rd. inv es tiga tors will p robably state t hat a djuvant Ne ver thel es s, the d oc um e n t g i ves the i mp ression ch emo t herapy is ma ndator y in s elect ed c ases. tha t a gr ee me n t e x i sts amon g s peci alists in t he t rea t- A mi no r rem a rk is tha t t he t er m of `com p art- me n t o f STS , and t ha t b y simply following t hes e me n t’ , so frequent ly a dop ted s ince t he e arly 19 8 0 s , gu i de line s, ev eryone i s allowed t o t rea t t hes e lesions . is no w ob solet e. In f act the o n l y us ef ul co mp artmen t In p ractic e t his is far from true . that d e termi ne s the q uality of surge r y is the m u s cu- In o ur ma jor Italian ca nce r c e nt re, a nd i t h appens lar fascia. Th e d e ® n i tion o f int ramus cul ar or ext ra- in m a ny ot he r E u r opea n institu t ions , 60 % of STS mu s cu l ar lesion s hould b e p referred. are seen fo r res cue t rea tme n t a fter ina dequa te t he ra- In c on c lus ion sarcoma s are rare, the d a ta con- pies p erforme d e lsew her e. After imp ortant p rogr es s fused a nd p hysicians should n o t h ave the p rivilege t o in s urgi ca l cul tur e a nd p roce du r es , the l ikel ihood o f treat p atien ts b y thei r own f eeling . It i s mo r e e thi cal ach iev ing a goo d l oca l con t rol i mp roved, but o nl y in for any eligi ble p atien t t o b e e nt ered i nto a clini cal ma jor ins tituti on s . trial, and t hat f or any simple o r co mp licate d c a se of Results o f treatme nt r ema in very poor. Sur geo ns STS , or wher e i t i s suspected, the p hysician no t and r adioth er apists ins ist on s tressing t hei r imp ort- familiar with t his di sea se should c o nt act a speci ® c ant r ole w hile u p to 5 0 % of patien ts w ith h igh -grade centre. tum o ur s di e f rom me tastat ic d i sea se waiting f or a A. AZZARELLI mi ssing e ffect ive m e d i cal t rea tme n t. I n t hes e ci r- Istituto Na zi onale Tumori cu m s tance s not hing u p to n o w can b e s tanda rdized Mi lan o, Italy or imposed. Surge ry is also no t o p tima l even w her e s kill and facilities are bes t, but t he o v erall survival for me t astatic s pread di sco ur age s us from mo re h ea vy, Sir: Ma ny thanks for asking m e t o c o mm e n t o n t he mu t ilating o r da nge rous proce du r es . Th e d o cu m e nt Du t ch n a tionw ide a cce pt ed c on s en sus do c um e nt o n di scus ses the p ossible d ang e rous imp act o f the u ne x - `Di agn o sis and T r eatme nt o f Soft Ti ssue T u m o ur s’ . pect ed ® nd i ng o f sarcoma , typica lly after mi no r Th ere i s no d o ub t t ha t t he m e cha nism by which op er ations performed i n n on - oncol og ical d ep art- this con s ens us doc um e nt w as dr awn u p has in i tself me n t s. De s pite e x p ect ations , these p atient s have, in bee n v er y ther apeut ic. I t a ppears to h ave bee n d e m- ou r series , a bet ter progn o sis (da ta reported a t t he oc ratica lly de r ived a fter a total o f ni ne m e et ing s and EC CO C onf erenc e , H amburg 1 99 7 ) . T h is fact i s cl early mu c h t hou gh t a nd e f fort h as gon e i nto i ts probably du e t o a favourable sel ect ion o f patien ts produc t ion. pres en ting w ith s mall super ® cial l es ions with a slow It i s a ver y prescr iptive d o cu me n t , d e ® n i ng w hat gr owing p atter n. should b e d on e i n v irtua lly ev er y ci rcu m s tance Radiothe r apy (RT) i s acc ep ted a s a ne ce ssary dr awing o n s tanda rd principl es o f sarcoma care. I proced ur e c o mp lem e nt ary to s urge ry, but i t i s com - have no d o ub t t hat t he c l ini ci ans who attend e d t he mo n e x pe rienc e t ha t i ts effect i s proportion a l to t he me e ting s and w ho w ere i nvolved i n t he p roduc tion quant ity o f tum o ur tissue l ef t behi nd ; the t rea tme nt of thes e gu i de line s wer e a ll better i nformed a nd is acc ep table if on ly a mi crosco pic r esidu al is lef t bet ter e q uipped t o d ea l with t he c h alleng es posed b y after an adequa te m a croscopic c o mp let e r es ec tion . soft tissue s arcoma s at t he c o mp letion o f the e xe r - It i s ine ffect ive i f ma cr osco pic r esidua l tum o u r is cise. left, ev en i f mi ni ma l. It s hould b e s tres sed, and i t i s Bu t t her e a re three p roblems posed by do c um e nt s no t c lea r in t he d o cu me n t , t ha t R T i s no t a rem e d y such as this: for inc o mp lete o r imp rovable s urge ry. Again t he timi ng o f RT i s no t y et s tanda rdized . Ma ny cen tres 1. Th e g ui de l ine s are ver y preci se a nd a llow for have a satisfactory e x p erien ce w ith p re-oper ative little l atitud e . N o n- adher en ce t o t hes e RT, and i n o ur Ins titut e w e a re performing a ne w gui de l ine s ma y now be u n a cce ptableÐ b oth 1357-714X/ 9 8/030193± 04 $9.00 Ó 1998 Ca rfax P ublishing L t d 194 Letters t o the E d itor clini cally and m e d ico l eg a lly. Any aberration How mi gh t g u i de line s such as this be u s ed i n t he from the d e ® ned p athway ma y lay that c l ini - Uni ted K ingd o m? cian o pen t o c laims of an un a cc ep table or It i s an un f ortuna te f act tha t i n t he U ni ted K ing- imp roper s tanda rd of ca re for tha t p atien t . do m s oft tissue s arcoma s con t inu e t o b e m a nage d Th is ma y be t he c ase b ut e qu ally it d o e s not by a variety o f surgeo ns us ing a whole h ost of allow for latitud e o f inn ov ation, particu l arly treatme nt s ma ny of which cou l d n o t b e c on s ider ed for the e x pe rienc e d c l ini ci an. Fo r the op tima l in l igh t o f cu r rent k no w led ge . Th ere i s occ asional surge o n, howe v er, thes e gu idel i- acc ept anc e t ha t t rea tme n t a nd o u t com e s tend t o b e ne s rep resent a mi ni mu m standard of ca re bet ter i f patien t s are treated i n s pecialist c en tres f or from which he o r she varies at h is/her peril. all forms of canc er but t he d a ta con ® rming t his is 2. Th ese g u idel ine s ma ke tur gi d r eadi ng . H ow simply no t a vailable f or soft tissue s arcoma s. ma ny clini cians who w ere n o t a ctivel y Int rod u ct ion o f a do c um e nt s uch as this wou ld inv olved i n d rawing u p the o r igi na l do cu - hel p to s tanda rdize t reatme nt i n t he b igge r ce nt res me nt w ill h ave rea d them or even b e a ware (wh ere t rea tme n t w ill a lready follow acce pt ed g u i de - of them . How ma ny will g o a nd l oo k it u p lines ) but w ill p robably no t i n¯ u e n ce i n t he s ligh test when a nd i f they get a patien t w ith a soft the o c ca siona l surge on w ho is unl ikely to r ea d let tissue s arcoma ? Fo r gu idel ine s to b e e f fec- alone a ck no wl edg e a do c um e nt s uch as thi s. He w ill tive t her e n e ed s to b e a na tion al cons ens us righ tly ins ist that t her e a re no d ata showing t ha t of no t o nl y how the p atien t s houl d b e treatme nt i n a speci alist cen tre i s bet ter f or the trea ted b ut a lso by whom the p atient s houl d ind i vidua l. Unt il t his is forthco mi ng I ® rmly believe be t reated . Shoul d a ll patient s be t reated a t that t he m o s t that c an b e d o ne i s to s et a mi ni mu m a na tiona l cen tre o r is ther e s till r oom f or the standa rd of care a nd t o w idel y circul ate t his in a `occ asional’ surge on i n t his ® eld o f brief and s ucci nc t d o cu m e nt ( see T a ble 1) . onc o log y . What a re the o ut com e s, do es it Na tiona l gu i de lines shoul d i nd ee d b e r each ed b y ma tter ?Ð I believe t hat t hes e are still q ue s- co ns en s us but s houl d i dea lly follow the r eco gn i tion tions withou t c om p let e a nswer s. of the s treng t hs and w ea knesses of a cou nt ry’ s cu r - 3. Any doc um e n t s uch a s thi s mu st h ave an rent o u tco me s in a n attemp t t o i mp rove thes e. ackno w led ge d t ime l imi t o n i t s uch t hat t he gu idel ine s are review ed a nd m o di ® e d a ppro- R. J. GRIMER priatel y in l ight o f cha ngi ng ® nd ing s a nd Bi rmingham Or thopaedi c O n cology fashions. Bi rmingham, UK. Letters t o the E d itor 195 Table 1. Document describi ng mi nimum standard o f c are in treatme nt of s oft tissue sarcoma s MA NA GEME NT G UI D ELINE S FOR B ONE AND SOFT T I SSUE SARC O MA S. Introd u ction Bon e a nd s oft t issue sarcoma s a re a r are and h eteroge ne ous g roup o f tumo u rs. M a ny c linician s w ill n e ver see a c ase d u ring their p rofessi ona l c areer. Their recog ni tion i s i mp ortant h owever , because time ly i nvesti gation a nd t reatme nt c an r esul t i n cure. Their ma na geme nt r equires c lose c ol laborat ion b etween d esigna ted s peciali sts i n a m u ltidisc iplinar y t eam a nd e arly referral to s uch a r egional o r s upra-r egiona l s ervice w ill l ead t o t he b est c lini cal a nd c ost e ffective care. There gui delines h ave b een p rod uced i n l ine with c ur rent a ccepted p ractice not o n ly i n t his c ou ntry b ut w ithin E ur ope a nd the U n i ted S tates. T hey s tem f rom c ons ensus c onferences i n t hese c oun tries a nd f rom p ub lished g ui delines w hich a re circulat ed t o a ll i ntereste d p arties i n t hese c ount ries. T here guide lines w ill b e submi tted f or agreeme nt t o t he R o yal C ollege of Surgeon s, T he Br itish O rthopaed ic A s soc iation, The Me dical R e search C ounc il. Pr esen tation Primary b one tum o ur s c an o c cur a t a ny a ge. W hereas o t eosarco ma a nd E wing’ s s arcom a h ave t heir p eak i ncidenc e in ado lescenc e and e arly a dul thood , c hondr osarcom a a nd o steosa rcoma s s econd ary t o r adiatio n d ama ge a nd P aget’ s d i sease increase in f requenc y w ith a ge. P ain i s t he c om m o ne st p resenti ng s ymp tom s i n p atients w ith p rima ry b on e t um o ur s. Un fo rtun ately, t his i s a c omm o n c omp laint a fter mi nor t rauma a nd s porting injuries, and a h igh i nd ex o f suspicio n i s r equired. Fe atures s uggest ive o f bony m a lignanc y a re: · night pain · non- me chani cal p ain ( ie. c ont inu ous p ain n o t a ggravat ed b y e xercise ) · swe lling t hat i s n ot a ssocia ted w ith a j oint The changes s een o n p lain r adiog raphs c an b e subtle, s uc h as p erios teal r eaction , bon e d estruct ion, new b one forma tion (calci® c ation ) a nd s oft t issue s welling . These ® nd ing s a re not s peci® c of a b one tumo ur b ut a re suggest ive a nd a lways w arrant further investig ation. Soft t issue sarcom a s i ncrease in f requenc y w ith a ge. S ome (particul arly i n y oung er patients ) m a y b e associat ed w ith f ami lial syndr ome s s uch a s n e ur o® b roma tosis a nd L i-Fr aume ni s yndr ome . They u sua lly p resent a s a p ainl ess m a ss. F e atures o f a s oft tissue l ump s uggest ive o f ma lignanc y a re: · size . 5 cm d iame ter enlarging · painful deep t o f ascia sol id m a ss ( not c ystic) recurrence at s ite o f previous e xcision ( whatever t he p revious h istolog y) An y s uch l esions s houl d i mm e diately b e referred to a s peciali st s arcoma s ervice. Investigat ions The biopsy s houl d i de ally b e unde rtaken b y t he s peciali st s urgeon w ho w ill b e respons ible fo r the d e ® nitive o peratio n b ecause the b iopsy t ract w ill n eed t o b e resected a t t hat p roc edur e. An e xcision b iop sy o r `s hell-ou t ’ is n ever a ppropr iate b ecause sarcoma s t ypicall y fo rm a p seudo capsul e t hat i s n ot a b arrier to s pread. The increasi ng u se o f cytog enetic a nalysi s i n d iagnos is and m o nitorin g of treatme nt r equi res f resh tissue f rom t he i nitial b iopsy. When a s arcom a i s s uspect ed o r demo ns trated, staging investi gations s houl d b e performed b y t he s peciali st t eam b efore biopsy, becaus e h aema toma , o ede ma a nd s car t issue reduc e the y ield o f di agno stic i nfo rma tion a nd d eline ation o f the t umo ur e xtent, particul arly i n r elation t o n e ur ovascul ar s tructur es. Films o f suspect ed s arcoma s s hould b e reviewed b y R a diolog ists t rained and e xperienced i n t his w or k. The followin g a re required b efore biopsy t o d e® n e the p rima ry t umo u r: high d e® n ition p lain r adiogra phs i n t wo p lanes · C.T. or p referably M . R. I scan o f the t umo u r The following a re required b efore de® n i tive s urgery t o e xclud e m e tastase s: · CXR and C .T scan o f thorax · bone s can ( for prima ry b one t umo u rs) · FBC, U1 E, LFT, LDH · in s elected c ases, b one ma rrow biopsy Close c o-op eration i s r equired b etwe en t he r adiolog ists, s urgeon , p atholo gist a nd o ncolog ists. S arcom a s a re not o n ly a heteroge ne ous g roup o f tumo urs b ut a w ide r ang e of appearan c es c an o c cur w ithin i ndividu al t um o ur s. S pecial t raining and me mb ership o f na tiona l b on e o r soft t issue sarcom a p atholog y p anels i s r equi red t o g ain e xperienc e in t he i nt erpretat ion o f these r are tum o ur s. I n o rder t o b e able t o c omm e nt o n g rade, n ecrosis , m i totic r ate a nd a de qua cy o f the m a rgins, t he p atholog ist mu st b e able t o o rientat e t he s pecime n a ccurate ly b efore tissue r etractio n o c curs. T rojani g radi ng s hould b e us ed f or soft t issue sarcoma s. P atholog y r eview by a s arcoma p anel i s o b ligator y fo r ent ry i nto c linical t rials a nd l eads t o r evision o f the d iagnos is in 1 0± 30% of cases. 196 Letters t o the E d itor De® nitive Surgery Surgery fo r prima ry b on e t um o ur s a nd m o st s oft tissue sarcoma s s houl d o n ly b e und ertaken b y a pprop riately t raine d s urgical on cologi sts. T his w ill u s ua lly i mp ly a n o rthopa edi c o ncolog ist f or bone tum o ur s o r a s urgeon w orking in a r ecognis ed s arcoma un it w ith a ppropr iate t raining and e xperience. I t i s e ssentia l t hey w ork w ithin a m u l tidiscip linary t eam w ith a ppropr iate s up port facilities . T here is n o p lace fo r the ª o ccasion a l o perator º fo r these t umo u rs. Surgery i s d e® n e d a s:- IncompleteÐ when a ny m a croscop ic t umo ur i s l eft in s itu. T his r equi res r e-excisi on . M arginalÐ when t he p seudoc apsule i s v isible o r a ny c learanc e ma rgin i s , 1 cm w ithou t fa scia W ide - w hen t here is a c uff of no rma l t issue a t l east 1 cm l aterally ( or a fa scial p lane) a nd 3 cm l on gitud ina lly RadicalÐ when t he e ntire m u scular comp artme nt Contami natedÐ when r up tur e of the p seudo capsul e r esults i n t umo ur s pillage Adj uvant Tr eatme n t For prima ry b one tumo u rs, p re and p os t o perativ e c hemo therapy s hould b e given i n a ccorda nce w ith t he c urrent M R C /EOI prot ocols. R e ferral to a r egion al o nc ol ogy u ni t i s e ssent ial f or this. For soft tissue s arcoma s, t he r ole o f adj uvant c hemo therapy i s u ncertai n. P atients w ith h igh g rade t umo urs s hould b e cons ide red f or enr olme nt i nto t he c ur rent E ORTC soft t issue s arcoma s tud y. T his r equires a n e arly d ecision r egarding chemo therapy a nd a ll c ases s hould b e di scus sed a t a m u ltidisc iplina ry m e eting f ollowin g d e® n itive h istol ogy o f the r esected tumo ur. A d juvant r adiothe rapy i s r equi red f or mo s t s oft t issue s arcoma s a nd c lose l inks b etween t he o peratin g surgeon , s urgical un it a nd t he l ocal r adi otherap ist a re essentia l. Follow u p a nd Re c urrence Patient s c an p resent w ith l ocal r ecurrence of any s arcoma m a ny y ears a fter prima ry s urgery. They s houl d b e promp tly r eferred back t o t he s peciali st s arcoma t eam f or conside ration o f de® n itive s ur gery. Some patients w ill h ave m e tastase s a t p resenta tion a nd m a ny o t hers w ill d e velop t hem a t a l ater d ate. S ome of these, particul arly p ul mo n ary m e tastase s, c an b e treated b y s urgery. For this r eason, sarcoma p atient s s hou ld b e followed u p b y t he speciali st s arcoma t eam, with r egular C XRs. W idesprea d m e tastase s a re incurabl e, but c an b e palliated b y c hemo therapy i n a p roporti on o f case. T reatme nt s hou ld b e given b y a m e dical o nc ol ogist i n t he s peciali st t eam u s ing c ur rent p rot ocols o f the MR C and E ORTC grou ps. PR O MP T R E FERR A L OF ANY S US PECT E D MU S CU L OSKELETA L MA LIGN A NC Y T O A RE GION A L/ SUP R A RE GION A L CE NT R E IS S TR O NG LY R E CO MM E ND ED. 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Sarcoma – Hindawi Publishing Corporation

Published: Jan 1, 1998

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