Tumors of Muscular Origin

Transcription

1 Chpter Tumors of Musulr Origin P.C. Seyneve, P.J.L. De Visshere, L.L. Mortelmns, A.M. De Shepper 18 Contents 18.1 Introdution Clssifition, Inidene, nd Clinil Behvior Tumors of Smooth Musle Benign Smooth Musle Tumors Mlignnt Smooth Musle Tumors Tumors of Strited Musle Benign Strited Musle Tumors Mlignnt Strited Musle Tumors Imging Imging Studies Other Thn MRI Plin Rdiogrphy Ultrsound Angiogrphy Sintigrphy Computed Tomogrphy MRI Findings Referenes Introdution Generlly speking, tumors of musulr origin re not ommon. The rdiologil literture is limited minly to se reports [17, 27, 38, 55, 59], nd even when series of soft tissue tumors re reported, the numer of musulr lesions remins low [33, 49]. In two fundmentl ppers sed on lesions seen y the Armed Fores Institute of Pthology over 10-yer period, Krnsdorf reported 311 enign tumors of musulr origin (leiomyoms) out of totl of 18,677 enign soft tissue tumors, or 1.7% [32]. Among the 12,370 mlignnt soft tissue tumors, there were 1,039 leiomyosroms (8.4%) nd 239 rhdomyosroms (1.9%). The reently onluded Multientri Europen Study on Mgneti Resonne Imging of Soft Tissue Tumors oserved 26 tumors of musulr origin out of totl of lmost 800 rndomly reported ses [14]. Although musle tumors hve een well desried nd lssified histologilly, imging tehniques hve remined of limited vlue in speifying the tissue dignosis of these msses. However, the newer imging modlities om- puted tomogrphy (CT) nd, espeilly, mgneti resonne imging (MRI) hve rought some progress in this field Clssifition, Inidene, nd Clinil Behvior Sine imging tehniques lk speifiity, it is not possile to lssify tumors y their rdiologil pperne. For this reson we will lssify the musulr tumors on the sis of histology ording to the WHO lssifition of soft tissue tumors: Smooth musle tumors A. Benign tumors 1. Angioleiomyom 2. Deep leiomyom 3. Genitl leiomyom B. Mlignnt tumors Leiomyosrom (exluding skin) Skeletl musle tumors A. Benign tumors Rhdomyom ) Adult rhdomyom ) Fetl rhdomyom ) Genitl rhdomyom B. Mlignnt tumors Rhdomyosrom ) Emryonl rhdomyosrom, spindle ell rhdomyosrom, otryoid rhdomyosrom, nplsti rhdomyosrom ) Alveolr rhdomyosrom, solid rhdomyosrom, nplsti rhdomyosrom ) Pleomorphi rhdomyosrom Tumors of Smooth Musle Benign Smooth Musle Tumors Superfiil or utneous leiomyoms must e divided into two entities: leiomyoms derived from the rretores pilorum musle omplex nd genitl leiomyoms [19].

2 294 P.C. Seyneve, P.J.L. De Visshere, L.L. Mortelmns, A.M. De Shepper d Fig. 18.1I d. Angiomyom in 46-yer-old mn with long stnding swelling in the left hnd, reently inresed in volume. Axil spin-eho T1-weighted MR imge. Sgittl spin-eho T1- weighted MR imge. Axil spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. d Axil STIR MR sequene. At the ulnoplmr spet of the fifth MCP joint, smll, nodulr, well-defined mss lesion is seen, with signl intensity equl to djent norml musle on T1-weighted MR imges (, ). After gdolinium ontrst injetion, the lesion shows no ovious enhnement (), ut on STIR MR sequene there is very high signl intensity (d). Illustrtion of n ngiomyom with nonenhnement on T1-weighted MR imges fter gdolinium ontrst injetion nd very high signl intensity on STIR MR sequene The rretores pilorum leiomyoms lie within the derml onnetive tissue. Most lesions onsist of entrl zone of smooth musle ells nd lend with the surrounding derml ollgen nd djent pilr musle. These leiomyoms re solitry or multiple pinful nodules with dimeter of 1 2 m. These smll ppules n eventully olese to fine liner pttern in the sme dermtome. They my e ssoited with dermtitis herpetiformis, HLA B8, premture uterine leiomyoms, inresed erythropoietin tivity, nd multiple endorine denomtosis type 1. They rise most frequently in the extensor surfes of the extremities. Angioleiomyom, ngiomyom, or vsulr leiomyoms re histologilly divided into three sutypes: solid, vernous, nd venous. All of these sutypes ontin nodulr onglomertes of smooth musle ells nd thik-wlled vessels (Fig. 18.1). These re rre; they ount for out 5% of ll enign soft tissue tumors. These tumors develop most frequently in women in the fourth to sixth dedes. Most of the solid histologil sutype tumors our in the lower leg s slowly growing solitry msses of severl yers durtion. Pin is prominent feture in out 50% of ll reported ses. Intrvenous leiomyomtosis onsists of enign smooth musle tissue nodules tht grow in the veins of the myometrium nd osionlly extend into uterine nd hypogstri veins. The pthogenesis remins unler. Whether these lesions develop s result of vessel invsion y one or severl endometril tumors, or whether they re formed y prolifertion of smooth musle ells within the vessel wll is still deted. The lesions develop minly in promenopusl women. Clinilly they present s norml vginl leeding nd pelvi pin. In out 50% of these ptients, the uterus is enlrged. In ertin ses rdi symptoms my our or even predominte owing to the presene of tumor in the ven v or the hert. Deep leiomyoms histologilly hve rih vsulriztion, whih my mimi soft tissue srom. The presene of stippled, plque-like, or lrger mulerry lifitions similr to those in uterine leiomyoms hs een desried in deep-seted soft tissue leiomyoms, espeilly in hildhood [18, 36, 38]. Deep leiomyoms re less frequent thn suutneous leiomyoms. Deep leiomyoms n our in the deep prts of the extremities. They ffet oth sexes eqully, wheres leiomyoms of the retroperitoneum or dominl vity our lmost exlusively in women. In our series (dtnk of soft tissue tumor of the University Hospitl Antwerp), only one histologilly proven leiomyom of the deep flexor omprtment of the rm ws found (Fig. 18.1). Leiomyomtosis peritonelis dissemint is nother rre entity nd is hrterized y multiple smooth musle nodules situted superitonelly throughout the

3 Chpter 18 Tumors of Musulr Origin 295 dominl vity. It is not ompnied y ny prenhyml disese within the dominl orgns nd does not extend extr-dominlly. Leiomyomtosis peritonelis dissemint only ours in women of hildering ge, is frequently ssoited with pregnny or orl ontreptives, nd tends to e frequent in Afro- Amerins. Genitl leiomyoms re histologilly heterogeneous lesions tht frequently ontin ells with myxoid nd epithelioid hnges. Genitl leiomyoms re smll, pinless lesions usully less thn 2 m in size tht pper in the reol of the nipple, srotum, li, penis, nd vulv Mlignnt Smooth Musle Tumors Leiomyosroms ount for etween 5 nd 10% of ll soft tissue tumors. They our typilly in dult life, lthough reently n ssoition of Epstein Brr virus with leiomyosroms in young people with AIDS nd fter orgn trnsplnttion hs een reported y severl groups [37, 40, 45]. All leiomyosroms hve the sme histologil hrteristis. They re, however, divided into three different sugroups euse of their linil nd iologil differenes [20]. Cutneous nd suutneous leiomyosroms must e differentited. The smller utneous lesions re illdefined tumors with tumor strnds lending in with the surrounding ollgen nd rretores pilorum musles. Ptients present with utneous disolortion, umilition, nd ulertion. The suutneous lesions re well irumsried nd form pseudopsule. These grow fster nd result only in skin elevtion. Cutneous nd suutneous leiomyosroms ount for 2 3% of ll superfiil soft tissue sroms. They re most ommon in men in the fifth to seventh dedes. Pin is prominent feture in oth tumors. As these tumors re mostly solitry, multipliity is lwys suggestive of metstsis from nother site. Wheres utneous leiomyosroms metstsize in 10% of ses, or less, suutneous lesions metstsize in 30 40% of ses. They differ from the retroperitonel leiomyosroms in their lk of regressive nd degenertive hnges whih is proly relted to their smller size. Leiomyosroms of the deep soft tissues re most frequent in the retroperitoneum nd in the dominl vity. More thn 60% of this sugroup our in women, usully fter menopuse. Symptoms re vgue nd nonspeifi. Less frequently, these tumors n e found in the deep soft tissues of the extremities, ffeting oth sexes eqully. Vsulr leiomyosroms re polypoid or nodulr msses tht re firmly tthed to the vessel wll nd spred long its surfe.in veins the extension to the d- jent tissues is reltively erly event, while in rteries (pulmonry rtery) the tuni elsti is usully preserved nd invsion of other orgns is sent. Vsulr leiomyosrom is rre tumor. The inferior ven v seems to e its primry site nd ounts for 50% of reorded ses, while the greter sphenous vein ounts for 25% nd the ulk of the rest rise from the femorl, internl jugulr, nd ili veins in delining order of frequeny [29, 30, 60]. Tumor reurrene is not ffeted y tumor grde, size, or djuvnt therpy [16], the prognosis depending rther on lotion nd surgil essiility. In 10% of ses, metstti disese, usully in the lung or liver, is lredy present t the time of dignosis [31]. Epitheloid or myxoid hnge nd the presene of grnulr eosinophili ytoplsm ells in leiomyosroms re rre. These tumors re lssified s epitheloid, myxoid, or grnulr ell leiomyosroms [22] Tumors of Strited Musle Benign Strited Musle Tumors In generl, enign soft tissue tumors re muh more frequent thn their mlignnt ounterprts. The opposite is true for strited musle tumors, where enign lesions ount for no more thn 2% of ll strited musle tumors. Adult rhdomyom is hmrtomtous proess tht is usully solitry nd well defined or orsely loulted. It shows gry-yellow to red-rown olor on mrosopi inspetion nd ontins lrge, round polygonl ells interseted y firous strnds [21]. It presents s pinless, round or polypoid mss in the nek, whih seems to rise from the rnhil musulture of the third nd fourth lefts. Clinilly it my use horseness or progressive diffiulties in swllowing. Most ses our in dults over 40 yers old nd re solitry tumors. However, multifolity hs een desried in out 20% ut is restrited to the nek (Fig. 18.2). Fetl rhdomyom is superfiil tumor often with muoid glistening surfe, usully polypoid or pedunulted nd less thn 5 m in size.both myxoid nd ellulr differentited sutypes n e found. It is even rrer thn the dult sutype nd ours in the hed nd nek regions of oth hildren nd dults. The medin ge is 4 yers (3 58 yers), with 2.4:1 mle predominne. The lssi fetl rhdomyom hs prediletion for the posturiulr soft tissue. Some my e relted to neuromusulr hmrtom (enign Triton tumor). Genitl rhdomyoms form group of polypoid or uliflower-like msses overed y epithelium. These tumors onsist of entrlly sttered musle fiers nd mtrix of ollgen nd muoid mteril. They present

4 296 P.C. Seyneve, P.J.L. De Visshere, L.L. Mortelmns, A.M. De Shepper d e Fig. 18.2I f. Adult rhdomyom of the forerm in 76-yer-old mn. Axil spin-eho T1-weighted MR imge. Axil spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. Axil spin-eho proton density MR imge. d Axil spin-eho T2-weighted MR imge. e Axil spin-eho T2-weighted MR imge with ft suppression. f Sgittl spin-eho T1-weighted MR imge with ft suppression. MRI shows round mss lesion etween the superfiil nd deep flexor digitorum musles. On T1-weighted MR imges, the lesion is inhomogeneous nd of higher signl intensity thn djent norml musle. There is entrl fous of high signl intensity (). After gdolinium ontrst injetion, mrked, inhomogeneous enhnement of the lesion is seen (). High signl intensity is seen on oth proton density nd T2-weighted MR imges (, d). On T2-weighted MR imge with ft suppression, the lesion remins hyperintense (e). On T1-weighted MR imge with ft suppression, ftty omponents n e exluded (f) f s slow-growing polypoid or yst-like msses in the vgin or the vulv of young nd middle-ged women. The medin ge is 42 yers (rnge yers). A similr lesion hs een desried in the prostte of 19-yer-old mle ptient [21, 44]. Rhdomyomtous mesenhyml hmrtoms re suutneous lesions omposed of poorly oriented skeletl musle undles lended with islnds of ft, firous tissue, nd proliferted nerves. They our in the oritl nd perioritl regions of infnts nd young hildren Mlignnt Strited Musle Tumors Rhdomyosrom is frequent in persons under 45 yers of ge. In hildren it is tully the most ommon soft tissue tumor. Aording to the Armed Fores Institutes of Pthology, fewer thn 15% of rhdomyosroms our in the extremities, with n equl distriution etween upper nd lower extremities [61]. Histologilly, this group ontins severl different types of tumors, depending on the ellulrity. The WHO lssifition modifies slightly the previously epted lssifition of Horn nd Enterline (1958). The mlignnt strited musle tumors re sudivided in n emryonl, lveolr, nd pleomorphi rhdomyosrom sugroup [28]. The emryonl group ontins the spindle ell, otryoid, nd nplsti rhdomyosrom sutypes. These sutypes rnge from poorly differentited tumors tht orrespond histologilly to the pperne of developing musle in n erly gesttionl stge to well-differentited tumors tht resemle mture fetl musle. Spindle ell rhdomyosrom is sutype of this group of tumors nd is hrterized y the prllel orienttion

5 Chpter 18 Tumors of Musulr Origin 297 d e Fig. 18.3I f. Alveolr rhdomyosrom of the left hypothenr in 11-yerold girl, presenting with pinless swelling for 6 months. The volume of the swelling hd inresed during the lst 2 weeks. Axil spin-eho T1-weighted MR imge. Axil spin-eho T2-weighted MR imge with ft suppression. Coronl spin-eho T2-weighted MR imge with ft suppression. d Axil spin-eho T1-weighted MR imge fter gdolinium ontrst injetion, dynmi sequene with sutrtion imges. e Axil spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. f Sgittl spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. The T1-weighted MR imge demonstrtes lrge mss within the hypothenr musles (dutor digiti minimi), inhomogeneous nd slightly hyperintense to djent norml musle. Infiltrtion is seen towrd the musle elly (). On T2-weighted MR imges with ft suppression, the lesion is ill-defined nd of very high signl intensity. Presene of in homogeneity with entrl sr-like omponent of low signl intensity (). On oronl plne the lesion is fusiform (). On dynmi T1-weighted MR sequenes fter gdolinium ontrst injetion, the lesion shows septl nd entrl enhnement (d). On T1-weighted MR imges, the lesion is inhomogeneous with nodulr omponents of hyper- nd hypointensity (e, f). A lrge, illdefined mss with inhomogeneous pperne on ll MR sequenes, rising from the hypothenr in hild f

6 298 P.C. Seyneve, P.J.L. De Visshere, L.L. Mortelmns, A.M. De Shepper Fig. 18.4I d. Pleomorphi rhdomyosrom of the right thigh in 63-yer-old mn. Axil spin-eho T1-weighted MR imge. Axil spin-eho T2-weighted MR imge. Axil spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. d Sgittl spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. The T1-weighted MR imge shows rounded, wellirumsried mss with mixed signl intensities (). The mss is even more inhomogeneous on T2-weighted MR imge, with the d presene of entrl fluid olletion nterior to n re of low signl intensity (). There is no enhnement of oth these res fter gdolinium ontrst injetion (, d). A mlignnt tumor is suggested y the inhomogeneous pperne in ll sequenes, lotion, nd volume of the lesion. The pleomorphi sutype is hrterized y res of nerosis lternting with res of mrked enhnement nd res of ring-like enhnement round low signl-intensity res of ells hving some resemlne to leiomyosrom. Reognition of this sutype is importnt euse of its good prognosis. Emryonl rhdomyosrom ounts for 50 60% of ll rhdomyosroms (3 million US hildren less thn 15 yers of ge).it priniplly ffets hildren up to the ge of 15 yers nd ours minly in the hed nd nek region, the orits, the genitourinry system, the retroperitoneum, nd the extremities. Botryoid rhdomyosrom is vrint with polypoid (grpe-like) growth pttern nd onsists minly of muoid mtrix with some sprse ells [46]. This myxom-like tumor is often overed y hyperplsti or squm-like epithelium. It ounts for 5 10% of ll rhdomyosroms nd ours priniplly in muoslined hollow viser suh s the vgin nd ldder. The nplsti rhdomyosrom is histologilly defined y the presene of enlrged, typil ells with hyperhromti nulei. Fol nplsti fetures n e seen in ll sutypes, ut in the nplsti sutype diffuse nplsi with the presene of lone-like lusters of nplsti ells re predominnt. Alveolr rhdomyosrom (solid nd nplsti) is omposed of individul ellulr ggregtes tht re seprted nd surrounded y frmeworks of dense, firous sept tht ontin dilted vsulr hnnels. It is the seond most frequent type nd ounts for 20% of ll mlignnt strited musle tumors. Adolesents nd young dults etween 10 nd 25 yers of ge re most frequently ffeted. It n e found in the sme lotions s the emryonl rhdomyosrom, lthough it tends to our more often s deep-seted mss in the extremities. Alveolr rhdomyosroms hs worse prognosis thn other rhdomyosroms (Fig. 18.3). Pleomorphi rhdomyosrom is histologilly diffiult to differentite from other pleomorphi soft tissue tumors. It ontins loosely rrnged, lrger pleomorphi ells. Cross-stritions tht re found in other su-

7 Chpter 18 Tumors of Musulr Origin 299 types re rre in this group. It ounts for 5% of ll rhdomyosroms nd ours minly in ptients over 40 yers of ge. Its prediletion site is the thigh (Fig. 18.4). Clinilly, rhdomyosroms re rpidly growing msses whih tend to use pin nd nerve ompression symptoms when they reh lrge volume. These tumors pper to involve one more frequently thn other soft tissue sroms, prt from synovil srom. In series pulished y Simmons nd Tuker, one invsion in ssoition with the primry tumor ws seen in more thn 20% of ses. Remrkly only flt ones were invded. Bone destrution ws permetive nd only exeptionlly well defined. Slerosis ws n unexpeted finding in ssoition with suh n ggressive tumor [52] Imging Imging Studies Other Thn MRI Plin Rdiogrphy Although plin rdiogrphy nnot e expeted to provide speifi informtion out the nture of soft tissue lesion, it remins useful to strt the evlution of ny soft tissue tumor with plin rdiogrphs [6, 29]. Aprt from giving some ide out the size nd lotion of the mss, this revels the presene of lifitions within the lesion nd shows ny oexisting one involvement [34, 50]. For est results, low-kilovoltge tehnique should e used to provide mximum density differentition etween tissues [9, 46] Ultrsound Ultrsound, s hep nd redily ville tehnique, is of use in the generl workup of soft tissue msses providing informtion out size nd internl hrteristis [5, 57, 68, 69]. Its min use for estlishing preise dignosis lies in guiding perutneous iopsy [3, 10, 33]. Color Doppler tehniques re useful in ssessing vsulrity ut offer no help in the dignosis of musulr tumors Angiogrphy Angiogrphy. Both onventionl ut-film nd digitl sutrtion ngiogrphy n e used for preopertive vsulr mpping nd to estlish ess for intr-rteril hemotherpy [17, 34]. Angiogrphi findings in soft tissue tumors re entirely nonspeifi, nd the use of ngiogrphy is therefore limited nowdys. This ertinly pplies to tumors of musulr origin. Lymphngiogrphy. Bipedl lymphogrphy hs een desried s useful only in deteting metstti lesions rising from musulr tumors in the lower trunk or in the lower extremities [4], ut is tody repled y CT Sintigrphy Both tehnetium nd gllium were one proposed s useful rdiophrmeutils, the former for deteting the presene nd delineting the extent of soft tissue tumor, nd the ltter for demonstrting mlignny. However, lk of speifiity ws lter estlished. The min role of sintigrphy lies in deteting skeletl metstses [34, 51, 53, 55] Computed Tomogrphy Before the dvent of MRI, CT ws the only imging modlity le to evlute soft tissue tumor extent nd medullry involvement [3, 35]. Its role is now restrited to the detetion of lifitions, one involvement, nd intrtumorl gs [48, 63]. In series of 14 tumors reported on y MLeod, leiomyosroms were usully lrge, with frequent neroti or ysti hnges. Clifitions were not seen. Neroti metstses were oserved in severl ses [41]. Although the CT pperne remins nonspeifi, the presene of neroti metstses n e suggestive of the dignosis. In series of 73 tumors of the thigh y Rih, there were only 4 rhdomyosroms; in ll 4 of these ses, the presene of symmetril thikening ws noted on CT sns [52] MRI Findings The vlue of MRI in grding nd hrteriztion of soft tissue tumors hs een the sujet of lrge numer of pulitions, some of them ontroversil [1, 2, 6 8, 11 15, 23, 33, 39, 43, 47, 56, 58, 62 67], nd hs een lrgely delt with in Chp. 11. In the uterus, leiomyoms pper s shrply mrginted, homogeneous res of lower signl intensity on T2-weighted imges thn the surrounding myometrium; inhomogeneity is inditive for omplited or vsulr firoids [54]. This does not seem to e the se in other lotions. In the ldder three leiomyoms presenting s n inhomogeneous mss with overll high signl nd dot-like foi of low signl on T2-weighted imges hve een pulished [42].

8 300 P.C. Seyneve, P.J.L. De Visshere, L.L. Mortelmns, A.M. De Shepper Fig. 18.5I f. Leiomyom of the flexor omprtment of the right rm in 56-yer-old womn. Ultrsound, xil plne. Plin CT. CT sn fter iodinted ontrst injetion. d Axil spin-eho T1- weighted MR imge. e Axil spin-eho T2-weighted MR imge. f Axil spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. Ultrsound shows well-irumsried, ovl nd slightly hypoehoi mss within the ieps musle (). The lesion is of low ttenution on plin CT () nd is enhned peripherlly fter ontrst injetion (). On MRI the well-defined lesion is hyperintense to musle on T1-weighted MR imges (d) nd shows n inhomogeneous, stippled high signl on T2-weighted imges (e), nd n inhomogeneous enhnement fter gdolinium ontrst injetion (f). Nonspeifi MR findings of well-irumsried, enign intrmusulr tumor d e f

9 Chpter 18 Tumors of Musulr Origin 301 Fig. 18.6I. Low-grde leiomyosrom of the vstus intermedius musle in 33-yer-old mn presenting with pin nd swelling of the left thigh. Coronl spin-eho T1-weighted MR imge. Coronl spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. Axil spin-eho T2-weighted MR imge. Presene of fusiform soft tissue mss deep in the left thigh hyperintense reltive to djent norml musle on T1-weighted imges (). After gdolinium ontrst injetion, there is mrked enhnement of the lesion (). On T2-weighted imges, the lesion is inhomogeneous nd of high signl intensity (). There re numerous feeding vessels inditing highly vsulr lesion.although the lesion exhiits imging fetures similr to those of n lveolr soft tissue srom (Fig ), histologil dignosis reveled low-grde leiomyosrom illustrting the diffiulty in MR pttern reognition versus histologil dignosis Fig. 18.7I,. Leiomyosrom of the thigh in 88-yer-old womn. Sgittl spin-eho T1-weighted MR imge. Sgittl spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. MRI shows lrge nd loulted mss displing the djent musles. On T1-weighted imges the lesion is iso- to hyperintense (). After gdolinium ontrst injetion, thik nd irregulr rim enhnement is seen surrounding res of entrl nerosis (). Inhomogeneity of mss on T1-weighted imges nd peripherl enhnement re in fvor of mlignnt tumor In our series of 26 musulr tumors, only one lesion proved to e leiomyom. It ws found in the deep soft tissues of the upper rm (Fig. 18.5). Leiomyosroms mostly present with speifi MR fetures, i.e., spindle-shped msses with long T1 nd long T2 relxtion time (Fig. 18.6). Overll low signl intensity or low signl intensity omponents llow more speifi dignosis [62]. In our own series, leiomyosroms mostly ppered s lrge msses with entrl nerosis nd peripherl rim-like enhnement fter gdolinium ontrst dministrtion (Fig. 18.7). Bone involvement is seen in 10% of ses (Figs. 18.8, 18.9) [25, 26].

10 302 P.C. Seyneve, P.J.L. De Visshere, L.L. Mortelmns, A.M. De Shepper Fig. 18.8I d. Leiomyosrom of the right rm in 36-yer-old womn. Coronl spin-eho T1-weighted MR imge. Axil spin-eho T1-weighted MR imge. Axil T2-weighted MR imge. d Axil spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. A spindle-shped mss is seen, lying long the long xis of the lim nd invding the humerus (). The lesion is inhomogeneous nd of intermedite signl intensity on the T1-weighted MR imges (, ). The tissue inhomogeneity is entuted on the T2-weighted MR imge, whih shows mixed signl intensities (). After gdolinium ontrst injetion, there is inhomogeneous enhnement (d). An ggressive musle tumor with one involvement d In ses of vsulr leiomyosroms, differentil dignosis with thromus is rther strightforwrd, sine tumor expnds the vessel to dimeter severl times the originl, while thromus never expnds the dimeter to more thn twie the originl one. In thromosis T1 nd T2 is generlly inresed, with ler delinetion of the vessel wll [59]. One se of myxoid leiomyosrom showed high signl intensity on T2-weighted imges nd mrked enhnement fter gdolinium ontrst injetion, more pronouned t the enter of the lesion (Fig ). Rhdomyosroms lso hve rther nonspeifi MR fetures, nd they re seldom loted in the extremities. Emryonl rhdomyosroms show more homogeneous low signl intensity on oth T1- nd T2- weighted imges nd no ovious intrtumorl nerosis (Figs , 18.12). Emryonl sutypes n use owing of tuulr ones in hildren, flsely mimiking slowly growing tumorl proess [62]. Alveolr rhdomyosroms re hrterized y multiple res of tumorl nerosis (Figs , 18.13, 18.14), while pleomorphi rhdomyosroms hve res of nerosis lternting with res of mrked ring-like enhnement round res of low signl intensity (Figs ). Reent dvnes in MRI tehnology llow higher-resolution imging in shorter quisition times ut no signifint progress is seen regrding tissue dignosis [24]. Dynmi ontrst-enhned MRI (prmetri imging) my help in: (1) defining res of tumor viility prior to iopsy, (2) determining response to hemotherpy, nd (3) evluting tumor reurrene following surgery [68]. Things to rememer: 1. Smooth vessel tumors hve no speifi imging fetures exept for vsulr leiomyosrom, whih mostly ours within the inferior ven v. 2. Benign strited musle tumors re extremely rre. 3. Rhdomyosrom is the most ommon soft tissue tumor in hildren, the emryonl sutype eing y fr the most frequent. 4. Leiomyosrom mostly present s lrge, spindleshped msses with vrile signl intensities, entrl nerosis,nd peripherl ontrst enhnement.

11 Chpter 18 Tumors of Musulr Origin 303 d e Fig. 18.9I e Leiomyosrom of the right thigh in 26-yer-old womn. Plin rdiogrph. Plin CT. Axil spin-eho T2- weighted MR imge. d Coronl spin-eho T1-weighted MR imge. e Coronl spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. Plin rdiogrphy demonstrtes n indistint soft tissue swelling with extensive mottled one involvement nd pthologil frture of the femorl nek (). CT onfirms these res of one erosion nd pthy slerosis (). The soft tissue mss surrounds the hip nd extends towrd the midline. It presents s very hyperintense mss on T2-weighted MR imge (), while the T1-weighted MR imge shows n infiltrting tumor (d) with essentilly peripherl enhnement fter gdolinium ontrst injetion (e).aggressive musle tumor with extromprtmentl (one nd suutis) extension

12 304 P.C. Seyneve, P.J.L. De Visshere, L.L. Mortelmns, A.M. De Shepper Fig I. Myxoid leiomyosrom of the nterior left thigh in 63-yer-old womn. Axil spin-eho T1-weighted MR imge. Axil spin-eho T2-weighted MR imge. Axil spin-eho T1-weighted imge fter gdolinium ontrst injetion. MR shows loulted mss tht is isointense to musle on T1-weighted MR imge () with mixed ut minly high signl intensity on the T2-weighted MR imge () nd inhomogeneous enhnement fter gdolinium ontrst injetion (). Contrst enhnement seems to derese from the enter to the periphery of the mss. Nonspeifi mss with overwhelming myxoid omponents

13 Chpter 18 Tumors of Musulr Origin 305 d e Fig I e. Emryonl rhdomyosrom of the left uttok in 22-month-old oy. Axil spin-eho T1-weighted MR imge. Axil spin-eho T2-weighted MR imge. Axil spin-eho T1- weighted MR imge fter gdolinium ontrst injetion. d Coronl spin-eho T1-weighted MR imge (2 months lter). e Coronl spin-eho T1-weighted MR imge fter gdolinium ontrst injetion (t the sme time s d). On T1-weighted MR imge, the lesion demonstrtes hypo- to isointensity ompred with the djent musle (), while there is high signl on T2-weighted MR imge with some internl strnds of low signl intensity (). After gdolinium ontrst injetion, there is evident enhnement of some prts of the tumor, ut lrge res do not enhne t ll (). No nerosis is seen. Comprle signl ptterns re seen on follow-up MRI study with plin T1-weighted MR imge (d) nd T1-weighted MR imge fter gdolinium ontrst injetion (e). A lrge tumor with mlignnt imging findings rising from glutel musle in young hild without distint res of nerosis. This emryonl sutype of rhdomyosrom shows mrked, slightly inhomogeneous enhnement without extensive nerosis

14 306 P.C. Seyneve, P.J.L. De Visshere, L.L. Mortelmns, A.M. De Shepper d Fig I,. Emryonl rhdomyosrom of the tongue in 15-yer-old oy, 14 yers fter tretment for rhdomyosrom t the sme lotion. Axil spin-eho T1-weighted MR imge. Axil spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. Huge mss t the tongue se, hrdly to differentite from the intrinsi tongue musulture on T1-weighted MR imge (). After gdolinium ontrst injetion, there is mrked inhomogeneous enhnement of the lesion () Fig I d. Alveolr rhdomyosrom of the right thigh in 71-yer-old womn. Sgittl spin-eho T1-weighted MR imge. Sgittl spin-eho T1-weighted MR imge fter gdolinium ontrst injetion. Axil spin-eho T2-weighted MR imge. d Axil spin-eho T1-weighted MR imge fter gdolinium ontrst injetion.a voluminous mss with very inhomogeneous signl hrteristis is seen on T1-weighted MR imges. There re hypointense res suggestive of entrl nerosis (). After gdolinium ontrst injetion, only peripherl enhnement is seen (, d). Higher signl intensity is seen on proton density nd T2-weighted MR imges espeilly in the neroti res (). Lotion, lrge size, inhomogeneous signl intensity on T1-weighted MR imges, high signl intensity on T2-weighted MR imges, nd mostly peripherl enhnement fter gdolinium injetion re in fvor of mlignnt musulr tumor. The lveolr sutype is hrterized y multiple res of nerosis

15 Chpter 18 Tumors of Musulr Origin 307 Fig I. Alveolr rhdomyosrom of the upper rm in 50-yer-old mn. Axil T1-weighted MR imge., Axil () nd sgittl () T1-weighted MR imge fter gdolinium ontrst injetion. Collr utton-shped mss within the trieps musle of the upper rm, hyperintense to djent norml musle on T1- weighted MR imges () nd strongly enhning fter gdolinium ontrst injetion (). On sgittl plne the lesion hs fusiform shpe with ill-defined mrgins proximlly nd distlly (). Histologil exmintion fter iopsy nd resetion reveled n lveolr rhdomyosrom. This se illustrtes the vrile morphology nd signl-intensity hrteristis of rhdomyosroms

16 308 P.C. Seyneve, P.J.L. De Visshere, L.L. Mortelmns, A.M. De Shepper d e Fig I f. Rhdomyosrom of the ddutor region of the right thigh in 78-yer-old womn. Plin CT. CT fter iodinted ontrst injetion. Arteriogrphy with diret retrogrde femorl rtery injetion. d Coronl spin-eho T1-weighted MR imge. e Axil spin-eho T2-weighted MR imge. f Coronl spineho T1-weighted MR imge fter gdolinium ontrst injetion. CT shows lrge nd loulted soft tissue mss with entrl hypodensities (). There is mrked enhnement fter iodinted ontrst injetion: rim-like enhnement is seen surrounding res of f nerosis (). Arteriogrphy revels the presene of neovsulrity nd of tumorl lush (). On MR imges there is mss with multiple entrl hypointensities on T1-weighted MR imges (d), whih eome hyperintense on T2-weighted MR imges (e). After gdolinium ontrst injetion, strong enhnement t the periphery is seen round the intrlesionl neroti res (f). This se of highly mlignnt tumor presents with lrge res of intrtumorl nerosis nd neovsulrity on ngiogrphy

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