The many faces of neuroblastoma

Transcription

1 RdloGrphlcs Index terms: GENITOURINARY IMAGING #{149} Adrenl MUULOKELETAL IMAGING #{149} Generl/Multiple sites PEDIATRI IMAGING #{149} GnItourlnry #{149} Musculoskeietl RADIATION THERAPY #{149} Generl/Multiple ites cumultive Index terms: Neurobistom Neoplsms In Infnts nd children ,-.--,---- The mny fces of neuroblstom Ruppert Dvid, M.D. 1 Frzin Eftekhri, M.D.t Neel Lmki, M.D. Ali hirkhod, M.D.t usn Fn, M.D.* Pjendr Kumr, M.D.1 Edwrd B. ingleton, M.D.* John E. Mdewell, M.D. Absfrct: Neurobistom is common tumor in childhood. It rises in the drenl glnd or In vrious extrdrenlprimiysites of the sympthetic chin. liniclly, it mypresent s n bdominl mss or s disseminted metsttlc disese. We studied 52 ptients with neuroblstom, nd the typicl nd unusul rdiogrphic fetures ofthe disese re presented. THI EXHIBIT WA DIPLAYED AT THE 74TH IENTIFI AEMBLY AND ANNUAL MEETING OF THE PA- DIOLOGIAL OIETY OF NORTH AMERIA. NOVEMBER 27-DEEM- BER HIAGO. ILLINOI. IT WA REOMMENDED BY THE GEN- ERAL RADIOLOGY AND MAGNETI REONANE IMAGING PANEL AND WA AEPTED FOR PUBLI- ATION AFTER PEER REVIEW AND REVIION ON APRIL From the Deprtments of Rdiology, Bylor ollege of Medicine (#{149}) nd the Univensity of Texs, M.D. Anderson Hospitl nd Tumor Institute (t), Houston, Texs; The University of Texs Medicl Brnch, Glveston, Texs (t); nd the Milton. Hershey Medicl enter. The Pennsylvni tte University. Hershey, Pennsylvni (s). II Presently t RJ orpontion, 68 Binbnidge Rod, olon. OH, Address reprint requests to N. Lmki, M.D., Deprtment of Rdiology, Bylor ollege of Medicine, One Bylor Plz, Houston, TX 77. Introduction Neuroblstom is the most common solid, extrcrnil tumor in infnts nd children. it is the third most common mlignnt neoplsm of childhood, fter leukemi nd brin tumors. It represents only 7% of ll cses of childhood cncer, yet results in 15% of cncer deths in children. Next to Wilms tumor, it is the second most common intrbdominl tumor in children. Neuroblstoms re of neurl crest origin, nd most cses rise in the drenl glnd. Less often, the tumor my rise in other extrdrenl sites long the sympthetic chin, prticulrly in the retropenitonel region of the bdomen nd the posterior medistinum (Tble I). It is srcom of nervous system origin, composed chiefly of neuroblsts, but the tumor hs n enigmtic clinicl chrcter, nd its pthogenesis is unknown. TABLEI PrImry ites of Neurobiostomo (%) ite Percentile Abdomen Adrenl Glnds 6% Extrodrenl 18% Bilterl Adrenls 7-% Posterior Medistinum of the Thorx 14% Neck 5% Pelvis 5% Brin 2% Other 1% Unknown 1% #{149} Reference 8. Volume 9, Number 5 #{149}eptember, 1989 #{149}RdioGrphics 859

2 The mny fces of neurob/stom Dvid et l. U Approximtely 9% of neunoblstoms re mnifested cliniclly in the first eight yers of life, nd roughly 5% of the ptients re under two yers of ge t the onset of the lesion. Infrequently, the tumor my occur in olden children, dolescents nd young dults. Both sexes re ffected eqully. The estimted number of new cses in the United ttes ws pproximtely 5, in The differentil dignosis of solid bdominl mss in child includes neunoblstom, Wilms tumor, rhbdomyosncom or liven neoplsm. An drenl hemorrhge should lso be considered in the newborn. The rdiologist should lso be cogniznt of the drenl nd extrdrenl primry loctions of this tumor. This rticle illustrtes mny ndiologic fetunes of neuroblstom on plin rdiogrphy, computed tomogrphy (T), ultrsonogrphy (U), rdionuclide scintigrphy, mgnetic resonnce imging (MRI) nd ngiogrphy. linicl Presenttion The clinicl presenttion of neunobistom depends on the site of the primry lesion or 1- ction of its metsttic spred. A plpble bdominl mss is present in 54% of ptients, long with bdominl distention. The tumor my lso pper s lower bdominl or pelvic mss rising from the orgn of Zuckerkndl ( cluster of prgngli ner the ortic bifunction) on from the sympthetic chin in the netnopenitoneum. Pin nd fever occur in pproximtely one-third of ptients. Remote effects of this cncer include the opsoclonusmyoclonus prneoplstic syndrome nd ecchymosis. Opsoclonus is sign of cenebellr disese in which the ptient hs spontneous, conjugte, nd chotic eye movements, prticulnly when voluntry eye movement is ttempted. There Is often ssocited myoclonus of the trunk nd extremities, with cerebellr txi. This complex my be detected in onefourth of ptients with neuroblstom. Another prneoplstic complex consists of intrctble wtery dirrhe with hypoklemi nd chlonhydni. It occurs in up to 9% of ptients, nd is cused by incresed mounts of vsoctive intestinl polypeptide (VIP) secreted by the tumor. There is in lmost ll cses n increse in the levels of ctecholmines nd their byproducts. Vnillylmndelic cid (VMA), homovnillic cid (HVA) nd cystthionine re found in the urine in oven 9% of ptients, nd cn serve s clinicl biologicl mrkers. Up to % of the ptients my be hypertensive becuse of the excessive secretion of these hormones. Both norepinephnine nd epinephnine produce profound dditionl metbolic effects including incresed lipolysis, elevted oxygen consumption, rise in body temperture, nd hyperglycemi. Neurobistom my occsionlly be present t birth, in either the loclized or disseminted form. Neuroblstom my rrely pper to be fmilil tumor (.2% nnully), nd it my be ssocited with gnglionosis of the bowel or congenitl hert disese. The tumor hs even been found in premture stillborn infnts. In up to 6% of ptients, metstsis is the first mnifesttion of the disese to be necognized. The most common metsttic sites re the skeleton, regionl lymph nodes nd liven (Tble II), Bone mrrow involvement is noted histologiclly in roughly 5% of ptients subjected to biopsy. The presence of primry drenl neunoblstom with extensive skeletl metstses, prticulrly in the skull with proptosis nd bone pin, hs been referred to s Hutchinson s syndrome. Pepper s syndrome represents mssive heptomegly cused by metsttic neuroblstom nd primry drenl tumor. When there re multiple metsttic skin lesions in the neonte, it is referred to s the blueberry muffin syndrome. 86 RdioGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

3 Dvid #{149}t l. The mny fces of neuroblstom keletl TABLEII Frequent ites of Neurobistom Metstsis ite Percentge of Ptients Lymph Nodes (Regionl) Liver Intrcrnil Pulmonry 6% 42% 15% 14% 1%.5.. Pthology The tumor my be brely discernible nodule or lrge mss. Grossly, the tumor is round, irregulr nd lobulted, usully weighing 5 to 15 grms on more. It is soft nd fnible on plption nd cn esily fll prt on sectioning. The cut surfces show drk res of hemorrhge nd necrosis (Figure IA). lcifiction is frequently found within the tumor, Microscopic studies revel smll round cells (slightly lrger thn lymphocytes) of neurl crest origin, with scnt cytoplsm (2 microns). The nuclei re ovl or sphericl in shpe, with conspicuous nucleoli (Figure IB). High power microscopic sections show Homer-Wright nosettes consisting of one or two lyers of neuroblsts surrounding centrl zone of tngled neurofibnillry processes (Figure I ). Figure 1A The cut surfce of neuroblstom shows lobulted ppernce. Note the res of hemorrhge (nrows) nd necrosis (rrowheds). Volume 9, Number 5 #{149}eptember, 1989 #{149}RdioGrphics 861

4 The mny fces of neurob/stom Dvid t l. >1. hb h Figures 1B & (B) A photomicrogrph shows multiple smll ovl nd round cells typicl of neuroblstom. () This photomicrogrph shows Homer-Wright rosette within the tumor (rrow). A typicl rosette hs one or two lyers of neuroblsts surrounding centrl zone of tngled neunofibniliry processes. (ourtesy of Dr. Rolndo Estrd, Houston, Texs) A. Primry Neurobistom I. INTRAABDOMINAL NEUROBLATOMA Up to 75% of neuroblstoms occur in the bdomen, nd 5% of these tumors occur in the drenl glnd. Plin rdiognphs usully show n bdominl mss, with bowel gs displcement. Elevtion of the ipsilterl diphrgm my lso be evident. Approximtely 1% of the drenl tumors re bilterl, About 6% of these tumors show clcifictions on rdiogrphs (Figures 2, nd 4). An excretory urognm will usully demonstrte displcement of the djcent kidney (Figures 2 nd 5). If the kidney displcement is inferolterl, drooping lily sign will be seen. The tumor my cuse ureterl obstruction nd hydronephrosis (Figure 6). onogrphy is the screening exmintion of choice for peditric bdominl msses. On songrphy, the mss is usully hyperechoic (Figure 7). Acoustic shdows, which represent clcifiction within the tumor, my be seen behind the mss (Figure 8). lntrbdominl metstses my lso be detected during the initil screening procedure with sonogrphy. I Gnglioneurom is mture. benign tumor of the sme cell type s neuroblstom. Mixed forms such s gnglioneuroblstom lso occur. ee pge RdloGrphlcs #{149}eptember, 1989 #{149}Volume 9, Number 5

5 Dvid t l. The mny fces of neuroblstom. z.. Figure 2 (A) This IVP shows gnglioneuroblstom1 rising in the right drenl, with peripherl curviliner clcifictions long its inferior spect. (B) T shows the clcified tumor rising in the right drenl. B Figure (A) A lrge, hevily clcified gnglioneurom rising in the right drenl is seen in this bdominl rdiogrph. Note tht the lrge mss crosses the midline nd displces bowel loops. (B) A rdionuclide bone scn with Tc-99m MDP revels uptke of the trcer in the clcified portion of the tumor (rrows). Volume 9, Number 5 #{149}eptember, 1989 RdioGrphics 86

6 4J The mny fces of neurob/stom Dvid et l. E. z E.5... I, 1 p I Figure 4 (A) A left drenl neuroblstom shows the presence of colescent morphous clcifictions. (B) A selective left drenl rteriogrm revels fine tumor vessels, which re stretched nd displced. Note the res of hypervsculnity within the mss.. A. 4. : p! J Figure 5 (A) A left extrdrenl, retropenitonel neunoblstom is cusing widening of the left psos shdow nd lterl displcement of the pnoximl left ureter. Bowel loops re lso displced. (B) An ortognm shows the mss to be hypovsculn. Note the contnlterl displcement of the bdominl ort nd the stretching of rteries on the left. 864 RdloGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

7 Dvid t l. The mny fces of neurob/stom. z.. (A)Th IVP shows lrge neuroblstom which rose in the orgn of Zukerkndl. Note tht the unnry bldder is displced to the right, nd the uretens re compressed nd lterlly devited, resulting in bilterl hydnonephrosis. (B) A vencvognm shows mrked elevtion nd displcement of the left common ilic vein with tethering of the right cornmon ilic vein. ollterl venous chnnels re present in the pelvis nd bdomen (rrows). i I - ::.v;...,- P- - - : i:..-, - -.,;,, I - -.., i_ - Figure 7 onogrphy shows gnglioneurom tht is seen s hypoechoic mss, M, nterior to the right kidney, K. onogrphy shows neuroblstom s solid mss in the right drenl. Note the coustic shdowing produced by clcifictions within the tumor. Volume 9, Number 5 #{149}eptember, 1989 #{149}RodioGrphics 865

8 The mny fces of neuroblstom Dvid t l. E. z E. T nd MRI re complementry imging methods in the dignosis nd stging of the tumon. These techniques fcilitte delinetion of the tumor, nd cn detect intrbdominl metstses (Figures 9 nd 1). T is the imging mode of choice for stging neuroblstom in ll sites, nd is of further id in the detection of clcifiction within the tumor, which my not be pprent on plin rdiogrphs (Figure I I). Intrspinl extension from prspinl tumors occurs in pproximtely 15% of ptients, nd cn be better seen on T nd MR1 thn on plin rdiogrphs (Figure 12). The dvntges of MRI reltive to T indude the bility to produce imges in multiple plnes, the elimintion of ionizing rdition nd intrvenous contrst gent, nd the improvement in tissue contrst resolution. Encsement of the bdominl vessels cn lso be pprecited. Multidirectionl imging is of benefit for preopertive surgicl nd rdition therpy plnning. The role of ngiogrphy in the dignosis nd stging of neuroblstom hs been limited since the dvent of T nd MRI, but my be useful in difficult cses (Figures 4 nd 1). Neuroblstom is reltively hypovsculr tumor, nd the common ngiogrphic findings minly consist of stretching nd displcement of vessels, which drpe round the mss. IOA hob Figure 1 (A) A T section of the pelvis shows gnglioneuroblstom tht rises in the pelvis nd is seen s dumbbell shped mss, M. Note the intrspinl extension with bone destruction, nd lso the displcement of the bldder, B. (B) A selective left hypogstnic rteniogrm performed from night fernorl pproch shows subtle hypervsculnity, with no definite neovsculnity. The vessels re encsed, stretched nd displced by the mss. Figure 9 An MR imge (T2 weighted) shows neunoblstom rising in the left drenl s lrge mss with diffusely incresed signl intensity. 866 RdioGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

9 Dvid etl. The mny fces of neuroblstom.. z. lhb Figure 1 1 (A) This chest rdiogrph (PA view) shows rnedistinl gnglioneurom tht extends into the left hernithorx. Note the posterior left nib erosions nd symmetric intercostl spces. (B) A thoncic T scn depicts smll intntumonl clcifictions within the tumor tht re not demonstrble on the plin rdiogrph. Volume 9, Number 5 #{149}eptember, 1989 #{149}RdioGrphics 867

10 The mny fces of neurob/stom Dvid et l. E. z E Figure 12 (A&B) hest rdiogrphs (PA nd lterl views) show primry neuroblstorn rising in the left posterior medistinurn. Note the medistinl shift to the right nd nteriorly. The left posterior ribs re eroded by the tumor. () A T scn revels tht the soft tissue mss, M, is cusing bone erosion, with vertebrl forminl encrochment nd widening. Note spinl cnl invsion, Ml, nd cord,, displcement. (D) A sgittl MR imge (Ti -weighted) confirms the presence of intr- nd prspinl extensions of the tumor. 868 RdioGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

11 Dvid t l. The mny fces of neuroblstom II. INTRATHORAI NEUROBLATOMA III. INTRARANIAL NEUROBLATOMA Primry intrthorcic neuroblstoms comprise 14% of ll neurobistoms. Initilly, the ptients my complin of chest pin, nd cough becuse of irwy obstruction. The chest rdiogrph will demonstrte mss, nd there my be extrinsic compression nd displcement of the min bronchus in the cse of lrge tumor. Thorcic neunoblstoms usully occur in the posterior medistinum, where they rise from the prvertebrl sympthetic chin. Rib erosion (Figures 1 1 nd 12) nd intrspinl extension my occur (Figure 12). Loss of vertebrl height with widening of the neurl formin my be present. Lrge msses my cuse medistinl shift (Figure 12). A metsttic neck mss my result in Homer s syndrome nd superior ven cvl syndrome. Primry intrcrnil neuroblstoms occur in 2% of ptients. Most commonly, the tumor rises in the olfctory nerve (esthesioneuroblstom), cerebellum or cerebrum, On rdiogrphs, bone destruction my be seen (Figures 1 nd 14). T nd MRI revel the presence of n intrcrnil, spce occupying, soft tissue mss. Peripherl tumors my be ssocited with either lytic or sclerotic bone chnges in the skull (Figures 1 nd 14). The tumors tht rise in the posterior foss my cuse hydrocephlus. Figure 1 (A) A plin rdiogrph shows neuroblstorn rising in the nterior crnil foss nd cusing erosion of the medil wll of the right orbit (rrows). (B) A T scn of the fcil bones demonstrtes the tumor involving the night orbit with destruction of sphenoid nd ethmoid bones. Volume 9, Number 5 #{149}eptember, 1989 #{149}RdioGrphics 869

12 The mny fces of neurob/stom Dvid t l. E.. z U h4a Figure 14 (A) This lterl view of the skull shows primry infrcrnil neuroblstom of the olfctory nerve (esthesioneuroblstom). Note the destruction of the pituitry foss. (ourtesy of Dr. Bo-hn Jing, Houston, Texs) (B) A T scn shows destruction of the greter wing of the sphenoid nd of the ethmoid bone s the tumor extends into the right middie crnil foss. The right orbit is lso involved. Note the pnoptosis on the right. B. econdry Neuroblstom I. KELETAL METATAE keletl metstses occur in up to 6% of cses. Bone scintigrphy with Tc-99m MDP is useful s survey method for the detection of metsttic bone lesions. The rdionuclide trcer my lso be tken up by the primry tumon (Figure ). Erly skeletl lesions my be missed when corticl destruction is limited, nd complementry rdiogrphic bone sunvey is dvocted to detect the subtle intrmedullry chnges of insidious disese. MRI cn lso detect erly bone metstses. Rdiogrphiclly, skeletl metstsis my mnifest itself s: (I) peniostel rection (Figures 15 nd I#{24}); (2) n osteolytic focus (Figures 17, 18 nd 19); () lucent horizontl metphysel line (Figure 2); (4) verticl liner rdiolucent streks in the metdiphysis of long bone (Figure 21); (5) pthologic frctune (Figure 22); (6) multicentnic lytic lesions (Figure 2); (7) vertebrl collpse (Figure 24); nd (8) metstses to the crnium, often mnifested s widening of the crnil suture lines becuse of subjcent durl metsttic lesions (Figure 25). With heling, the osteolytic lesion my become sclerotic (Figures 26 nd 27). Metsttic bone lesions my mimic other round cell tumors, such s Ewing s srcom, rhbdomyosrcom, leukemi nd lymphom on rdiogrphs. 87 RdioGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

13 Dvid et l. The mny fces of neuroblstom U) This rdiogrph of the left knee (AP view) shows fine, hir on end peniostel rection in the distl femur (rrows). Figure 17 A rdiogrph of the right shoulder revels smll ovl, lytic metsttic lesion in the proximl humerus (rrow). Volume 9, Number 5 #{149}eptember, 1989 #{149}RdioGrphics 871

14 The mny fces of neuroblstom Dvid tl... U) Figure 18 This rdiogrph of the right knee shows n osteolytic metstsis in the distl metphysis tht extends cross the physis into the epiphysis of the right femur. Figure 19 A rdiogrph of the right knee shows moth eten pttern of bone destruction in the proximl tibi. Figure 2 A rdiogrph of the left elbow demonstrtes permetive bone destruction nd lucent metphysel line (lrge rrow) in the proximl rdius. Peniostel rection is present in the distl humerus (smll rrow). 872 RdioGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

15 Dvid t l. The mny fces of neuroblstom U) TIEs rdiogrph of the right humerus shows n osteolyticmetstsis in metdiphysis. itis mnifested s verticl streks. Figure 22 A rdiogrph of the left knee (AP view) shows permetive lesion with peniostel rection nd n undisplced pthologic frcture in the proximl tibi, Figure 2 This rdiogrph of the pelvis shows ubiquitous osteolytic skeletl metstses. Note the prtil obstruction of the left distl ureten produced by nodl mefstses. Volume 9, Number 5 #{149}eptember, 1989 #{149}Rdioorphics 87

16 The mny fces of neurob/stom Dvid #{149}t l. ạ. U) Figure 24 This lterl view of the lumbr spine shows metstses cusing mrked vertebrl collpse of L5. 25A. -AI.. -, 1 25B Figure 25 (A) This lterl skull rdiogrph shows widened crnil sutures cused by dunl metstses. (B) A followup rdiogrph, i 8 months lter, shows progressive widening of the sutures despite tretment. 874 RdioGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

17 Dvid t l. The mny fces of neuroblstom U) Thisskufl rdiogrph (lterl view) shows mixed sclerotic An AP view of the skull demonstrtes n untnetnd lytic bone lesions fter nine months of systemic che- ed mixed lytic nd sclerotic metstsis in the motherpy. frontopnietl region. II. INTRARANIAL AND MAXILLOFAIAL METATAE I. Intrcrnil metstsis is commonly seen s spce occupying lesion, nd my be mnifested cliniclly by crnil nerve deficits nd bone destruction. 2. Metstses my occur in the dun nd brin substnce. When lrge, durl metstses my compress the brin nd cuse destruction of the bone of the overlying crnium (Figures 26 nd 27). Bone sclerosis is often seen nd probbly represents rective process. Direct bone metstsis to the skull my produce lytic lesion.. Durl metsttic involvement usully is seen s widening of the crnil sutures on plin rdiogrphs (Figure 25). 4. T nd MR1 cn esily detect most intrcrnil nd crniofcil metstses (Figures 28 nd 29). T is prticulrly helpful for the detection of recurrent disese. Volume 9, Number 5 #{149}eptember, 1989 #{149}RdioGrphics 875

18 The mny fces of neuroblstom Dvid #{149}t l. U.5 U Figure 28 A T scn of the skull shows durl metstsis in the night pnietl region, which extends into the djcent bone. Figure 29 A T scn of the fcil bones shows neuroblstom metstsizing to the intrconl re of the orbit nd the ethmoid sinus on the right. III. INTRATHORAI METATAE Metsttic lesions of neurobistom re often seen in the thorx. Pulmonry metstses my occur s nodulr infiltrtes (Figure ). Bone metstses re often present in the rib cge nd thoncic spine. Rib erosions my lso occur s result of direct involvement by pnimry neuroblstom rising in the thorx (Figures I I, 12 nd 1). A ndionuclide bone scn my be required for the detection of skeletl lesions. Medistinl nd retrocrurl lymphdenopthy is commonly present (Figure 2). Figure This chest rdiogrph shows left pulmonry metstses from n drenl neunoblstom. 876 RdioGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

19 Dvid et i. The mny fces of neurob/stom Figure 1 A chest rdiogrph shows metsttic rib erosions (most mrked in the left posterior ribs) secondny to medistinl neuroblstom. Note the right thorcic scoliosis.. Figure 2 An 1VP shows left drenl neuroblstom displcing the left kidney infeniorly nd lterlly, producing the drooping lily sign. Note the nefrocrurl medistinl lymphdenopthy (blck rrows) with clcifiction (rrowhed). Volume 9, Number 5 #{149}eptember, 1989 #{149}RdioGrphics 877

20 The mny fces of neuroblstom Dvid t l. IV. INTRAABDOMINAL METATAE Intnbdominl metstses usully nepresent spred from n bdominl primry neuroblstom. These metstses my involve vnous bdominl viscer, lymph nodes (Figures nd 4), bones (Figure 9), the mesentery (Figures 4 nd 5), nd pelvic orgns (Figure 6). At times, clcifiction my be seen in intrbdominl metsttic lesions. After thenpy, T nd MRI re helpful in detecting recurrent disese, Figure onogrphy revels netropenitonel lymphdenopthy in child with left drenl neunoblstom. Note the nterior displcement of the ort. Figure 4 This T scn of the bdomen shows metsttic neunoblstom with cystic degenertion within the tumon (lrge rrows), night kidney invsion (smll nnow) nd retnopenitonel metstses (rrowheds). Figure 5 This MR imge shows right drenl neuroblstom (rrows), with mesentenic nd retropenitonel metstses, tht ws discovered t surgery. Note the night hydnonephrosis, the displced bdominl vessels, nd the mixed signl intensity in the night bdominl mesentery. 878 RdioGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

21 Dvid t l. The mny fces of neuroblstom.. I Figure 6 (A) A contrst medium filled urinry bldder from n IVP shows extninsic compression nd displcement becuse of metsttic left pelvic mss, rising from left drenl neuroblstom. (B) This trnsverse sonogrm demonstrtes left pelvic mss representing metsttic lymph nodes on the left side of the urinry bldder. (B = bldden, M = mss) Therpy The stge of the tumor will determine the mode of its therpy, but surgicl extirption is the most definitive mode of therpy, complemented with djuvnt chemotherpy nd inrdition (Tble III). Preopertive irrdition nd chemotherpy re utilized to debulk the tumor nd to down stge the disese in selected cses. Nonsungicl therpy my lso be helpful in the relief of pin cused by bone metstses. Rdioiodine lbeled met-iodobenzylgunidine (111-MIBG) hs been used for dignosing, stging nd treting tge IV neuroblstom in children, nd it my gin greten currency s more experience is chieved. Volume 9, Number 5 #{149}eptember, 1989 #{149}RdioGrphics 879

22 The mny fces of neurob/stom Dvid #{149}t l. >1. I- TABLEIII Antomic tging nd Pro9nosis of Neuroblstom tge Definition Therpy Prognosis I Tumor limited to primry orgn urgery 8% urvivl Rte II Tumor extends in continuity to ip- urgery in combintion with rdi- 6% urvivl Rte silterl structures, the homo- tion therpy or chemotherpy lterl regionl lymph nodes, or both. but does not cross the midline. III Tumor extends cross the mid- urgery plus postopertive rdi- % urvivl Rte line nd my involve regionl tion therpy lone or with chelymph nodes bilterlly. motherpy. V Tumor metstsizes to remote ombintions of surgery, che- 7% urvivl Rte res of skeleton, soft tissues, motherpy nd rdition therdistnt lymph nodes nd other orgns. py. IV- tge I or II Tumor, with metst- Vrible Therpy 75%-87% urvivl Rte with sis confined to one or more of vrious forms of therpy or no Reference 5. the following sites: liver, skin therpy, prticulrly under 1 2 or bone mrrow. months of ge. Nturl ourse of Neuroblstom Although neunoblstoms re mlignnt tumors, their clinicl course, evolution, nd response to therpy vry gretly. urrently, the physicin s bility to imge neuroblstom fr outweighs his bility to tret the disese, pnticulrly in ptients with metstses. The specific loction of the metstses, the ge of the ptient, nd the stge of the tumor on mitil recognition, re importnt prognostic fctons. Neuroblstom tends to be cliniclly less ggressive in infnts under one yer of ge, nd this group hs greter survivl rte. Neunoblstoms rising in the upper bdomen hve less fvorble prognosis thn those rising in the thorx nd pelvis. Also intriguing is the potentil of neuroblstom for mtuntion to well differentited, cliniclly less ggressive, nd indeed benign neoplsm, the gnglioneunom. uch mturtion to gnglioneunom is reported to occur in.2% of ptients with neunoblstom. It my occur either spontneously on following tretment (Figune 7). The underlying mechnism of the spontneous mturtion is still obscured. Mixed pthologic forms of the tumor, such s gnglioneuroblstoms, hve lso been neported (Figure 9). econdry mlignnt neoplsms my occur in the previously irrdited sites in the survivors of neuroblstom, 88 RdloGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5

23 Dvid t l. The mny fces of neuroblstom z z. 7A 7B Figure 7 Mturtion of neunoblstom to gnglioneunom. (A) This chest rdiogrph shows right neck mss, displcing the trche to the left. (B) A followup chest rdiogrph tken eight yers fter chemotherpy, shows tht the neck mss hs mrkedly regressed in size. A biopsy reveled it to be benign gnglioneunom. Volume 9, Number 5 #{149}eptember, 1989 #{149}RdioGrphics 881

24 The mny fces of neuroblstom Dvid t l. uggested 1. Dnemn A. Adrenl neoplsms In children. emin Roentgenol 1988; (2: Lopez-Ibor B, chwrtz AD. Neuroblstom. Peditr lin North Am 1985; 2: trk DD. Moss AA, Brsch R. et l. Neuroblstom: Dignostic imging nd stging. Rdiology 198; 148: Appelbum FR. Intensive chemotherpy or chemordiotherpy with utologous mrrow support s tretment for ptients with solid tumors. Hemtol Oncol lin North Am 1988; 2: Finklestein JZ. Neuroblstom: The chllenge nd frustrtion. Hemtol Oncol lin North Am 1987; 1: tout AP. Gnglioneurom of the sympthetic nervous system. urg Gynecol Obstet 1947; 84: Jffe N. Neuroblstom: Review of the literture nd n exmintion of fctors contributing to its enigmtic chrcter. ncer Tret Rev 1976; : Pge D. DeLellis R. Hough A. Tumors of the drenl. In: Atls of tumor pthology. Wshington. D: Armed Forces Institute of Pthology, Redings 9. Evns AE. D Angio GJ. Koop E. Dignosis nd tretment of neuroblstom. Peditr lin North Am 1976; 2: Evns AE. Nturl history of neuroblstom. In: Evns AE. ed. Advnces in neuroblstom reserch. New York: Rven I I. Kumr R. Dvid R. yle BA. et l. Adrenl scintigrphy. emin Roentgenol 1988; 2: Munkner T. 11I-met-iodobenzylgunidine scintlgrphy of neuroblstoms. emin NucI Med 1985; 15: Hdley GP, Rbe E. cnning with iodine-11 MIBG in children with solid tumors: An initil pprisl. J NucI Med 1986; 27: Hrbert J. Met(11)iodobenzylgunidine therpy of mlignnt pheochromocytoms nd other neuroendocrine lesions. In: Nucler medicine therpy. New York: Thieme, 1987; Rubin E. Aponte GE. Frber JL. Pthology. Phildelphi: Lippincott RdloGrphics Index terms: Figure 2 previously ppered in Rdiologic Oncology of the Abdomen nd Pelvis (hicgo: Yer Book Medicl pges 72 nd 725). We wish to thnk Mrs. Becky Bxter nd the supporting stff for the preprtion of this mnuscript. 882 RdioGrphics #{149}eptember, 1989 #{149}Volume 9, Number 5