Cornputed tomography of retroperitoneal ganglioneuroma Gase reporl U!j B.

Frennhy. Il. .Vgrnnn. a n d P . Aspelin

Dcpartiiiciit ofDi;igiiosl.ic: Iliiili~~logy. I'nivcrsity oil.~iiid.Alliiiiiiiiiii sjiikhiisei. Malniij. Swcdcii

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Retroperitoneal diagnostics

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gnnglioneurorria - CT

Schlüsselwörter

oii both prc- and postcoiitrast scans. A thin lincar structiire was secn on pnstcontrasl. scans posterior to tha i n k r i o r vena cava and betweeii ttie liver and thc mass. This slriiclure was thought to represent the right adrcnal. Ultrasonography showed a lesion with mixed echogenicity and a sonographically guided finc-ncedle hiopsy was perforrncd although no cytologic spccirncn was obtainc~d.At explorntion a retroperitoneal 14 x 1 2 X 9 cni solid turnour surrounded by a tliin capsule was found. It did adhere to thc aorta but not 1.0 ariy

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Rctroperitoneales Ganglioneurorn - CT

Ganglioiieiiroina is an uncommon benign tiiinoiir ccbrriposed of mature ganglion cclls and nerve fibres. Thr turnours arc gcncrally encapsulnted. slowly growing and witti no hormoiial activity (5. 6. 8. 13-15). Stout (14) rcvicwcd tlie liternIiire iii 1947 and found that 60 % of thc cascs (243 patit!iits) occurred beiore thc agc of 20 with n slight predorninance of fernalcs ( 3 : 2). They nre niost frequeritly found retroperitoncally along the syiiipatlieticganglion chain and i n thc adrenal iiiedulla bitt less frequently i n thc ccntral ncrvous systein (5. 8, 14). Ganglioneuromas havc also bean reported iri the bladder. kidney. ovarics. Uterus, speririal.ic cord and gastrointestinal canal among othar sites (2. 5. 7, 14, 15). Whcn associatcd with voii Recklirighausen's discasc thcrc might be iiiultiple Liiniours (7. 14). Wc rcport case or a liilly dirferentiated rctropcritoneal ganglioiieiiroi~lairl an adult Woman. our k m w Ierlge tliere tias bren no previoiis rcport of such a cnss invesiigated bv c o m ~ u t c d tomoera~hv(CT) in adiilts.

Case report

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Fig. 1 a Pre-contrastscanshowinga right-sidedparaspinal tumour with a homogeneousdensityless than that of the parenchymatousorgans. vascular structuresandmuscles.

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A 32-year-old previously hcalthy fcrnale presented with a 4-year history of i n t c r m i t t ~ nattncks t of rigtit-sided abdominal pain. On physicnl exnniinaliori no abdominal mass or focnl tcndarness was found. Laboratory tcsts wcrc normal. At iritravenoiis pyelography a suprarcnal mass displacirig the right kidney downwards was sncn. Abdominal (:T (Pig. 1) deirioiistrated a rightsided rctropcritoncal mnss about 1 2 cin i n diameter. 'l'hc attcnuation was honiogeneous arid measured about 25 Hounsli~ldunits (HU). The lesion did not enhancc during an i. V . inriisiori of contrast rncdium (total dosc 33 g of iodine). No capsule was idcntificd. The tuinour was abuttirig the liver, posterio-medial aspect of tlis diaptiraarn, inferior vena cava. portal veiii. aortr?. diiodenum and head-of thc pancrcas but there were no direct C.1' SignS of infiltration oT these organs. Ttie attenuation of thc livcr appeared normal -

Fortschr. Röntgenstr. 156.2 (1992) 198--199 0 Ceorg 'l'hieme Verlag Stuttgart . New York

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Fig. 1b Post-contrastscanshowingenhancement of vascular structures and parenchymatousorgans but not of the tumour. Theinferiorvena Cava isdislocated laterallyand the duodenum (smallarrows) and head of the pancreas (largearrows)anteriorly. Fig. 1 Computed tomogramsof the abdomen.

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Microscopic exarnination of thc t.urnour showed eloiigated Schwann cells arranged i i i spirals niid widcly scattcrcd mature gaiiglioii cells with prominent nucleoli. Tlie tiiinoiir c:ontaincd scantly distributed siiinll cnlibrc vcsscls and a surrounding c,apsule with numcrous hypertrophic iierves. Thr! pathologic diagnosis wiis i i l'iilly din'erontiatcd ganglioneuromii witliciiil sigiis of nccrosis.

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In the developiiig ernbryo ncuroblasts undergo matiiration to I'orm ganglion cells. The immature iieuroblasts may give rise to neiiroblastomas. They have a strong tendency ro riietasiasize and are the most tindiffereritiated among tumours of sympathetic ganglia, i.e. neuroblastorna, ganglioneuroblastoilia, and ganglioneurorna. Ganglioneurornas on the other hand are niature, iully differentiated. benign ttinioiirs, wliile gaiiglioneuroblastornas are an interrnediate form that is partially differentiated and can occasionally give rise lo nietatases ( 8 , 12-14). Ganglioiieuronias niuy originate from mature ganglioii cells. In addition neuroblastornas rnay during treatment or spontaneoiisly uiidergo matiiration to form a ganglioireuronia (8. 13. 14). On CT the ganglioneurorna in the present case had a "cystic" appearance with a hornogeneous density, smooth borders. no identifiable capsiile and there was no enhancenient after adiniinistration of i.v. contrast rnediiiiri. Its attenuation was, however, sornewhat Iiigher (25 HU) than norrnally expected liwm a simple cyst. lkezoe et al. (9) reported five intrathoracic ganglioneurornas with a homogeneous appearance on plain Cr ranging from 15-45 HU. Three of the five cases were subjected to i.v. contrast medium administration, and contrary to our case all three tumours demonstrated enhancernent resulting in a n attenuation ranging from 40 to 70 M['. This discrepancy rnight be explained by differences in vascularity arid volunie of interstitial space. Lane et al. (10) reported two retroperitoneal ganglioiieurornas in children with a n inhomogeneous appearance on plain CT. Cariglioneuroblastomas and neuroblastornas have been described to be inhoinogeneous on plain Cr and the inhornogeneity was intensified on contrast enhanced CT images (9). A retroperitoneal paravertebral location is also Seen in other nerirogenic turnours such as neurofibrornas and schwannomas. Both appear as well-defined masses with attenuation values ranging from about 15-30 HU. Schwannomas rnay be slightly inhomogeneous on plain CT and contrast medium injection may result in rim enhancernent with a central low density area due to cyst forrnation or necrosis. Neurofibrornas appear a s hornogeneous masses both on plain and post-contrast CT (9.10). Calcifications are rarely found in these tiirnours (7). Benign paragangliomas also appear with sharp boundaries but with a density as muscle and enhances after i . V. contrast medium (10).

Cystic retroperitoneal masses rnust also be considered in the differential diagnosis such a s adrenal and renal cysts, cystic pheochromocytomas (3), and lymphangiomas (11). However, cysts originating from the adrenal or the cranial pole of the kidney should rather dislocate the inferior vena cava anteriorly than laterally as in this case. Malignant retroperitoneal tumours are a diverse group thnt mostly appear inhomogeneous on CT due to necrosis and haemorrhage (4. 10). l'he Cl' appearance of the individual turnour types correlates generally with the histopathologic lind-

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ings (10).Sorne of these turnours may appear cystic sucli a s Ieioniyosarcomil (4). liposarcoma (4). and rhabdomyosarcorna (4) These pseudocystic lesions sliow at least partially thickoned irregular and nodular walls which makes it possible lo dimer tlieiii l'roni benign tuinours (4). Calcifications have been dernonstrated by CT in ahoiit 40 % 01' iieurnblastornas and 20 '% of ganglioneuromas ( 1 . 7. 9. 12). Aniorphous calcificatioris appetir iiiore orten thnn discretc punctatc or rim calcifications in thosr tuinotirs (1). Anoltier leaiure of ihese tiinioiirs is that they. from a paraspinal location. rnay extend into the spinal carial in n dunil)l)ell l'asliion ( 1 . 2 . 6 . 8 ) . Neither of these features was Seen in otir rase. l'he present case report sliows thnt a retroperitoneal ganglioneuroma rnay exhibit features sirnilar to thal or cystic Iesioiis. i.e.. a well circumscribed homogeneous mass without enhancernent and no capsule biit with a densi1.y sliglitly higher than a typical cyst. With such a Cf finding, ganglionerirorna rniist be considered in the differential diagnosis.

References

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ilrrnstrong, E. il.. ß. C. F. Ilarwood-Nash, C. R. Ritz. S. H. C'huang, H. Pettersson. D. J. Mcirtin: CT of neuroblnstonias nnd ganglioiieuroinas in children. Amer. J. Roentgenol. 239 (1982) 571-576 Bar-Ziu, J.. M. U. .Vogrady: Mediastinal neiiroblastonia and ganglioneurorna. l'he differentiation between prirnary and secondary iiivolverneiit on tlie chest roentgcnograin. Amei. J. Hoentgenol. 125 (1975) 380-390 ßiish, W. 11.. .I. S.Elclcr, H. k'. C'rane, L. H. Wclles: Cystic pheochromocytorna llrology 25 (1985) 332 334 Cohan, R. H.: Computed tomography ofpriinnry retroperitoneal nialignancies. J . Comput. Assist. 'I'omogr. 1 2 (1988) 804-81 0 Dahl, F. V.. J. M. Waugh. D. C Dahlin: (;astrointestinal ganglioneuromas. Brief review with report of a duodenal ganglioneuroma. Am. J . Pathol. 33 (1957) 953-961 Fagan, C. J., L. E Sr1)isch~~k: Dumbbell neuroblastorna or ganglioneurorna of the spinal canal. Ainer. J. Roentgenol. 1 2 0 (1974) 453-460 ' Graharn, R. T.. S. {lerschorn. .I. Srigley: Calcified ganglioneuroma of the bladder. I'rol. Radiol. 9 (1987) 177-180 Harnilton, .I. P., E. Koop: Canglioneuromas in children. Surg. Gynecol. 0bsi.et. 121 (1965) 803-812 Ikczoe, J.,S. Sone. T. Higashihara. S. Morirnoto. J. Arisawa, K. Kuriyarna, Y. Monden, K. Nakahara, Y. Ogawu, H. Shiozaki: CT of intrathoracic iieurogeiiic tumours. Europ. J. Radiol. 6 (1986) 266-269 l 0 1,ane. R. H., D. H. Stcplzens, H. M. Reiman: Primary retroperitoneal neoplasms: CT findings in 90 cases with clinical and pathologic correlation. Amer. J. Roentgenol. 152 (1989) 83-89 l 1 Munechika, H., M. Honda, T. Kushihashi, K. Koizurni. T. Gokan: Computed tornography ol' retroperitoneal cystic lyrnphangiomas. J . Comput. Assist. Tomogr. 11 (1987) 116-1 1 9 l 2 Ostertr~n. B., Th. Harder, K. 1'. Heetz: Canglioneurom irn kleinen Becken. Fortschr. Röiitgenst.r. 147 (1987) 93-94 l 3 Perez, C. A.. T. Vietti, L. V. Ackerrnan, M. D. Eagleton, W. E Powers: Tumors of the sympathetic nervous System in children. An appraisal of trentment and results. Radiology 88 (1967) 750-760 l 4 Stout, A. P.: Ganglioneuroma of the sympathetic nervous systern. Surg. Cynecol. Obstet. 84 (1 947) 101-110 ' W y m a n , H. E.. B. S. Chappell. W. Jones Russe1 jr.: Gnnglioneuroma of bladder: rcport of a case. J . Urol. 63 (1950) 526-532 I

ßo Frennby, M. D. DepartiiientorDia@iostic Radiology Malmö General Hospital S-214 01 Malmö. Swcden

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othcr organ and il. coiild easily br rrrnoved. 'l'he adrenal was iderii.ilied and appeared normal. Tlie turnour was supplied by small vessels frnm thc aorta. 'l'ho organ oforigiii rould not be identificd but frorn tlie localization it was assurned that it was the syinpathetic ganglion chain. The postoperati\~r!coursc was uneoentiiil and the pntient was dischargcd frorn tlie hospilirl 10 days after surgcry.

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Computed tomography of retroperitoneal ganglioneuroma. Case report.

Cornputed tomography of retroperitoneal ganglioneuroma Gase reporl U!j B. Frennhy. Il. .Vgrnnn. a n d P . Aspelin Dcpartiiiciit ofDi;igiiosl.ic: Ili...
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