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Primary adrenal ganglioneuroma: CT findings in four patients.Primary Adrenal Ganglioneuroma: CT Findings in Four Patients Gregory L. Johnson1 Ralph H. Hruban2 Fray F. Marshall3 Elliot K. Fishman1 OBJECTIVE. The purpose of this study was to characterize the appearance of adrenal ganglioneuroma on CT scans and to define the specific imaging features of the tumor. CONCLUSION. Adrenal ganglioneuroma is an uncommon benign tumor that is re- vealed as a solid adrenal mass on CT scans. Specific CT features such as calcification and le- sion enhancement may simulate a primary adrenal carcinoma. However. in our four patients. no signs of local invasion or vascular extension were noted. The specific diagnosis of adrenal ganglioneuroma requires either biopsy or surgical removal for documentation. Received October 2. 1996; accepted after revision November 26, 1996. tThe Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins Medical Institutions, Baltimore, MD 21287. Address correspon- dence to E. K. Fishman, Department of Radiology, Johns Hopkins Hospital, 600 N. Wolfe St., Baltimore, MD 21287. 2Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 21287. 3Department of Urology, The Johns Hopkins Medical Institutions, Baltimore, MD 21287. AJR 1997;169:169-171 0361-803X/97/1 691-169 nign neoplasm arising from neural crest tissue. Adrenal ganglioneuro- composed of mature ganglion cells and Schwann’s cells in a fibrous stroma [I ]. Char- acteristically, ganglioneuromas do not secrete excess catecholamines or steroid hormones, and they are rarely associated with hyperten- sion [2]. Because of this lack of hormone production. ganglioneuromas are usually undergoing abdominal imaging studies for unrelated reasons. Because it is an uncommon lesion. adrenal ganglioneuroma is not typi- cally included in the differential diagnosis of incidental asymptomatic adrenal masses. We have collected the records of four patients whom we treated for pathologically proven ganglioneuromas. and we present the CT de- scription of this unusual entity with patho- logic correlation. available at our institution. The patients were two men and two women (25-70 years old: mean age. 49 years old). Clinical presentations varied. Pa- tients typically presented with abdominal pain. but no patient was suspected of having an adrenal tu- mor before radiologic examination. pertensive agent. None had clinical symptoms or biochemical evidence suggestive of adrenal corti- cal or medullary hyperfunction. No primary ma- lignancies or other evidence of metastatic disease were identified in any of the patients. Unenhanced and enhanced CT scans were ob- tamed in all four cases. Measurement of the CT at- tenuation values were available in two cases. In the other two cases. attenuation was defined rela- tive to the liver or kidney. Results The maximum diameter of the four tu- mors ranged from 5 to 9 cm. In all four pa- tients, the mass occurred in the right adrenal gland (Figs. 1-4). All lesions were fairly homogeneous in attenuation and ap- pearance on the unenhanced scans and in three cases on the enhanced scans. No evi- dence of necrosis or hemorrhage was seen. The lesions had smooth borders, and no ev- idence of local extension or invasion was noted. Clear planes were present between the liver and adrenal masses. No evidence of invasion of the renal vein, adrenal vein, or inferior vena cava was seen. In two of the four cases, areas of calcification were noted either centrally or in the periphery of the lesion. Definite enhancement was noted in two cases in which IV contrast material was administered. The range of enhance- D ow nl oa de d fr om w w w .a jr on lin e. or g by 2 7. 70 .1 29 .2 0 on 0 3/ 22 /2 3 fr om I P ad dr es s 27 .7 0. 12 9. 20 . C op yr ig ht A R R S. F or p er so 170 AJR:169, July 1997 Fig. 1.-61-year-old woman with 5 x 4 cm right adrenal mass. A, Unenhanced CT scan shows homogeneous mass (solid arrow) measur- ing 20 H. Right adrenal mass displaces upper pole of right kidney (open arrow) posteriorly. B, Enhanced CT scan shows enhancement of 51 H (solid arrow). Posteriorly displaced strongly enhancing upper pole of right kidney is again seen (open arrow). Adrenal ganglioneuroma was pathologically confirmed. Fig. 2.-41-year-old man with incidentally detected 6 x 5 cm right ad- renal mass. A, Unenhanced CT scan shows mass as homogeneous and of solid at- tenuation, measuring 20 H. Left renal stone is incidentally seen. B, Enhanced CT scan shows enhancement to 57 H. Adrenal ganglio- neuroma was pathologically confirmed. Fig. 3-70-year-old woman with 5 x 4 cm right adrenal mass. A, Unenhanced CT scan shows mass of attenuation equal to that of kid- ney. Note central area offaint calcification. Mass showed no significant enhancement Adrenal ganglioneuroma was pathologically confirmed. B, High-power photomicrograph shows histologically mature ganglion cells (arrows) embedded in matrix of irregular bundles of benign Schwann’s cells. (H and E, x 200) Fig. 4-25-year-old man with 9 x 7 cm right adrenal mass. A, Unenhanced CT scan shows mass of lower attenuation than liver. Foci of calcification (arrow) are also presentwithin periphery of lesion. B, Enhanced CT scan shows mass as minimally enhanced. Adrenal ganglioneuroma was pathologically confirmed. #{149}ft:1Patient Data and CT Characteristics of Primary Adrenal Ganglioneuromas Patient Lesion None None Central Peripheral AJR:169, July 1997 171 unenhanced CT scans the tumors typically measured approximately 20 H, and, after contrast material administration, they ranged from 30 to 57 H (Table 1). Discussion sympathetic nervous system that occurs in all age groups but is more common before the age of 60 years. Ganglioneuromas may arise anywhere along the paravertebral sym- pathetic plexus and occasionally are found within the adrenal medulla [2]. Rarely, neu- roblastoma may mature into ganglioneu- roma [11. Also, ganglioneuroma may occur in association with other tumors such as pheo- chromocytoma. These cases are not ad- dressed in this report. manifestation of disease would not be cx- pected. The tumor is commonly identified serendipitously in patients undergoing radio- logic study for other reasons. This occult nature often presents a challenge to the radiologist at- tempting to differentiate adrenal ganglion- euroma from other nonhyperfunctioning cal carcinoma, myelolipoma, and hemangi- oma. CT is an effective diagnostic tool for asymptomatic cortical lesions such as my- elolipomas or adenomas because of the pres- ence of fat and low attenuation, respectively [3-5]. Ganglioneuroma offers no such dis- criminating components, but certain charact- eristics may help to support or at least suggest the diagnosis. These characteristics include a solid round adrenal mass without evidence of tumor invasion. nal mass depends on many factors, includ- ing lesion size, attenuation value, clinical history of endocrine dysftmction (e.g., Cush- ing’s disease), or the presence of a known pri- mary tumor (e.g.. lung cancer). Most centers will monitor an incidental adrenal mass that is less than 4 cm in maximum diameter in the absence of adrenal dysfunction or known pri- mary cancer [6]. However, a lesion larger than 4 cm even in these patients is more problematic. Because adrenal carcinoma ble to distinguish between a silent adrenal cancer and a ganglioneuroma without biopsy or surgical resection. nal masses often includes scintigraphic evalu- ation. Because of the lack of exogenous hormone secretion, scintigraphic studies of ganglioneuroma typically reveal discordant the side with the mass) [6, 7]. However, this scintigraphic pattern is also seen with adreno- cortical carcinoma. Careful analysis of the CT appearance of the lesion may help distin- guish between the two entities. However, we cautiously note that no single characteristic could definitively distinguish between the two lesions. carcinoma are typically solid and may en- hance with iodinated contrast material. How- ever, in our series ganglioneuromas enhanced homogeneously (three cases) or minimally heterogeneously (one case), whereas a more irregular enhancement pattern would be cx- pected in cases of adrenal carcinoma. Local invasion into vascular structures including the adrenal vein, the renal vein, and the infe- rior vena cava is present in more than 50% of cases of adrenal carcinoma. No evidence of vascular invasion by adrenal ganglioneuroma was seen in this series, and the finding has been reported as uncharacteristic by other authors [2, 8]. The presence of calcification can occur in either lesion and is not helpful in differentiating these lesions. in various areas of the body and noted that many of the lesions were of low CT attenu- ation [9], which does not appear to be the case in our series of adrenal ganglioneu- romas. The lesions appear to be generally solid in attenuation, which would correlate with the histopathology (Fig. 3B). All four masses occurred in the right adrenal gland. This occurrence, although interesting, is likely attributed to the small sample size of our study. In conclusion, primary adrenal ganglio- neuromas are uncommon benign neural crest tumors. In our series, the CT appearance was usually that of a homogeneous solid mass greater than 5 cm in maximum dimension with enhancement after contrast media in- jection. Although the appearance, including the presence of calcification, may overlap with that of primary adrenal carcinoma, the lack of both clinical symptoms and local in- vasion may help suggest the diagnosis of adrenal ganglioneuroma. with this work. Semin Roentgenol 1988:23:205-215 2. Karsner HT. Thmors of the adrenal. In:Karsner HT. ed. Atlas oftumorpatholog 2nd series. Be- thesda, MD: Armed Forces Institute ofTumor Pa- thology, 1986:253-260 3. Korobkin M, Bmdeur FJ,Yutzy 0G. et al. Differen- Dation of adrenal adenomas from nonadenomas us- ing CT attenuation values. AiR 1996;166:53l-536 4. Dunnick NR, Korobkin M. Characterization of adrenal masses. AJR 1995:164:637-642 5. Francis IR, Gros MD, Shapiro B. Korobkin M. Quint LE. Integrated imaging of adrenal disease. Radiology 1992:184:1-13 tally discovered adrenal masses: a functional and morphological study. Ep Clin Endocrinol 1993: 101: 13 1-137 7. Nakajo M, Yshiaki N, Yonekura R, Iwashita S. Goto T. The role of adrenocortical scintigraphy in the evaluation of unilateral incidentally discov- ered adrenal and juxtadrenal masses. Ann NucI Med 1993:7:157-166 patients. Pediatr Radio! 1983:313: 1 1-18 9. Kumar AJ, Kuhadja FP, Martinez CR. Fishman EK, Jezio DV, Siegleman SS. Computed tomog- raphy of extracranial nerve sheath tumors with pathological correlation. J Comput Assist Tornogr 1983:7:857-865