A Paraganglioma of the Organ of Zuckerkandl Credits Jia-Hwia Wang*

Urological Science 23 (2012) 13e14

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Urological Science

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Practical uroradiology q CME A paraganglioma of the organ of Zuckerkandl Credits Jia-Hwia Wang*

Department of Radiology, Taipei Veterans General Hospital, School of Medicine, National Yang-Ming University, Taipei, Taiwan article info

Article history: Received 1 November 2011 Accepted 3 November 2011 Available online 24 January 2012

1. Introduction value, 95w446) pg/mL and high urine norepinephrine of 117.2 (normal value, 10 w 80) mg/24 hour. MRI of the abdomen revealed Extra-adrenal pheochromocytomas are called paragangliomas. a soft-tissue mass measuring about 3.7 Â 3.3 cm adjacent to the Most of these tumors are located in the abdominal sympathetic bifurcation of the abdominal aorta that showed intermediate ganglia, including the organ of Zuckerkandl adjacent to the signal intensity (SI) on T1-weighted images, heterogeneous high abdominal aortic bifurcation. Most patients present with parox- SI on T2-weighted images, and intense enhancement on contrast- ysmal hypertension accompanied by headaches, sweating, palpi- enhanced T1-weighted images (Fig. 2), and normal bilateral tations, and facial ﬂushing. A diagnosis can be made if elevated adrenal glands; they were compatible with a paraganglioma of the levels of serum catecholamines and urine vanillylmandelic acid organ of Zuckerkandl. The tumor was surgically excised, and the (VMA) are measured. Detection and localization are essential pathologic report revealed a paraganglioma. After the operation, before surgical resection. Computed tomography (CT) and magnetic the patient’s blood pressure returned to normal, and the post- resonance imaging (MRI) have good sensitivity for detecting and operative course was smooth. The patient was discharged, and locating paragangliomas. outpatient clinic follow-up was recommended.

2. Case report

A man who was 29 years of age had recently been complaining of headaches and chest tightness. He visited another hospital where his systolic blood pressure measured up to 200 mm Hg. He had also experienced similar episodes a few years previous, which had spontaneously resolved. Serum VMA was checked and found to be 19.81 (normal value, 1.0w7.0) mg/24 hour. CT of the abdomen was done which revealed a heterogeneous intense contrast- enhanced tumor measuring about 4 cm in diameter adjacent to the bifurcation of the abdominal aorta (Fig. 1), and normal bilateral adrenal glands; a paraganglioma of the organ of Zuckerkandl was the impression. Then, he visited our hospital, and admission was recommended for surgical excision of this tumor. After admission, a physical examination revealed normal ﬁndings except for high blood pressure, while laboratory examinations revealed normal ﬁndings except for high serum norepinephrine of 720 (normal

* Department of Radiology, Taipei Veterans General Hospital, 201 Shih Pai Road, Section 2, Taipei 112, Taiwan. Fig. 1. Axial contrast-enhanced computed tomography scan showing a heterogeneous E-mail address: [email protected]. intense contrast-enhanced soft-tissue mass measuring 4 cm in diameter adjacent to q There are 3 CME questions based on this article. the bifurcation of the abdominal aorta (arrow).

1879-5226/$ e see front matter Copyright Ó 2012, Taiwan Urological Association. Published by Elsevier Taiwan LLC. All rights reserved. doi:10.1016/j.urols.2011.12.004 14 J.-H. Wang / Urological Science 23 (2012) 13e14

Fig. 2. (A) Axial unenhanced T1-weighted magnetic resonance (MR) image showing a 3.7 Â 3.3-cm intermediate signal intensity (SI) soft-tissue mass adjacent to the bifurcation of the abdominal aorta (arrow); (B) axial unenhanced T2-weighted MR image showing heterogeneous high SI of this soft-tissue mass (arrow); (C) axial contrast-enhanced T1-weighted MR image showing intense contrast enhancement of this soft-tissue mass (arrow).

3. Discussion Conﬂicts of interest statement

Pheochromocytomas are catecholamine-releasing tumors of The author declares that he has no financial or non-financial the adrenal medulla. About 10% of pheochromocytomas are extra- conflicts of interest related to the subject matter or materials dis- adrenal and are called paragangliomas. Paragangliomas can be cussed in the manuscript. found in locations from the carotid body to the pelvic floor, and most of them are located in the abdominal sympathetic ganglia, including the organ of Zuckerkandl adjacent to the abdominal References aortic bifurcation.1 Most patients present with paroxysmal 1. Cryer PE. Pheochromocytoma. West J Med 1992;156:399e407. hypertension accompanied by headaches, sweating, palpitations, 2. Dunnick NR, Korobkin M. Imaging of adrenal incidentalomas: current status. and facial flushing. But approximately 10% of pheochromocytomas Am J Roentgenol 2002;179:559e68. and paragangliomas are incidentally discovered by imaging 3. Baguet JP, Hammer L, Mazzuco TL, Chabre O, Mallion JM, Sturm N, et al. Circumstances of discovery of pheochromocytoma: a retrospective study of 41 examinations that are performed to evaluate patients with unre- consecutive patients. Eur J Endocrinol 2004;150:681e6. 2,3 lated symptoms. If a pheochromocytoma or paraganglioma is 4. Quint LE, Glazer GM, Francis IR, Shapiro B, Chenevert TL. Pheochromocytoma suspected, a diagnosis can be made by measuring elevated levels and paraganglioma: comparison of MR imaging with CT and I-131 MIBG scintigraphy. Radiology 1987;165:89e93. of serum catecholamines and urine-related metabolites (meta- 5. Hayes WS, Davidson AJ, Grimley PM, Hartman DS. Extraadrenal retroperitoneal nephrines or VMA). Detection and localization are essential before paraganglioma: clinical, pathologic, and CT findings. Am J Roentgenol 1990; surgical resection of a pheochromocytoma or paraganglioma. CT 155:1247e50. 6. Francis IR, Korobkin M. Pheochromocytoma. Radiol Clin North Am 1996;34: and MRI have good sensitivity for detecting and locating para- 1101e12. 4e8 gangliomas. Unenhanced CT reveals a well-defined soft-tissue 7. Mannelli M, Ianni L, Cilotti A, Conti A. Pheochromocytoma in Italy: a multi- mass and marked contrast enhancement after intravenous centric retrospective study. Eur J Endocrinol 1999;141:619e24. administration of iodine-containing contrast medium. On MRI, 8. Sahdev A, Sohaib A, Monson JP, Grossman AB, Chew SL, Reznek RH. CT and MR imaging of unusual locations of extra-adrenal paragangliomas (pheochromo- it is typically seen as a mass lesion with a high signal on cytomas). Eur Radiol 2005;15:85e92. T2-weighted sequences and contrast enhancement on contrast- 9. Falke TH, van Gils AP, van Seters AP, Sandler MP. Magnetic resonance imaging e e enhanced T1-weighted sequences.8 11 Paragangliomas or meta- of functioning paragangliomas. Magnet Reson Q 1990;6:35 64. 10. Bravo EL. Evolving concepts in the pathophysiology, diagnosis, and treatment static paragangliomas can be detected by I-123 metaiodobenzyl of pheochromocytoma. Endocr Rev 1994;15:356e68. guanine or 6-[18F]-fluorodopamine positron emitting tomog- 11. Jalil ND, Pattou FN, Combemale F, Chapuis Y, Henry JF, Peix JL, et al. raphy.4,8 A biopsy is not strongly recommended due to the Effectiveness and limits of preoperative imaging studies for the localization of pheochromocytomas and paragangliomas: a review of 282 cases. French potential for a hypertensive crisis. Treatment is through surgical Association of Surgery (AFC), and The French Association of Endocrine excision. Surgeons (AFCE). Eur J Surg 1998;164:23e8.