Hypertension Research (2014) 37, 595–597 & 2014 The Japanese Society of Hypertension All rights reserved 0916-9636/14 www.nature.com/hr

CORRESPONDENCE

Retroperitoneal bronchogenic cyst: a rare incidentaloma discovered in a juvenile hypertensive patient

Hypertension Research (2014) 37, 595–597; doi:10.1038/hr.2014.38; published online 6 March 2014

Bronchogenic cysts are incidentally discov- In the laboratory examinations, the blood latent neoplasm. The resected tissue showed ered by radiological examination or during cell count, liver and renal functions, blood a brownish and dark-gray cystic tumor surgical procedures. The symptoms are glucose, and related tumor markers, (Figure 1f). Partly compressed normal adre- usually related to a tracheobronchial pathway including ((CEA), nal tissue was attached to the outer surface of obstruction and/or secondary infection or 1.90 ng ml À1 (normal, o5.0)), carbohydrate the tumor (Figure 1g). Microscopically, the hemorrhage. These cysts most commonly antigen 19-9 ((CA19-9), 6.9 Uml À1 (normal, cystic portion was partially lined by ciliated occur in the subcarinal and parahilar areas o40)), and neuron-specific enolase columnar (Figure 1h) resting on in the mediastinum. However, they have also (8.95 ng ml À1 (normal, o16.3)), were fibrous connective tissue and smooth muscle been found in retroperitoneal locations.1,2 normal, although moderate hypokalemia cells containing mucous glands and hyaline Here, we present an interesting case of (3.8 mmol l À1) was concomitantly detected. (Figure 1i). Pulmonary parenchyma or ter- juvenile hypertension accompanying a left The preoperative basal endocrine data were atomatous components were not found in adrenal incidentaloma, which was patho- as follows: adrenocorticotropin, 27.6 pg ml À1 the tumor. The resected tumor was patholo- logically diagnosed as a bronchogenic cyst (normal, 7.2–63.3); , 14.0 mgdlÀ1 gically diagnosed as an extra-adrenal retro- post surgery. (normal, 8–25); plasma renin activity, peritoneal bronchogenic cyst. Post surgery, A 27-year-old hypertensive man was 1.5 ng ml À1 h À1 (normal, 0.2–2.7); aldoster- glucocorticoid replacement therapy was not referred to our hospital for examination of one, 10.5 ng dl À1 (normal, 3.6–24); dehydro- required. As shown in Figure 1j, the patient’s a left that was incidentally epiandrosterone sulfate, 164 mgdlÀ1 (normal, systolic and diastolic blood pressure was discovered by abdominal computed tomo- 138–519); , 0.01 ng ml À1 (normal, readily normalized (117/67 mm Hg) after graphy (CT). Hypertension and obesity had o0.1);noradrenaline,0.22ngmlÀ1 the adrenalectomy, and the serum potassium been noted in annual checkups dating back (normal, 0.1–0.5); and , level gradually normalized (4.3 mmol l À1) 5 years. The patient showed moderate hyper- 0.02 ng ml À1 (normal, o0.03). The urinary within 3 weeks. After one postoperative year, tension (140–149/70 to 86 mm Hg) and obe- levels were within normal both the serum cortisol (12.0 mgdlÀ1)and sity (body mass index, 31.3), although he had ranges, including adrenaline (10.7 mgper aldosterone (8.5 ng dl À1) levels were also no Cushingoid features, such as central day, normal, 3–41), noradrenaline (108.8 mg decreased compared with their preoperative obesity and skin lesions. The abdominal CT per day, normal, 31–160), levels. showed a 5-cm circumscribed mass measur- (0.13 mg per day, normal, 0.04–0.18), norme- Bronchogenic cysts arise from the foregut ing 50 Hounsfield units (HU) in the left tanephrine (0.19 mg per day, normal, 0.1– as malformations when normal bronchial adrenal region (Figure 1a, upper), and the 0.28) and vanillylmandelic acid (4.1 mg per tubes are developmentally separated. Most right was detected to be day, normal, 1.5–4.3). Note that the urinary bronchogenic cysts occur in the mediastinum normal (Figure 1a, lower). An abdominal excretions of aldosterone (8.9 mgperday, region, and only a few cases have been ultrasound showed a cystic pattern in the left normal, 0–7.5) and cortisol (127 mgperday, reported in the retroperitoneum.1,2 There adrenal region (Figure 1b). On magnetic normal, 26–187) were slightly elevated. An are no gender-related incidence differences, resonance imaging (MRI), the adrenal adrenal scintigram using 131I-adosterol but the age range of onset (i.e., 40–70 years) tumor was detected as an ISO-intensified showed an approximately normal pattern of is older than our patient. Retroperitoneal tumor by T1-weighted images (Figure 1c), bilateral uptake in the adrenal regions; bronchogenic cysts are usually localized in in which no T2-hyperintense signal was with 131I-metaiodobenzylguanidine, the scin- the left adrenal region and the superior observed (Figure 1d). A fat suppression and tigram showed no significant accumulation in body of the pancreas.3 Most bronchogenic spectral attenuated inversion recovery a whole-body scan. cysts are pathologically benign, although (SPAIR) scan of the MRI studies showed Laparoscopic left adrenalectomy was per- malignant forms have been reported due to little inclusion of adipose tissue in the tumor formed based on the size of the adrenal characteristically increased serum levels of (Figure 1e). tumor (54 Â 38 mm2) and considering a tumor markers, including CEA and CA19-9. Correspondence 596

180 5 Lt. Adrenalectomy

SBP 4.3 4.5 120 // 3.8 Serum K 4

3.5 60 // DBP Serum K (mEq/l)

SBP, DBP (mmHg) SBP, 3

0 // 2.5 0714 30 (days) Admission Discharge

Figure 1 The radiological and pathological findings of the left adrenal tumor and the clinical course. (a) The left adrenal mass (indicated by an arrow) and right normal adrenal gland (indicated by an arrowhead) on plain CT. (b) An abdominal ultrasound. (c) A T1-weighted MRI (indicated by an arrow). (d) A T2- weighted MRI (indicated by an arrow). (e) SPAIR-MRI. (f) The gross features of the resected adrenal tumor. (g) The histological findings of the adjacent adrenal tissue, hematoxylin and eosin (H&E) staining  40. (h) The histological findings of the resected tumor, H&E staining  100. (i) The microscopic findings of the resected tumor composed of bronchial-epithelial cells, H&E staining  400. (j) Clinical course. SBP, systolic blood pressure; DBP, diastolic blood pressure; Serum K, serum potassium. A full color version of this figure is available at the Hypertension Research journal online.

In the present case, the levels and the rate of preoperative discovery of the left adrenalectomy preparation was were within the normal ranges, and the such tumors is only 0.1%.6,7 Likewise, performed with an alpha-blocker (phenoxy- imaging study and pathological workup bronchogenic cysts are often misdiagnosed benzamine), the authors reported that confirmed that the resected adrenal tumor as solid adrenal tumors because of their palpation during surgical exploration of the was benign. rich protein contents.3,8 In the present case, tumor immediately caused tachycardia The incidence of adrenal incidentalomas the CT and MRI results indicated little and hypertension, possibly mediated by has been increasing because of recent inclusion of adipose tissue in the solid a catecholamine release because of an advances in the availability of radiological tumor, while ultrasound was informative expanding mass or hemorrhage.10 Given devices. The CT attenuation value is infor- and differentiated a cystic composition in that hypertension and moderate hypo- mative for differentiating between benign and the adrenal region. kalemia were normalized after surgery in malignant adrenal tumors. A homogeneous The clinical hallmarks used as indicators our case, it is possible that a small amount mass with a smooth border and low HU to determine surgical intervention of of deviation of adrenocortical value (o10 HU) indicates a benign ade- adrenal incidentalomas are the initial size, contained in the tumor and/or those noma, except for lipid-poor adenomas.4 MRI growth rate and imaging characteristics induced by the compressed normal adrenal studies show benign adenomas as clear fat of the adrenal tumor and the endocrine was, at least somewhat, associated with the suppression of the signals by chemical-shift behavior.9 In the present case, the existence of preoperative hypertension. imaging,5 whereas adrenal metastasis is possibility of Cushing’s syndrome, pheo- Collectively, an accumulation of similar hypointense in T1-weighted images but chromocytoma and primary aldo- cases and pathophysiological investigations hyperintense in T2-weighted images. Note steronism was excluded preoperatively by are needed in future studies. The present that radiological examination occasionally endocrine data, including adrenal pressor case further emphasizes the need to consider uncovers various unexpected lesions that hormones and adrenal scintigrams. bronchogenic cysts as a differential diagnosis have originated from extra-adrenal tissues, However, considering the clinical course, for left adrenal incidentaloma with juvenile namely adrenal pseudotumors, which may the existence of the adrenal tumor may hypertension. mislead the diagnosis of tumors. Adrenal have been involved in the complication of 10 pseudotumors, such as gastric diverticulum, hypertension. Doggett et al. have reported a ACKNOWLEDGEMENTS splenic lobulation, renal, pancreatic or similar case of an adrenal bronchogenic cyst We thank Dr Tetsuro Sei and Dr Nanako Ogawa hepatic masses, and periadrenal varices, that was preoperatively considered to be for their constructive discussion regarding are found in B0.7% of incidentalomas, . Despite the fact that radiology.

Hypertension Research Correspondence 597

Tomohiro Terasaka1,2, Fumio Otsuka2, Dentistry and Pharmaceutical Sciences, washout characteristics on delayed enhanced CT. Kanako Ogura-Ochi1, Kitaku, Okayama, Japan Abdom Imaging 2003; 28: 709–715. 1 1 5 McDermott S, O’Connor OJ, Cronin CG, Blake MA. Tomoko Miyoshi , Kenichi Inagaki , E-mail: [email protected] Radiological evaluation of adrenal incidentalomas: Yasuyuki Kobayashi3, Yasutomo Nasu3 and current methods and future prospects. Best Pract Res Hirofumi Makino1 Clin Endocrinol Metab 2012; 26:21–33. 6 Gokan T, Ohgiya Y, Nobusawa H, Munechika H. 1 Commonly encountered adrenal pseudotumours on Department of Medicine and Clinical 1 Govaerts K, Van Eyken P, Verswijvel G, Van der Speeten K. CT. Br J Radiol 2005; 78:170–174. Science, Okayama University A bronchogenic cyst, presenting as a retroperitoneal cystic 7 Kodera R, Otsuka F, Inagaki K, Miyoshi T, Ogura T, Graduate School of Medicine, Dentistry and mass. Rare Tumors 2012; 4: e13. Tanimoto Y, Sei T, Makino H. Gastric diverticulum 2 O’Neal PB, Moore FD, Gawande A, Cho NL, King EE, simulating left adrenal incidentaloma in a hypertensive Pharmaceutical Sciences, Kitaku, Moalem J, Ruan D. Bronchogenic cyst masquerading patient. Endocr J 2007; 54: 969–974. 2 Okayama, Japan; Department of as an adrenal tumor: a case of mistaken identity. 8 Haddadin WJ, Reid R, Jindal RM. A retroperitoneal General Medicine, Okayama University Endocrine Pract 2012; 18: e102–e105. bronchogenic cyst: a rare cause of a mass in the 3 Yang DM, Jung DH, Kim H, Kang JH, Kim SH, Kim JH, adrenal region. J Clin Pathol 2001; 54: 801–802. Graduate School of Medicine, Dentistry and Hwang HY. Retroperitoneal cystic masses: CT, clinical, 9 Aron D, Terzolo M, Cawood TJ. Adrenal incidentalomas. Pharmaceutical Sciences, Kitaku, and pathologic findings and literature review. Radio- Best Pract Res Clin Endocrinol Metab 2012; 26: 69–82. Okayama, Japan and 3Department graphics 2004; 24: 1353–1365. 10 Doggett RS, Carty SE, Clarke MR. Retroperitoneal 4 Kebapci M, Kaya T, Gurbuz E, Adapinar B, Kebapci N, bronchogenic cyst masquerading clinically and radi- of Urology, Okayama Demirustu C. Differentiation of adrenal adenomas (lipid ologically as a phaeochromocytoma. Virchows Arch University Graduate School of Medicine, rich and lipid poor) from nonadenomas by use of 1997; 431:73–76.

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