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Case Report

iant Nonfunctioning Carcinoma ofhe Adrenal Cortex Mimicking Renalell Carcinoma: A Diagnostic Dilemma

zhar A. Khan, Iqbal S. Shergill, Rizwan Hamid, and Sandeep S. Gujral

drenocortical carcinoma is a rare, highly malignant neoplasm that originates in the adrenal cortex and is difficult toifferentiate from renal cell carcinoma, especially if it is gigantic and nonfunctional. We report the case of a 40-year-oldan with an incidental mass in the right upper abdomen. Magnetic resonance imaging revealed that the mass

riginated from the right kidney and was highly suggestive of renal cell carcinoma. However, histologic examinationfter radical nephrectomy confirmed the mass to be an adrenocortical carcinoma compressing the kidney. We discuss

he obscurity and implications of such a diagnosis. UROLOGY 70: 178.e1–178.e2, 2007. © 2007 Elsevier Inc.

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drenocortical carcinoma is a rare malignantgrowth, affecting only 1 or 2 persons per 1 mil-lion population, which originates in the cortex

f the adrenal gland.1 They are generally found at andvanced stage and are usually associated with poor prog-osis, especially when they are nonfunctioning. Weerein describe the case of a giant adrenocortical carci-oma that was diagnosed as renal cell carcinoma (RCC)reoperatively and review the literature to describe theifficulties in reaching a preoperative diagnosis in suchases.

ASE REPORT40-year-old man was incidentally found to have a largeass in the right upper abdomen. Magnetic resonance

maging (MRI) revealed the mass as originating from theight kidney (Fig. 1). It was highly suggestive of renal cellarcinoma (RCC). The case was further discussed in aultidisciplinary team meeting comprising urologists, ra-

iologists, and oncologists. Initial diagnosis made by theadiologist was maintained. There was no associatedymphadenopathy or distant metastases. The patient un-erwent a right radical nephrectomy, which showed a0 � 25 � 25-cm mass (Fig. 2). Peroperatively it seemedo originate from the kidney, and no distinction could beade with the adrenal gland. However, histologic exam-

nation confirmed the mass to be an adrenocortical car-inoma compressing the kidney, which had undergoneomplete atrophy.

The patient made a smooth postoperative recovery. Heas reviewed in light of histopathologic diagnosis. He didot show any clinical signs of adrenocortical disease. His

rom the Bristol Urological Institute, Bristol; and Harold Wood Hospital, Essex,nited KingdomAddress for correspondence: Azhar Khan, Bristol Urological Institute, Southmead

tospital, Bristol, United Kingdom. E-mail: docazhar@gmail.comSubmitted: October 12, 2006; accepted (with revisions): March 12, 2007

2007 Elsevier Inc.ll Rights Reserved

rinary and biochemical profile related to the adrenalland was within the normal range. He remains asymp-omatic 16 months after operation.

OMMENTdrenocortical carcinomas are rare cancers occurring in

he endocrine tissue of the adrenals. Approximately 80%f adrenal tumors are functional.2 Functional tumors areore common in children and young women, whereasonfunctional tumors occur more frequently in olderatients.3 It is uncertain whether nonfunctional tumorsre truly so or whether the metabolites are so low that

igure 1. Magnetic resonance imaging shows a mass aris-ng from the right kidney and occupying the right upperbdomen.

hey neither cause physiologic change nor can be picked

0090-4295/07/$32.00 178.e1doi:10.1016/j.urology.2007.03.038

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p by the available assays. Computerized tomographyCT) or MRI is used for diagnostic purposes. In small (3o 6-cm) lesions fine-needle biopsy can be performed. Ifhe aspirate is diagnostic then up to 95% of adrenalesions can be accurately diagnosed.4

Fimmano et al.5 reported a similar case of a largeonfunctioning adrenal carcinoma with a 24 � 18-cmass. Their patient had a preoperative fine-needle biopsy

uggestive of RCC without any clinical signs of adrenalisease. However, peroperatively the mass seemed to bef adrenal origin and was distinct from the kidney, whichas preserved. Kunieda et al.6 reported a patient in whomgiant adrenocortical carcinoma was found to have re-

urred in the contralateral adrenal gland and intrapelvicavity 6 years after his initial operation. They also con-luded that larger tumors are linked with better prognosisecause aggressive tumors give rise to distant metastasesnd therefore become symptomatic at an early stage.

Computerized tomography or MRI can be used foriagnosis of RCC. The radiologic diagnosis has a sensi-ivity of more than 90%. A fine-needle aspiration biopsys not recommended because this has a high false-nega-ive rate.7 Selective angiography and adrenal venographyan distinguish tumors of the adrenal gland from tumors

Figure 2. Mass after radical nephrectomy.

f the upper pole of the kidney.

78.e2

It is obviously important to differentiate between ad-enal and renal tumors preoperatively. Although radicalurgical excision can be curative for patients with local-zed malignancies, only 30% of these malignancies areonfined to the adrenal gland at the time of diagnosis.8 Ifhe diagnosis is known before surgery, CT and/or MRI ofhe abdomen can provide useful information about localnvasion or spread to the kidney. Positron emission to-ography may be effective in identifying unsuspected

ites of metastases.9 Even in the presence of a giantdrenocortical carcinoma, involvement of the kidney cane assessed during the operation, and attempts can beade to preserve the kidney.5

However, this differential from kidney tumors mightot always be possible. To our knowledge, there is noeported case of RCC greater than 20 cm. Hence, weelieve a high index of suspicion should be maintained inumors larger than 20 cm to make a correct diagnosis, toither save the kidney or be prepared to deal with adrenalathology both preoperatively and postoperatively.

eferences. Copeland PM: The incidentally discovered adrenal mass. Ann In-

tern Med 98: 940–945, 1983.. Luton JP, Cerdas S, Billaud L, et al: Clinical features of adrenocor-

tical carcinoma, prognostic factors, and the effects of mitotanetherapy. N Eng J Med 322: 1195–1201, 1990.

. Wooten MD, and King DK: Adrenal cortical carcinoma: epidemi-ology and treatment with mitotane and a review of the literature.Cancer 72: 3145–3155, 1993.

. Nguyen GU, and Akin MR: Fine needle aspiration cytology of thekidney. Ren Pelvis Adrenal Clin Lab Med 18: 429–459, 1998.

. Fimmano A, Pettinato G, Bonuso C, et al: Giant, non-functioningcarcinoma of the adrenal cortex. N Eng J Med 345: 700, 2001.

. Kunieda K, Saji S, Mori S, et al: Recurrence of giant adrenocorticalcarcinoma in the contralateral adrenal gland 6 years after surgery:report of a case. Surg Today 30: 294–297, 2000.

. Herts BR, and Baker ME: The current role of percutaneous biopsy inthe evaluation of renal masses. Semin Urol Oncol 13: 254–261,1995.

. Norton JA: Adrenal tumors, in DeVita VT Jr, Hellman S, andRosenberg SA (Eds): Cancer: Principles and Practice of Oncology, 7thed. Philadelphia, Lippincott Williams & Wilkins, 2005, pp 1528–1539.

. Becherer A, Vierhapper H, Pötzi C, et al: FDG-PET in adrenocor-

tical carcinoma. Cancer Biother Radiopharm 16: 289–295, 2001.

UROLOGY 70 (1), 2007