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Adrenocortical Carcinoma presenting with Cushing Syndrome A 63-year-old female with a long standing history of hypertension presented with shortness of breath, leg swelling, weight gain and worsening hypertension despite several months of treatment. She had been recently diagnosed with type II diabetes mellitus. Laboratory tests revealed elevated dehydroepiandrosterone and free urinary cortisol, and decreased plasma metanephrines. Clinical and laboratory findings were consistent with Cushing disease. Contrast enhanced computerized tomography (CT) of the abdomen showed a 5.5 cm left adrenal soft tissue, irregular enhancing mass with punc½F1 tuate and coarse calcifications (see figure). The differential diagnosis of an adrenal mass includes myelipoma, cyst, hemorrhage, hyperfunctioning and nonhyperfunctioning benign adenoma, pheochyromocytoma and adrenal cortical carcinoma.1 A hyperfunctioning adrenal tumor can either be an adrenal adenoma or adrenal cortical carcinoma arising from the adrenal cortex, producing
aldosterone (aldosteronoma), cortisol (Cushing disease) or steroid precursors (virilization), or a pheochromocytoma arising from the adrenal medulla. While metastases are the most common malignant lesion to involve the adrenal gland, they are rarely first identified in patients without a known primary malignancy.1 On average 55% of all primary adrenal tumors are functional, manifesting as Cushing syndrome, feminization, virilization or mixed Cushing syndrome and virilization. Hypertension is common in all clinical syndromes of adrenal origin. Patients are considered to have functional tumors if they have abnormal hormone levels of 17-hydroxycorticosteroids, 17-ketosteroids or cortisol in serum and/or urine basally and after dexamethasone suppression. Elevated levels of plasma testosterone, androstenedione and dehydroepiandrosterone sulfate, or plasma estradiol-17ß, aldosterone and 11-desoxycorticosterone are also suggestive of functional
Axial (A) and coronal (B) contrast enhanced CT images show left adrenal soft tissue mass (short fat arrow) with lobular margins, heterogeneous enhancement, and multiple punctate and coarse calcifications predominantly along posterior border (long thin arrow). Note suprarenal mass (arrow) with preserved fat plane between mass and left kidney (double ended arrow).
0022-5347/14/1913-0001/0 THE JOURNAL OF UROLOGY® © 2014 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION
AND
RESEARCH, INC.
http://dx.doi.org/10.1016/j.juro.2013.12.019 Vol. 191, 1-2, March 2014 Printed in U.S.A.
www.jurology.com Dochead: Radiology Page
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RADIOLOGY PAGE
adrenal tumors.2 Cross-sectional imaging with CT or magnetic resonance is the best means to identify an adrenal neoplasm with reported sensitivity for small adrenal lesions approaching 100%.3 Small adrenal tumors can be imaged without contrast simply to localize the mass, while for larger tumors intravenous contrast is recommended to assess for adrenal vein, inferior vena cava or adjacent organ involvement from a potential carcinoma. Primary carcinoma of the adrenal gland is rare, affecting approximately 1 to 2 patients/million.3,4 The tumor has a bimodal peak (1st and 4th decades), although the peak prevalence of this disease occurs in patients 30 to 70 years old. Tumors, particularly functioning tumors, tend to develop more often in females (65% to 90%) than males.4 Adrenocortical carcinoma (ACC) is typically a large mass at diagnosis, with the majority measuring more than 6 cm (range less than 4 cm to 25 cm). ACC may contain hemorrhagic or cystic components and calcifications. Calcifications are present in as many as 33% of cases, commonly microcalcifications. ACC is typically a unilateral suprarenal mass but it can be bilateral in as many as 10% of cases.4 There are no imaging features specific to adrenal cortical carcinoma. Tumors may be heterogeneous on unenhanced CT from necrosis. After contrast infusion, ACC enhances heterogeneously, often peripherally, with a thin rim of enhancing capsule. Common sites of metastases include the liver, lung and lymph nodes.4 ACC is heterogeneous on T1 and T2-weighted magnetic resonance images due to internal hemorrhage and necrosis. Hemorrhagic byproducts, principally methemoglobin, can result in areas of high signal intensity within the lesion on T1-weighted images and areas of necrosis have high signal intensity on T2-weighted images.5 Adrenocortical carcinoma can contain foci of intracytoplasmic lipid, which results in a loss of signal intensity on out-of-phase images, a feature commonly seen throughout a lipid rich adrenal adenoma. Larger
Dochead: Radiology Page
adrenal carcinomas may invade the adrenal vein and inferior vena cava, which is diagnostic of malignancy.5 ACC may be seen on ultrasound or fluorodeoxyglucose positron emission tomography scans. On ultrasound the appearance is variable, with larger tumors demonstrating heterogeneous echo texture, whereas on fluorodeoxyglucose positron emission tomography scans the tumor will show abnormal increased uptake.4 Patients with small primary adrenal lesions undergo tumor resection via laparoscopic adrenalectomy. Patients with larger tumors or tumors with vascular extension undergo open radical resection with resection of ipsilaterally involved adjacent structures. Prognosis is poor, with a mean survival of 18 months, which is longer in children. The 5-year survival rate for advanced disease is less than 30%. Early detection and surgical resection show good prognosis.2 Our patient underwent laparoscopic converted to open surgical resection of the adrenal mass due to poorly defined borders encountered on the laparoscopic approach. Final pathological examination confirmed adrenocortical carcinoma. Nidhi Sharma and Brian R. Herts Imaging Institute Cleveland Clinic Cleveland, Ohio 1. Song JH, Chaudhry FS and Mayo-Smith WW: The incidental adrenal mass on CT: prevalence of adrenal disease in 1049 consecutive adrenal masses in patients with no known malignancy. AJR Am J Roentgenol 2008; 190: 1163. 2. Luton JP, Cerdas S, Billaud L et al: Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med 1990; 322: 1195. 3. Stifelman MD and Fenig DM: Work-up of the functional adrenal mass. Curr Urol Rep 2005; 6: 63. 4. Johnson PT, Horton KM and Fishman EK: Adrenal mass imaging with multidetector CT: pathologic conditions, pearls, and pitfalls. RadioGraphics 2009; 29: 1333. 5. Elsayes KM, Mukundan G, Narra VR et al: Adrenal masses: MR imaging features with pathologic correlation. RadioGraphics, suppl., 2004; 24: S73.
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Adrenocortical Carcinoma presenting with Cushing Syndrome A 63-year-old female with a long standing history of hypertension presented with shortness of breath, leg swelling, weight gain and worsening hypertension despite several months of treatment. She had been recently diagnosed with type II diabetes mellitus. Laboratory tests revealed elevated dehydroepiandrosterone and free urinary cortisol, and decreased plasma metanephrines. Clinical and laboratory findings were consistent with Cushing disease. Contrast enhanced computerized tomography (CT) of the abdomen showed a 5.5 cm left adrenal soft tissue, irregular enhancing mass with punc½F1 tuate and coarse calcifications (see figure). The differential diagnosis of an adrenal mass includes myelipoma, cyst, hemorrhage, hyperfunctioning and nonhyperfunctioning benign adenoma, pheochyromocytoma and adrenal cortical carcinoma.1 A hyperfunctioning adrenal tumor can either be an adrenal adenoma or adrenal cortical carcinoma arising from the adrenal cortex, producing
aldosterone (aldosteronoma), cortisol (Cushing disease) or steroid precursors (virilization), or a pheochromocytoma arising from the adrenal medulla. While metastases are the most common malignant lesion to involve the adrenal gland, they are rarely first identified in patients without a known primary malignancy.1 On average 55% of all primary adrenal tumors are functional, manifesting as Cushing syndrome, feminization, virilization or mixed Cushing syndrome and virilization. Hypertension is common in all clinical syndromes of adrenal origin. Patients are considered to have functional tumors if they have abnormal hormone levels of 17-hydroxycorticosteroids, 17-ketosteroids or cortisol in serum and/or urine basally and after dexamethasone suppression. Elevated levels of plasma testosterone, androstenedione and dehydroepiandrosterone sulfate, or plasma estradiol-17ß, aldosterone and 11-desoxycorticosterone are also suggestive of functional
Axial (A) and coronal (B) contrast enhanced CT images show left adrenal soft tissue mass (short fat arrow) with lobular margins, heterogeneous enhancement, and multiple punctate and coarse calcifications predominantly along posterior border (long thin arrow). Note suprarenal mass (arrow) with preserved fat plane between mass and left kidney (double ended arrow).
0022-5347/14/1913-0001/0 THE JOURNAL OF UROLOGY® © 2014 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION
AND
RESEARCH, INC.
http://dx.doi.org/10.1016/j.juro.2013.12.019 Vol. 191, 1-2, March 2014 Printed in U.S.A.
www.jurology.com Dochead: Radiology Page
SCO 5.2.0 DTD JURO10971_proof 13 December 2013 3:51 pm EO:
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RADIOLOGY PAGE
adrenal tumors.2 Cross-sectional imaging with CT or magnetic resonance is the best means to identify an adrenal neoplasm with reported sensitivity for small adrenal lesions approaching 100%.3 Small adrenal tumors can be imaged without contrast simply to localize the mass, while for larger tumors intravenous contrast is recommended to assess for adrenal vein, inferior vena cava or adjacent organ involvement from a potential carcinoma. Primary carcinoma of the adrenal gland is rare, affecting approximately 1 to 2 patients/million.3,4 The tumor has a bimodal peak (1st and 4th decades), although the peak prevalence of this disease occurs in patients 30 to 70 years old. Tumors, particularly functioning tumors, tend to develop more often in females (65% to 90%) than males.4 Adrenocortical carcinoma (ACC) is typically a large mass at diagnosis, with the majority measuring more than 6 cm (range less than 4 cm to 25 cm). ACC may contain hemorrhagic or cystic components and calcifications. Calcifications are present in as many as 33% of cases, commonly microcalcifications. ACC is typically a unilateral suprarenal mass but it can be bilateral in as many as 10% of cases.4 There are no imaging features specific to adrenal cortical carcinoma. Tumors may be heterogeneous on unenhanced CT from necrosis. After contrast infusion, ACC enhances heterogeneously, often peripherally, with a thin rim of enhancing capsule. Common sites of metastases include the liver, lung and lymph nodes.4 ACC is heterogeneous on T1 and T2-weighted magnetic resonance images due to internal hemorrhage and necrosis. Hemorrhagic byproducts, principally methemoglobin, can result in areas of high signal intensity within the lesion on T1-weighted images and areas of necrosis have high signal intensity on T2-weighted images.5 Adrenocortical carcinoma can contain foci of intracytoplasmic lipid, which results in a loss of signal intensity on out-of-phase images, a feature commonly seen throughout a lipid rich adrenal adenoma. Larger
Dochead: Radiology Page
adrenal carcinomas may invade the adrenal vein and inferior vena cava, which is diagnostic of malignancy.5 ACC may be seen on ultrasound or fluorodeoxyglucose positron emission tomography scans. On ultrasound the appearance is variable, with larger tumors demonstrating heterogeneous echo texture, whereas on fluorodeoxyglucose positron emission tomography scans the tumor will show abnormal increased uptake.4 Patients with small primary adrenal lesions undergo tumor resection via laparoscopic adrenalectomy. Patients with larger tumors or tumors with vascular extension undergo open radical resection with resection of ipsilaterally involved adjacent structures. Prognosis is poor, with a mean survival of 18 months, which is longer in children. The 5-year survival rate for advanced disease is less than 30%. Early detection and surgical resection show good prognosis.2 Our patient underwent laparoscopic converted to open surgical resection of the adrenal mass due to poorly defined borders encountered on the laparoscopic approach. Final pathological examination confirmed adrenocortical carcinoma. Nidhi Sharma and Brian R. Herts Imaging Institute Cleveland Clinic Cleveland, Ohio 1. Song JH, Chaudhry FS and Mayo-Smith WW: The incidental adrenal mass on CT: prevalence of adrenal disease in 1049 consecutive adrenal masses in patients with no known malignancy. AJR Am J Roentgenol 2008; 190: 1163. 2. Luton JP, Cerdas S, Billaud L et al: Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med 1990; 322: 1195. 3. Stifelman MD and Fenig DM: Work-up of the functional adrenal mass. Curr Urol Rep 2005; 6: 63. 4. Johnson PT, Horton KM and Fishman EK: Adrenal mass imaging with multidetector CT: pathologic conditions, pearls, and pitfalls. RadioGraphics 2009; 29: 1333. 5. Elsayes KM, Mukundan G, Narra VR et al: Adrenal masses: MR imaging features with pathologic correlation. RadioGraphics, suppl., 2004; 24: S73.
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