Introduction
Background
Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis.
A 68-year-old woman with a large right upper quadrant primary adrenocortical carcinoma with curvilinear calcification. Low-attenuation regions anteriorly are consistent with necrosis.
T2-weighted MRI demonstrates a large right adrenocortical carcinoma with high signal intensity involving the right lateral aspect.
The reported incidence of adrenal carcinoma is 2 cases per million persons. When identified, tumors frequently are large, measuring 4-10 cm in cross-sectional diameter. Adrenal carcinomas arise from the adrenal cortex and are bilateral in up to 10% of patients. Approximately 50-80% are functional tumors, with most causing Cushing syndrome.
In one large patient-population study in the United States, 3982 patients diagnosed with ACC from 1985 to 2005 were identified from the National Cancer Data Base (NCDB), and tumor, treatment, and hospital factors associated with survival after resection were examined. Of the patients with nodes examined, 26.5% had nodal metastases, and distant metastases were found in 21.6% of patients. Overall 5-year survival for all patients who underwent resection was 38.6% (median survival, 31.9 months). A higher risk of death was associated with increasing age, poorly differentiated tumors, involved margins, and nodal or distant metastases.1
Pathophysiology
The adrenal glands reside within the Gerota fascia. The cortex and medulla comprise the 2 functional components of the adrenal gland; anatomically, the gland is composed of a body and lateral and medial limbs.
Frequency
United States
Adrenal carcinoma has an incidence of 1 case in 1,700,000 adults. Adrenal carcinoma accounts for 0.002% of childhood malignancies.
Mortality/Morbidity
Adrenal carcinoma accounts for 0.02% of malignant tumors and 0.2% of cancer deaths.
Race
Adrenocortical carcinoma has no racial predilection.
Sex
Male-to-female ratio is approximately 1:1; however, functional tumors are slightly more common in women, while nonfunctional tumors are slightly more common in men. Male patients with adrenocortical carcinoma often tend to be older than female patients and often have a worse prognosis.
Age
Adrenocortical carcinoma has a bimodal occurrence, with the first peak in the first decade of life and the second peak in the fourth to fifth decades of life.
Although adrenocortical carcinoma is rare in adults, it is even more uncommon in children, comprising only 0.002% of childhood malignancies. In children, adrenal carcinoma is rare and is responsible for only 6% of adrenal tumors, although it is the most common adrenal cortical cancer. In children, 90% of adrenal masses are neuroblastomas (adrenal medulla). Adrenal carcinomas in children are associated with hemihypertrophy and Beckwith-Wiedemann syndrome.2,3,4
Anatomy
The adrenal gland is composed of the main body, a lateral limb, and a medial limb and often has a characteristic inverted Y, V, or T shape. On CT, the limbs should demonstrate a thickness no greater than that of the adjacent diaphragmatic crus. The right adrenal gland lies anteromedial to the superior pole of the right kidney in a space bordered by the inferior vena cava, liver, and right crus of the diaphragm. The left adrenal gland lies more caudal than the right adrenal gland. It is located anteromedial to the superior pole of the left kidney in a space bordered by the left crus of the diaphragm and splenic vein and is posterior to the pancreas. The adrenal gland is made up of the cortex and the medulla, which are derived from the mesoderm and the neural crest, respectively. The adrenal cortex secrets cortisol, aldosterone, and androgens, while the adrenal medulla secretes epinephrine and norepinephrine.
Arterial supply of the adrenal gland arises from 3 main vessels. The superior adrenal artery originates in the inferior phrenic artery, the middle adrenal artery branches directly off the aorta, and the inferior adrenal artery is a branch of the renal artery.
Venous drainage is simpler, with the adrenal vein entering directly into the vena cava on the right. On the left, the adrenal vein enters into the inferior phrenic vein before exiting into the left renal vein.
Presentation
Patients usually present with a large palpable mass, abdominal pain, or Cushing syndrome.5 Primary adrenocortical carcinomas are large tumors usually measuring more than 5 cm at presentation. Because they are large, the organ of origin often is difficult to determine (Image 1).6,7,8,9
A 68-year-old woman with a large right upper quadrant primary adrenocortical carcinoma with curvilinear calcification. Low-attenuation regions anteriorly are consistent with necrosis.
Cushing syndrome is the most common clinical presentation in adults with adrenal cortical carcinoma, although Cushing syndrome results from adrenal carcinoma in only 5-10% of patients. Conversely, Cushing syndrome in childhood usually is caused by adrenocortical carcinoma. Clinical presentation depends on whether the tumor is functional. In addition to Cushing syndrome, patients can present with virilization, feminization, precocious puberty, or Conn syndrome, although these manifestations are rare. In children, the most common clinical presentation is virilization, followed by Cushing syndrome.
Functional tumors more often are detected in female patients than in male patients. They often are detected earlier than nonfunctional tumors because of the secretion of hormones.
The following endocrine syndromes are associated with adrenocortical carcinoma:
- Cushing syndrome
- Virilization and precocious puberty
- Feminization
- Primary hyperaldosteronism
Preferred Examination
CT is the study of choice in the evaluation of an abdominal mass or, more precisely, to differentiate a benign adrenal mass from a malignant lesion.10,11,12,13
Limitations of Techniques
Although CT is used most widely for evaluating abdominal masses, the origin of the mass often is difficult to discern. In addition, the presence or absence of invasion of adjacent structures is difficult to determine in some patients.
Differential Diagnoses
Other Problems to Be Considered
Metastatic disease
More on Adrenal Carcinoma |
 Overview: Adrenal Carcinoma |
| Imaging: Adrenal Carcinoma |
| Follow-up: Adrenal Carcinoma |
| Multimedia: Adrenal Carcinoma |
| References |
| Further Reading |
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References
Bilimoria KY, Shen WT, Elaraj D, Bentrem DJ, Winchester DJ, Kebebew E, et al. Adrenocortical carcinoma in the United States: treatment utilization and prognostic factors. Cancer. Dec 1 2008;113(11):3130-6. [Medline].
Hishiki T, Kazukawa I, Saito T, Terui K, Mitsunaga T, Nakata M, et al. Diagnosis of adrenocortical tumor in a neonate by detection of elevated blood 17-hydroxyprogesterone measured as a routine neonatal screening for congenital adrenal hyperplasia: a case report. J Pediatr Surg. Oct 2008;43(10):e19-22. [Medline].
Agrons GA, Lonergan GJ, Dickey GE, Perez-Monte JE. Adrenocortical neoplasms in children: radiologic-pathologic correlation. Radiographics. Jul-Aug 1999;19(4):989-1008. [Medline].
Kay R, Schumacher OP, Tank ES. Adrenocortical carcinoma in children. J Urol. Dec 1983;130(6):1130-2. [Medline].
McNicol AM. A diagnostic approach to adrenal cortical lesions. Endocr Pathol. Winter 2008;19(4):241-51. [Medline].
Boushey RP, Dackiw AP. Adrenal cortical carcinoma. Curr Treat Options Oncol. Aug 2001;2(4):355-64. [Medline].
Dackiw AP, Lee JE, Gagel RF, Evans DB. Adrenal cortical carcinoma. World J Surg. Jul 2001;25(7):914-26. [Medline].
Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol. Jan 2003;169(1):5-11. [Medline].
Vierhapper H. Adrenocortical tumors: clinical symptoms and biochemical diagnosis. Eur J Radiol. Feb 2002;41(2):88-94. [Medline].
Chien HP, Chang YS, Hsu PS, Lin JD, Wu YC, Chang HL, et al. Adrenal cystic lesions: a clinicopathological analysis of 25 cases with proposed histogenesis and review of the literature. Endocr Pathol. Winter 2008;19(4):274-81. [Medline].
Lockhart ME, Smith JK, Kenney PJ. Imaging of adrenal masses. Eur J Radiol. Feb 2002;41(2):95-112. [Medline].
Maurea S, Mainolfi C, Bazzicalupo L, et al. Imaging of adrenal tumors using FDG PET: comparison of benign and malignant lesions. AJR Am J Roentgenol. Jul 1999;173(1):25-9. [Medline].
Mayo-Smith WW, Boland GW, Noto RB, Lee MJ. State-of-the-art adrenal imaging. Radiographics. Jul-Aug 2001;21(4):995-1012. [Medline].
Gross MD, Gauger PG, Djekidel M, Rubello D. The role of PET in the surgical approach to adrenal disease. Eur J Surg Oncol. Feb 23 2009;[Medline].
Soga H, Takenaka A, Ooba T, Nakano Y, Miyake H, Takeda M, et al. A twelve-year experience with adrenal cortical carcinoma in a single institution: long-term survival after surgical treatment and transcatheter arterial embolization. Urol Int. 2009;82(2):222-6. [Medline].
Allolio B, Hahner S, Weismann D, Fassnacht M. Management of adrenocortical carcinoma. Clin Endocrinol (Oxf). Mar 2004;60(3):273-87. [Medline].
Cirillo RL Jr, Bennett WF, Vitellas KM, et al. Pathology of the adrenal gland: imaging features. AJR Am J Roentgenol. Feb 1998;170(2):429-35. [Medline].
Dunnick NR. Adrenal carcinoma. Radiol Clin North Am. Jan 1994;32(1):99-108. [Medline].
Dunnick NR. Hanson lecture. Adrenal imaging: current status. AJR Am J Roentgenol. May 1990;154(5):927-36. [Medline].
Fishman EK, Deutch BM, Hartman DS, et al. Primary adrenocortical carcinoma: CT evaluation with clinical correlation. AJR Am J Roentgenol. Mar 1987;148(3):531-5. [Medline].
Hutter AM Jr, Kayhoe DE. Adrenal cortical carcinoma. Clinical features of 138 patients. Am J Med. Oct 1966;41(4):572-80. [Medline].
Further Reading
Clinical guidelines
ACR Appropriateness Criteria® incidentally discovered adrenal mass.
American College of Radiology - Medical Specialty Society. 2000 (revised 2007). 8 pages. NGC:005995
The diagnosis of Cushing's syndrome: an Endocrine Society clinical practice guideline.
The Endocrine Society - Disease Specific Society. 2008. 29 pages. NGC:006662
Clinical trials
Antineoplaston Therapy in Treating Patients With Stage IV Adrenal Gland Cancer
Study of Adrenal Gland Tumors
Adrenal Tumors - Pathogenesis and Therapy
Related eMedicine topics
Adrenal Carcinoma (Oncology)
Adrenal Carcinoma (Pediatrics: General Medicine)
Adrenal Surgery
Cushing Syndrome
Li-Fraumeni Syndrome
Keywords
adrenal carcinoma, adrenal cancer, adrenal gland carcinoma, adrenal gland cancer, cancer of the adrenal gland, carcinoma of the adrenal gland
