Pediatric Case of the Day Annual Meeting, 1986
Robert A. Kaufman, M.D. Richard B. Towbin, M.D.
William S. Ball, M.D.
History: This 7 year and 4 month old white boy had mild, acute gastro- enteritis symptoms about one month prior to admission. He gradually improved although he still had complaints of generalized malaise and poor appetite. On followup exami- nation, his liver was thought to be enlarged. His monospot test was negative. His “liver enlargement” persisted. The physical examination was otherwise normal. Survey radiography before (Figure 1) and after the administration of contrast media for CT examination (Figure 2), real time sonography (Figure 3) and computed tomography (Figure 4) were performed.
From the Department of Radiology, University of Cincinnati College of Medicine and Division of Radiology, Children’s Hos- pital Medical Center, Cincinnati, Ohio.
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Figure 4A
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Findings
There is a large, round, encapsulated mass measuring 12.5 x 10.5 x 11 cm. Its margin with the liver is well defined as is its margin with the kid- ney. The right kidney is displaced and pan- caked, but not invaded (Figures 3 and 4). The enhancement characteristics of the lesion are interesting. Prior to the administration of an intra- venous contrast medium (Figure 4A), the lesion is heterogeneous internally. During bolus injec- tion of the contrast medium and dynamic scan- ning with table incrementation (Figures 4B and C), large vessels in the periphery enhance while
draining toward the center of the lesion. These are likely venous structures. The attenuation of the center of the lesion is low, probably repre- senting necrosis. After an equilibrium phase has been reached, the enhancement of the periph- ery and vasculature rapidly fades, although de- marcation of the necrotic central portion re- mains striking (Figure 4D). The relationship of the mass to the liver and right kidney, together with its sharply defined margins, suggests a supra- renal lesion of adrenal origin.
Diagnosis: Adrenocortical carcinoma
Discussion
Adrenocortical carcinoma is a rare tumor with an annual incidence of 0.2 per 100,000. It can occur at any age, with the greatest incidence be- tween 40 and 70 years. Most such tumors are functioning. Hormone producing tumors occur more often in women than in men, while nonhor- mone producing tumors have a male predominance.
Adrenocortical carcinoma must be differentiated from benign adenomas. Histologi- cally, adrenocortical car- cinomas may have a wide spectrum of appearances. Even highly aggressive tumors may be well encapsulated.
Adrenocortical carcinoma presents clini- cally in two ways: 1. Hormonal presentation in- cludes Cushing’s syndrome, virilization in females, feminization in males, hypertension, sodium retention and hypokalemic alkalosis. Uri- nary 17-ketosteroid is frequently increased. 2. Nonhormonal presentation usually consists of abdominal mass, pain and less frequently, weight loss, weakness and fatigue. Adrenocorti- cal carcinoma spreads most commonly locally, but it does metastasize to lymph nodes, liver, lung and, occasionally, bone.
This patient had the nonhormonal form of the disease. In a large review of such tumors by Lewinsky, only 9 of 178 cases were less than 20 years of age. The overall prognosis of nonhor- monal adrenal cortical carcinoma was poor, with
a 67% mortality in the first year. More than half of these deaths occurred within two months of diagnosis. The age and sex of the patient did not seem to influence the prognosis.
Treatment of adrenocortical carcinoma is mainly surgical excision. Adjuvant chemotherapy or radiotherapy or both are reserved for meta- static disease, when success is poor. Our patient has had complete removal of his tumor, and has been carefully reevaluated every two months since he was first seen earlier this year.
The CT appearance of a large adrenal mass with central necrosis is typical of ad- renocortical carcinoma, but it can occasionally be seen with pheochromocytoma or a large metastasis to the adrenal gland. It is not at all typical of neuroblastoma.
Suggested Readings
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3. Dunnick NR, Heaston D, Halvorsen R, Moore AV, Korobkin M. CT appearance of adrenal cortical carcinoma. J Comput Assist Tomogr 1982; 6:978-982.
4. Hutter AM Jr., Kayhoe DE. Adrenal cortical carcinoma: Clinical features of 138 patients. Am J Med 1966; 41:572- 580.
5. Lewinsky BS, Grigor KM, Symington T, et al. The clinical and pathologic features of “non-hormonal” adrenocortical tumors: Report of twenty new cases and review of the literature. Cancer 1974; 33:778-790.
6. Scott HW, et al. Tumors of the adrenal cortex and Cush- ing’s syndrome. Ann Surg 1985; 201:587-594.
7. Sefczek DM, Bowen AD. Nonhormonal adrenocortical car- cinoma. Am J Dis Child 1982; 136:163-165.
8. Slotten H, et al. Morphologic characteristics of benign and malignant adrenocortical tumors. Cancer 1985; 55:766-773.
9. Stewart DR, Jones PH, Jolleys A. Carcinoma of the adrenal gland in children. J Pediatr Surg 1974; 9:59-67.
The authors wish to thank Ms. Juanita Hunter for manuscript preparation and adminis- trative support.