Radiological Case of the Month
Donna M. Sefczek, MD; A’Delbert Bowen, MD (Contributors); Lionel W. Young, MD (Section Editor)
A 5-year 11-month-old girl had a rapidly enlarging and tender right upper-quadrant mass. She was a
Contributed from the Department of Radiolo- gy, Children’s Hospital of Pittsburgh, University of Pittsburgh Medical School.
Reprint requests to Department of Radiology, Children’s Hospital of Pittsburgh, 125 DeSoto St, Pittsburgh, PA 15213 (Dr L. W. Young).
thin, cheerful child in no apparent distress. The mass extended 10 cm below the right costal margin and 2 cm across the midline. There were no clinical or laboratory signs of hor- monal abnormality. Plain films of the abdomen were obtained (Fig 1), and intravenous (IV) urography was per- formed (Fig 2).
Denouement and Discussion Nonhormonal Adrenocortical Carcinoma
Fig 1 .- Anteroposterior abdominal roentgenogram shows calcific shadows in right suprarenal region.
Fig 2 .- Excretory urogram; right kidney is displaced inferomedially, and collecting system is distorted (arrows).
Fig 3 .- Resected specimen viewed from posterior aspect. Tumor covers ventral (anterior) surface of kidney.
Fig 4 .- Same perspective with kidney reflected off tumor. Note cut surface of superior pole of tumor and altered appearance of kidney surface caused by pressure of tumor. Kidney was not invaded.
Fig 5 .- Large, spherical (“cannonball”) pulmonary metastases.
A suprarenal mass can displace the kidney and occasionally may distort its intrarenal urographic appearance. Marked compression and deformity of the kidney as seen on this patient’s urogram suggest an intrarenal mass, but dense calcification (Fig 1) is unu- sual in Wilms’ tumor’ and other intrarenal masses in children. Of adrenal tumors, calcification is most common in neuroblastoma but can be seen with adenoma, carcinoma, or dermoid tumors.2 Adrenal calcifica- tion also occurs idiopathically, follow- ing adrenal hemorrhage, and in tuber- culosis, Wolman’s disease, and un- commonly in other nonneoplastic con- ditions.2-4
Angiography demonstrated tumor vascularity from the right adrenal artery and probably from capsular branches of the right renal artery. Much of the mass was relatively avas- cular.
The patient underwent laparotomy, right nephrectomy, and excision of a well-encapsulated mass 15 cm in di- ameter (Figs 3 and 4). The kidney was adherent to and compressed by the tumor. Despite the angiographic find- ings and the presence of tumor nodules at the renal hilum, the kidney parenchyma was not invaded. The mass was also adherent to the inferior hepatic surface. Pathologic diagnosis was adrenocortical carcinoma.
The child received postoperative irradiation and chemotherapy on an ovarian carcinoma treatment proto- col. Six months after surgery, rounded pulmonary metastatic nodules were present (Fig 5). They did not respond to a trial of hexamethylmelamine.5,6 Death from progressive pulmonary insufficiency occurred 18 months af- ter surgery.
Adrenocortical carcinomas are un- common. Most produce hormonally
active substances and clinical signs of masculinization or feminization, Conn’s syndrome, or Cushing’s syn- drome.7 However, because our patient had no evidence of endocrine dysfunc- tion, her tumor was considered non- hormonal. Lewinsky et al7 in 1974 reviewed 178 cases of nonhormonal adrenocortical carcinoma of which only nine occurred in patients less than 20 years of age. We found only one additional description of a pediat- ric patient in the English-language literature since 1974.8
Some children who initially appear to have nonfunctioning carcinomas begin to show endocrine signs late in the course of their disease. The patient of Marsden et al9 only showed signs of Cushing’s syndrome shortly before her death; another child had only hypertension preoperatively but had elevated urinary steroid excretion after removal of the tumor.10
10
Although “nonhormonal” carcino- mas do not produce hormonally active compounds, Fukushima and Galla- gher11 showed that they do secrete pregnenolone. An enzymatic defect probably accounts for pregnenolone’s nonconversion to hormonally active forms. Metabolic products of pregnen- olone detected in the urine may aid in diagnosis and in gauging the effec- tiveness of treatment.
A palpable mass is the most com- mon clinical feature, but patients may also have pain, weakness, anorexia, or, uncommonly, fever.7,8
The radiologic examination of most value has traditionally been the IV urogram, which may show a supra- renal mass displacing and occasional- ly deforming the kidney. However, ultrasound and computed tomography are excellent methods for imaging adrenal masses and their surrounding structures.12-14 Iodocholesterol I 131 radionuclide scintigraphy may show these tumors15 but it entails substan- tial radiation (20 to 49 rad/mCi) to the adrenals and ovaries.16 Arteriography and lymphangiography may aid in preoperative planning.
Nonhormonal adrenocortical carci- noma carries a poor prognosis with a 69% mortality in the first year, more than half the deaths occurring within two months of diagnosis. Neither age nor sex influence survival.7 Treatment consists primarily of resecting the neoplastic mass. Irradiation and chemotherapy produce inconsistent results,7 although mitotane has been used with some success.17 Hexameth- ylmelamine has shown promise in clinical trials for a variety of neo- plasms5.6 but produced no response in our patient.
References
1. Cope JR: Radiographic, angiographic, and radiokinetic findings in Wilms’ tumors, in Pochedly C, Miller D (eds): Wilms’ Tumor. New York, John Wiley & Sons Inc, 1976, pp 31-53.
2. Reeder M, Felson B: Gamuts in Radiology. Cincinnati, Audiovisual Radiology of Cincinnati Inc, 1975, p H-45.
3. Young LW, Sty JR, Babbit DP: Radiological case of the month: Wolman’s disease. Am J Dis Child 1979;133:959-960.
4. Schullinger JN, Santulli TV, Berdon WE, et al: Calcific thrombi of the inferior vena cava in infants and children. J Pediatr Surg 1978;13:429- 434.
5. Longacre D, Donovan M, Paladine W, et al: Hexamethylmelamine, vincristine, and metho- trexate chemotherapy in advanced neoplasms. Cancer Treat Rep 1977;61:919-922.
6. Legha SS, Slavik M, Carter SK: Hexameth- ylmelamine: An evaluation of its role in the therapy of cancer. Cancer 1976;38:27-35.
7. Lewinsky BS, Grigor KM, Symington T, et al: The clinical and pathologic features of ‘non- hormonal’ adrenocortical tumors. Cancer 1974;33:778-790.
8. Constantopoulos A, Karpouzas J, Xypolita A, et al: Nonhormonal case of adrenal cortical carcinoma. Arch Dis Child 1978;53:827.
9. Marsden HB, Morris Jones P, Lees PD, et al: Late functioning adrenocortical carcinoma in a 5-year-old girl. Arch Dis Child 1978;53:341-342.
10. Visconti EB, Peters RW, Cangir A, et al: Unusual case of adrenal cortical carcinoma in a female infant. Arch Dis Child 1978;53:342-344.
11. Fukushima DK, Gallagher TF: Steroid pro- duction in ‘nonfunctioning’ adrenal cortical tumor. J Clin Endocrinol Metab 1963;23:923-927.
12. Bernardino ME, Goldstein HM, Green B: Gray scale ultrasonography of adrenal neo- plasms. AJR 1978;130:741-744.
13. Sample WF, Sarti DA: Computed tomogra- phy and gray scale ultrasonography of the adre-
nal gland: A comparative study. Radiology 1978;128:377-383.
14. Eghrari M, McLoughlin MJ, Rosen IE, et al: The role of computed tomography in assess- ment of tumoral pathology of the adrenal glands. J Comput Assist Tomogr 1980;4:71-77.
15. Rizza RA, Wahner HW, Spelsberg TC, et al: Visualization of nonfunctioning adrenal ade- nomas with iodocholesterol: Possible relation- ship to subcellular distribution of tracer. J Nucl Med 1978;19:458-463.
16. Bossuyt A, Somers G: 1311-19-Iodocholes- terol visualization of an adrenal carcinoma with- out clinical manifestations. J Nucl Biol Med 1975;19:225-227.
17. Hogan TF, Citrin DL, Johnson BM, et al: o,p’-DDD(mitotane) therapy of adrenal cortical carcinoma: Observations on drug dosage, toxici- ty, and steroid replacement. Cancer 1978;42:2177- 2181.