CONTINUING EDUCATION
From the
LEARNING
OBJECTIVES
After reading this article
and taking the test, the
reader will:
• Be familiar with the
spectrum of clinical, path-
ologic, and radiologic
findings in children with
adrenocortical neoplasms.
• Recognize the differ-
ences in clinical presenta-
tion and biologic behavior
between adrenocortical
tumors in children and
similar lesions in adults.
• Understand the role of
imaging studies in diag-
nosis and clinical staging
of adrenocortical neo-
plasms in children.
Archives of the AFIP
Adrenocortical Neoplasms in Children:
Radiologic-Pathologic Correlation1
Geoffrey A. Agrons, MD • Gael J. Lonergan, Lt Col, USAF, MC • Glenn E. Dickey, Lt
Col, USAF, MC • Juan E. Perez-Monte, MD
Primary neoplasms of the adrenal cortex are rare in children and differ signifi-
cantly in epidemiology, clinical characteristics, and biologic features from their
counterparts in adults. In children, the inclusive term adrenocortical neo-
plasm is applied because adrenal adenoma and adrenal carcinoma may be dif-
ficult to distinguish histopathologically. Pediatric adrenocortical neoplasms
typically occur before 5 years of age, affect young girls more commonly than
boys, and are associated with hemihypertrophy and Beckwith-Wiedemann and
Li-Fraumeni syndromes. Most children with an adrenocortical neoplasm pres-
ent with signs and symptoms of endocrine abnormality, including virilization
and Cushing syndrome. Cross-sectional imaging studies typically demonstrate a
large, circumscribed, predominantly solid suprarenal mass with variable het-
erogeneity due to hemorrhage and necrosis. Calcification is not uncommon.
Local invasion and metastases to the lungs, liver, and regional lymph nodes
may be present at diagnosis. When friable tumor thrombus extends into the
inferior vena cava, it poses a high risk of pulmonary embolization. The finding
of increased retroperitoneal fat due to hypercortisolism on computed tomo-
graphic and magnetic resonance images of children with an adrenal mass fa-
vors the diagnosis of adrenocortical neoplasm. Surgical resection is the main-
stay of therapy, with chemotherapy used for patients with metastases or persis-
tent elevated hormone levels following surgery. Patients younger than 5 years
with aggressive adrenocortical neoplasms fare better than older children.
Index terms: Adrenal gland, neoplasms, 862.30 • Children, genitourinary system, 862.30 • Infants, genitourinary sys-
tem, 862.30 • Neoplasms, in infants and children, 862.30
RadioGraphics 1999; 19:989–1008
1From the Departments of Radiology, Pennsylvania Hospital, 800 Spruce St, Philadelphia, PA, 19107 (G.A.A., J.E.P-M.);
Radiologic Pathology (G.J.L.) and Pediatric Pathology (G.E.D.), Armed Forces Institute of Pathology, Washington, DC;
Radiology, Children’s Hospital of Philadelphia (G.A.A.), Penn; and Radiology and Nuclear Medicine, Uniformed Services
University of the Health Sciences, Bethesda, Md (G.J.L.). Received February 4, 1999; revision requested March 3 and re-
ceived April 14; accepted April 15. Address reprint requests to G.A.A.
The opinions and assertions contained herein are the private views of the authors and are not to be construed as official
nor as reflecting the views of the Departments of the Air Force or Defense.
© RSNA, 1999
989
 ■ INTRODUCTION
Neoplasms of the adrenal cortex are rare in
childhood. Unlike similar tumors in adults, adre-
nal adenomas in children have no histopatho-
logic features that allow them to be reliably dis-
tinguished from carcinomas. In addition, the
biologic behavior of pediatric adrenocortical
neoplasms may be difficult to predict on the
basis of morphologic criteria. Thus, the term
adrenocortical neoplasm is currently used to
designate both benign and malignant tumors of
the adrenal cortex in children. Adrenocortical
neoplasms of childhood, particularly those oc-
curring in infants, merit separate discussion
from their counterparts in adults because they
have distinctive epidemiologic and clinical fea-
tures (1).
Each year, an estimated 25 adrenocortical
neoplasms occur in patients younger than 20
years of age, representing an annual incidence
rate of three per million (2). Of adrenal tumors
in children, adrenocortical neoplasms are far
less common than neuroblastomas but more
common than pheochromocytomas (3). De-
spite their relative rarity, adrenocortical neo-
plasms represent the most common tumor of
the pediatric adrenal cortex (4,5).
The hormonal activity of childhood adrenal
neoplasms has been recognized since 1948,
when Wilkins (6) reported an unusual case of
an estrogen-secreting adrenal tumor that caused
gynecomastia in a 6-month-old-boy. It is now
recognized that most children with an adreno-
cortical neoplasm show clinical evidence of an
endocrine abnormality, in contrast to the be-
havior of adrenocortical tumors in adults (1).
Thus, diagnostic imaging of adrenocortical neo-
plasms in children is typically guided by clinical
presentation; similar tumors in asymptomatic
adults are discovered incidentally during cross-
sectional imaging studies performed for unre-
lated indications (7).
This article, illustrated with cases contained
in the radiologic pathology archives of the
Armed Forces Institute of Pathology, examines
the clinical, pathologic, and radiologic features
990 ■ Continuing Education
of adrenocortical neoplasms in children and dis-
cusses differential diagnosis, treatment, and
prognosis.
■ CLINICAL FEATURES
Most adrenocortical neoplasms occur in chil-
dren with no underlying disorder. However, in
some cases, these tumors are associated with
other syndromes. In 1967, an association be-
tween adrenocortical neoplasms and congenital
hemihypertrophy was established by Fraumeni
and Miller (8), who reviewed the charts of 62
pediatric patients with adrenocortical neo-
plasms. Of these patients, two (3%) had hemi-
hypertrophy. The adrenal tumors did not al-
ways lateralize to the enlarged side of the body.
Children with Beckwith-Wiedemann syndrome
(sometimes referred to as EMG [exomphalos-
macroglossia-gigantism] syndrome) have an in-
creased risk of benign and malignant tumors of
multiple organs (8,9), and the most common
neoplasm associated with this syndrome is
nephroblastoma (Wilms tumor), followed by
adrenocortical carcinoma and hepatoblastoma
(1). Beckwith-Wiedemann syndrome is also
commonly accompanied by nonneoplastic en-
largement of the adrenal glands caused by corti-
cal hyperplasia. In addition, fetal adrenocortical
cells are characteristically enlarged in patients
with Beckwith-Wiedemann syndrome. This con-
dition, called bilateral adrenal cytomegaly, has
been associated with congenital metastasizing
adrenocortical carcinoma in one case report
(10). The Li-Fraumeni syndrome, also known as
the SBLA (sarcoma; breast and brain tumors;
leukemia, laryngeal carcinoma, and lung cancer;
and adrenocortical carcinoma) syndrome, repre-
sents a familial aggregation of neoplasms that
includes adrenocortical carcinoma (11,12). Pa-
tients with this syndrome may have alterations
in the p53 tumor suppressor gene located on
the short arm of chromosome 17, band 13 (13,
14). Finally, in rare cases, adrenocortical neo-
plasms have been reported in association with
congenital urinary tract abnormalities such as
duplication of the collecting system, tumors
such as ganglioneuroma and ganglioneuroblas-
toma, and congenital adrenal hyperplasia (1,15–
19).
Volume 19 Number 4
 a.
Figure 1. Mixed endocrine syndrome in a 7-month-old boy with an adrenocortical neoplasm.
(a) Photograph of the patient at 4 months shows normal facies. (b) Photograph of the same patient at
7 months demonstrates moon facies, acne, and bitemporal excess hair growth. Penile enlargement and
premature pubic hair growth were also present.
In a study of 32 pediatric patients with
adrenocortical neoplasms, the age at diagnosis
ranged from 6 months to 19 years (mean age, 8
years; median age, 5 years), with a predominant
number of patients being 5 years of age and
younger (20). In another study of 42 patients
with adrenocortical neoplasms, two-thirds were
younger than 5 years of age (21). In addition,
rare examples of congenital adrenocortical neo-
plasms have been reported (22–26). In an analy-
sis of 40 Brazilian children, in whom adrenocor-
tical tumors are more common than in United
States children (27), the median age at diagno-
sis was 3.9 years and over half were girls (28).
A female-to-male ratio of 2.2 to 1 was noted in a
recent literature review (29).
The primary tumor may not be apparent at
physical examination. In a retrospective review
by Teinturier and colleagues (30), a palpable
mass was found in only 57% of 45 children with
adrenocortical neoplasms. However, unlike
adult patients with tumors of the adrenal cor-
tex, most children with an adrenocortical neo-
plasm present with signs or symptoms of endo-
July-August 1999
b.
crine abnormality. In a review of over 200 tu-
mors in children by Neblett et al (29), only 17
nonfunctioning adrenocortical neoplasms were
identified.
Precocious puberty refers to secondary sex
characteristics that appear earlier than 8 years
of age in girls and before 9 years in boys. Preco-
cious puberty may be gonadotropin-dependent
(true precocious puberty) or gonadotropin-in-
dependent (pseudoprecocious puberty). Fur-
thermore, precocious puberty may be charac-
terized as isosexual when secondary sex charac-
teristics are appropriate for the patient’s gender
or heterosexual when they are inappropriate.
Heterosexual precocious puberty manifests as
virilization in girls and feminization in boys. Be-
cause functioning adrenocortical neoplasms
represent a gonadotropin-independent source
of endogenous androgens and cortisol, they
usually produce pseudoprecocious puberty,
Cushing syndrome, or a mixture of the two
(Fig 1).
Agrons et al ■ RadioGraphics ■ 991
 2. 3a.
Figures 2, 3. (2) Virilization in a 3-year-old
girl with an adrenocortical tumor. Clinical pho-
tograph of the external genitalia shows clitoro-
megaly and early pubic hair growth. (3) Adre-
nocortical neoplasm in a 16-year-old girl with
amenorrhea and hirsutism. (a) Clinical photo-
graph shows facial and chest hair and absence
of breast development. (b) Axial contrast ma-
terial–enhanced computed tomographic (CT)
scan of the abdomen demonstrates a heteroge-
neous left flank mass containing a large focus
of low attenuation (arrowhead), consistent
with necrosis. Retroperitoneal adenopathy (ar-
row) partly encases the abdominal aorta.

Young girls with an adrenocortical neoplasm

most commonly come to clinical attention be-
cause of virilization and less frequently present
with a mixed endocrine syndrome composed
of cushingoid features and virilization. Viriliza-
tion manifests as abnormal size and strength
due to increased muscle mass (herculean habi-
tus), clitoromegaly (Fig 2), facial hair (Fig 3),
advanced pubic and axillary hair development,
and advanced bone age. Overproduction of an-
drogen by adrenocortical tumor causes isosex-
3b.
ual pseudoprecocious puberty in boys, evi-
denced by the early development of acne, pu-
bic hair, and penile enlargement (Fig 4). Pure
Cushing syndrome, feminization in boys caused
by secretion of estrogen, or hypertension due
to primary hyperaldosteronism (Conn syn-
drome) are unusual (1,31,32). Infants with
Cushing syndrome tend to have generalized
obesity, rather than the truncal distribution of
increased fat found in adults (33).

992 ■ Continuing Education Volume 19 Number 4

 Figure 4. Adrenocortical neoplasm in a 3-year-old
boy with a 2-year history of pubic hair growth, penile
enlargement, deepening voice, and acne. (a) Frontal
photograph of the patient demonstrates precocious
development of secondary sexual characteristics, evi-
denced by penile enlargement, pubic hair, and in-
creased muscle mass. (b) Photograph of the patient’s
back shows extensive acne. (c) Frontal radiograph of
the left hand demonstrates accelerated skeletal matura-
tion (estimated at 12 years). (d) Transverse abdominal
sonogram shows a heterogeneous retrohepatic mass
(arrow). (e) Contrast-enhanced CT scan reveals the
slightly ill-defined heterogeneous right suprarenal mass.
a.

b. c.

d. e.

July-August 1999 Agrons et al ■ RadioGraphics ■ 993

 Laboratory abnormalities in children with
functioning adrenocortical neoplasms include
an increase in 24-hour urinary ketosteroid ex-
cretion and increased levels of serum cortisol,
testosterone, androstenedione, and estradiol (5).
■ PATHOLOGIC FEATURES
● Microscopic Features
Adrenocortical adenomas comprise a heteroge-
neous group of benign neoplasms that histologi-
cally resemble the appearance of the normal
zona fasciculata, the zona glomerulosa, or most
often, a combination of both. Cells are typically
arranged in nests separated by a delicate fibro-
vascular stroma. Cytologic features vary from
large, pale vacuolated cells with vesicular nu-
clei characteristic of the zona fasciculata to
smaller cells with eosinophilic cytoplasm and
condensed chromatin similar to those in the
zona glomerulosa and zona reticularis (Fig 5).
In general, adrenocortical adenomas are
bland with low nuclear-to-cytoplasmic ratio,
very little necrosis or hemorrhage, and absent
or exceptionally rare mitoses or bizarre nuclear
forms. In children, however, benign adrenocor-
tical tumors are more likely to display marked
nuclear atypia, pleomorphism, necrosis, and
mitotic activity than similar tumors in adult
patients. Occasionally, central degenerative
changes, including necrosis, hemorrhage, cystic
alteration, and vascular proliferation, may be
seen in adenomas, particularly as a complica-
tion of thrombosis following adrenal venogra-
phy (34). Despite some association of cell types
with secretory products, it is not possible to re-
liably predict endocrine function (eg, hyperal-
dosteronism, hypercortisolism, virilization) on
the basis of histologic characteristics.
Adrenocortical carcinomas show a wide
range of differentiation on histologic examina-
tion, not only between different tumors but
within the same tumor. Morphology ranges
from normal-appearing adrenal cells to com-
pletely undifferentiated cells. Broad fibrous
bands often separate the tumor into multiple
nodules. Most cells are lipid-poor and eosino-
philic, and they may be arranged in nests, trabe-
culae, or sheets. Hyperchromatic nuclei, pleo-
994 ■ Continuing Education
morphism with bizarre giant cells and multi-
nucleated forms, necrosis (especially confluent
areas), mitotic activity (including atypical mito-
ses), and vascular or capsular invasion may be
seen (Fig 5). However, none of these histologic
features is necessarily diagnostic of malignancy
in children (see adenoma vs carcinoma) (2,35).
● Gross Features
In a clinicopathologic study of 30 patients with
adrenocortical neoplasms by Lack et al (2), 60%
of lesions arose in the right adrenal gland, and
no patients had bilateral involvement. Ectopic
adrenocortical tumors are exceedingly rare
(36).
Adenomas are typically spherical, unilateral
and solitary, and well-demarcated but often un-
encapsulated, and they weigh less than 50 g.
Adenomas range in color from yellow to red-
dish-brown and may appear black if the tumor
contains a large amount of the piment lipofus-
cin (34,37–39).
Carcinomas are usually over 100 g (although
smaller weights have been reported) with
coarse trabeculations, multinodular contour,
and yellow to brown color. Areas of hemor-
rhage and necrosis are frequently seen (Fig 5)
(38,40). In adrenocortical neoplasms of child-
hood, malignant behavior is usually associated
with lesions that weigh more than 500 g, where-
as most tumors that weigh less than 500 g are
benign (35). Cystic change may be seen in both
adenomas and carcinomas, but it is more com-
mon in carcinomas and larger adenomas.
● Adenoma versus Carcinoma
An attempt to clarify the pathologic distinction
between adrenocortical adenomas and carcino-
mas has been made in at least several studies re-
viewing adrenocortical tumors in the general
population. These classification attempts in-
clude criteria for malignancy based on tumor
weight, clinical findings, and histologic features
proposed by Hough and coworkers (41) and
two systems based solely on histologic criteria
proposed by Weiss and colleagues and van
Slooten and colleagues (42–44). Division of car-
cinomas into low grade and high grade based
on mitotic rate has also been proposed (45,46).
Adrenocortical tumors in children, however,
cannot be reliably distinguished based on these
systems (39,46,47).
Volume 19 Number 4
 a.
c.
Lack et al (2) considered mitotic activity, vas-
cular invasion, and extent of tumor necrosis to
be the most prognostically useful histologic pa-
rameters in pediatric adrenocortical neoplasms.
In children, histologic characteristics and tumor
size may be suggestive of malignant potential,
but no single parameter (except for detection
of metastases) allows benign tumors to be dis-
criminated from malignant ones.
Special studies are often of limited value in
the pathologic diagnosis of adrenocortical tu-
July-August 1999
b.
Figure 5. Pathologic features of
adrenocortical neoplasm. (a) Photo-
micrograph (original magnification,
×300; hematoxylin-eosin [H-E] stain)
shows large, pale vacuolated cells (ar-
row) and smaller cells (arrowhead)
with eosinophilic cytoplasm, repre-
senting benign features. (b) Photo-
micrograph (original magnification,
×300; H-E stain) of a malignant adre-
nocortical neoplasm shows nuclear
atypia, pleomorphism, multinucleated
forms (straight arrow), and atypical
mitoses (curved arrow). (c) Photo-
graph of sectioned surgical specimen
of an adrenocortical carcinoma dem-
onstrates a nodular appearance, ex-
tensive hemorrhage, and necrosis.
Scale is in inches.
mors. Immunohistochemistry is not helpful in
distinguishing between benign and malignant
adrenocortical tumors, but it may be helpful in
diagnosing other primary neoplasms that metas-
tasize to the adrenal gland (48). Adrenocortical
carcinomas are usually vimentin positive and
often negative for cytokeratin, epithelial mem-
brane antigen, and carcinoembryonic antigen.
Agrons et al ■ RadioGraphics ■ 995
 The electron microscopic appearance of ad-
enomas resembles that of cells of the normal ad-
renal cortex, with steroid-producing cells show-
ing tubulovesicular or tubulolamellar mitochon-
dria, abundant smooth endoplasmic reticulum,
and parallel stacks of rough endoplasmic reticu-
lum. In addition, carcinomas may show abnor-
mal numbers and morphology of mitochondria
and dissolution of the basement membrane sur-
rounding alveolar groups of cells (2,34). At DNA
analysis, the presence of aneuploidy tends to fa-
vor malignancy, although this characteristic is
also found in benign tumors, especially larger
ones (44,49). Cytogenetic studies of carcinomas
have shown loss of heterozygosity of several
gene loci in some cases (44,50).
The histologic differential diagnosis of adre-
nocortical neoplasms includes nonneoplastic
adrenal nodules, multinodular hyperplasia
(which is usually bilateral and associated with
hyperplasia of intervening cortical areas), pheo-
chromocytoma, and metastatic carcinoma (par- Figure 6. Adrenocortical tumor in a 17-year-old
ticularly renal cell carcinoma, which is rare in girl with a 4-month history of lassitude. Spot radio-
children). graph from a selective right inferior adrenal arterio-
gram demonstrates neovascularity and puddling of
● Metastases contrast material within a large right suprarenal
The lung is the most common site of metastases mass.
from adrenocortical carcinoma, followed in fre-
quency by the liver. Other metastatic sites in-
clude the peritoneum (29% of cases), pleura ■ RADIOLOGIC FEATURES
or diaphragm (24%), abdominal lymph nodes The clinical presentation of an infant or child
(24%), and kidney (18%) (2). Direct extension with a functioning adrenocortical tumor may be
of tumor thrombus from the adrenal veins into indistinguishable from that of patients with ad-
the inferior vena cava represents an important renal hyperplasia or extraadrenal causes of iso-
mechanism of nonhematogenous malignant sexual or heterosexual precocious puberty (5).
spread and may be clinically silent. Moreover, the adrenal mass may be radiologi-
Tumor invasion of the inferior vena cava has cally occult. In a review of 17 children with
been reported in six of 17 (35%) patients with adrenocortical neoplasms, conventional radiog-
clinically malignant adrenocortical neoplasms, raphy of the abdomen demonstrated a soft-tis-
sometimes producing lower extremity and trun- sue mass in eight, of whom only three had tu-
cal edema (2). There has been a case report of mor calcification (5). Thus, before the wide-
spontaneous regression of metastatic skin nod- spread availability of ultrasound (US) and CT,
ules and brain metastases 4 months after surgi- the final diagnosis of adrenocortical tumor was
cal resection of a right-sided adrenocortical car- often delayed (3,4). Cross-sectional imaging
cinoma that manifested at birth (22). studies, including US, CT, and magnetic reso-
nance (MR) imaging, have largely supplanted
use of invasive procedures such as arteriogra-

996 ■ Continuing Education Volume 19 Number 4

 a.
c.
phy (Fig 6), venography, and venous sampling
in the evaluation of adrenal abnormalities in
children (51).
Sonography is an effective screening study
for a child with a suspected abdominal mass.
When carefully performed, US helps character-
ize the mass as solid, cystic, or complex and of-
ten reveals preserved tissue planes between the
tumor and adjacent organs, allowing distinction
of a suprarenal mass from one arising in the ad-
jacent kidney or liver. Large adrenal masses may
rotate the ipsilateral kidney or displace it inferi-
July-August 1999
b.
Figure 7. Adrenocortical neoplasm in a 2-year-old
girl with an abdominal mass and recent development
of pubic hair. (a) Longitudinal US image of the abdo-
men reveals a homogeneous, solid right suprarenal
mass. (b) Contrast-enhanced CT scan demonstrates
the circumscribed right suprarenal mass (arrow),
which enhances slightly more than the adjacent liver.
(c) Photograph of the bisected surgical specimen
shows a smooth, homogeneous, reddish-brown tu-
mor.
orly (52). In addition to its usefulness in the ini-
tial detection of the primary tumor, US with
color flow imaging facilitates the detection of
venous tumor thrombus from adrenocortical
carcinoma, which may extend into the right
atrium and pose a risk of embolism (53).
Hamper et al (54) described the sonographic
features of adrenocortical tumors in 26 patients,
including seven children with pathologically
proved adrenocortical carcinoma. The size of
the adrenal lesions ranged from 3 to 22 cm. All
were rounded or ovoid circumscribed masses
that commonly displayed a lobulated border
and, in seven (27%), a thin echogenic capsule-
like rim. Five adrenocortical carcinomas that
measured 6 cm or less were homogeneous solid
masses nearly isoechoic to renal cortex (Fig 7).
Agrons et al ■ RadioGraphics ■ 997
 a. b.
Figure 8. Adrenocortical tumor in an 18-month-old
boy with precocious puberty. (a) Longitudinal sono-
gram of the left flank demonstrates a large, heteroge-
neous mass (straight arrows, M) containing scattered
cystic spaces. The left kidney (curved arrow) is com-
pressed and displaced inferiorly. (b) Contrast-en-
hanced CT scan shows the heterogeneous left flank
mass with a faintly enhancing, capsule-like rim (ar-
row), associated with a contralateral retroperitoneal
nodal mass (arrowhead). (c) Photograph of the sec-
tioned surgical specimen demonstrates extensive cys-
tic change due to hemorrhage and necrosis. At sur-
gery, one of seven nodes contained tumor.

The larger lesions were heterogeneous and con- c.

tained central or diffuse hypoechoic regions
that corresponded to necrotic foci in the surgi-
cal pathology specimens (Fig 8). In two pa-
tients, central tumor necrosis produced a
multiseptated cystic appearance at sonography.
Tumor calcification was demonstrated in five
cases (19%) (Fig 9).
Fourteen children with adrenocortical carci-
noma were evaluated with US in a study by
Prando et al (55). The lesions, which ranged
from 2.5 to 19 cm in maximum diameter, were
all well-circumscribed. Smaller lesions were ho-
mogeneous and either uniformly hypoechoic or
hyperechoic. A complex, predominantly hyper-
echoic pattern was demonstrated in the 10 pa-

998 ■ Continuing Education Volume 19 Number 4

 a.
c.
tients with larger tumors, of which eight
showed radiating linear echoes. The authors
called this nonspecific stellate pattern the “scar
sign,” and considered it suggestive of cortical
carcinoma. To our knowledge, the predictive
value of this sign has not been determined. Vas-
cular invasion or retroperitoneal adenopathy
was noted in three patients.
July-August 1999
b.
Figure 9. Adrenocortical tumor in an 8-month-old
girl with pubic hair. (a) Longitudinal sonogram dem-
onstrates a heterogeneous, solid, left flank mass con-
taining a shadowing echogenic focus (arrow), consis-
tent with calcification. (b) Axial unenhanced CT scan
of the abdomen shows the large left flank mass with
faint calcifications (arrow). (c) Contrast-enhanced CT
scan shows heterogeneous enhancement of the mass,
low-attenuation regions (curved arrow) consistent
with necrosis, and faint enhancement of a capsule-
like rim (straight arrows).
CT is considered the most valuable tech-
nique for examining the adrenal glands (56).
The CT findings of adrenocortical neoplasms in
adults and children have been described (5,57–
59). On CT scans, adrenocortical tumors are
typically circumscribed, appear variably hetero-
geneous due to hemorrhage and necrosis, and
may display a thin capsule-like rim (Fig 9).
Larger lesions tend to enhance heterogeneously
following intravenous administration of contrast
material. In a study of 38 patients with adreno-
cortical carcinoma by Fishman et al (59), nine
(24%) had detectable calcification at CT (Fig
10).
Agrons et al ■ RadioGraphics ■ 999
 Figure 10. Adrenocortical neoplasm in a 13-year-old girl with hirsutism and hypertension. (a) Contrast-en-
hanced CT scan of the abdomen reveals a bulky, lobulated, heterogeneous left flank mass. Curvilinear foci of high
attenuation (arrow), consistent with calcification, delimit tumor lobules. (b) Coronal T1-weighted (repetition time
msec/echo time msec = 670/15) MR image shows a left flank mass of heterogeneous and predominantly low signal
intensity. A renal origin is suggested by the beak of normal renal parenchyma (arrowhead) about the inferior as-
pect of the tumor. The kidney is seen below the mass (arrows). (c) Coronal proton density–weighted (2,500/22)
MR image demonstrates heterogeneous increased signal intensity within the lesion. (d) Photograph of the surgical
specimen sectioned sagittally shows the inferior margin (arrows) of the lobulated and necrotic mass apparently in-
vading the upper pole of the left kidney. However, no invasion of the renal capsule was seen at microscopy.

a. b.

c.

d.

1000 ■ Continuing Education Volume 19 Number 4

 Figure 11. Adrenocortical tumor in an 18-month-old boy with pubic hair. (a) Longitudinal right renal sono-
gram depicts a round, circumscribed, hypoechoic suprarenal mass (arrow). (b) Coronal T1-weighted (530/15)
MR image shows the homogeneous mass that is nearly isointense relative to the renal cortex. (c) Axial T2-
weighted (3,400/90) MR image reveals a moderate increase in signal intensity within the lesion, which appears
distinct from the normal right adrenal gland (arrow). A well-encapsulated mass arising from a posterior limb of
the right adrenal gland was found at surgery.

a. b.

studied in adrenocortical tumors in children. It

c.
The distinction between adrenocortical car-
cinoma and adenoma in children is difficult to
make radiologically unless there is evidence of
hematogenous metastases or venous spread. In
adult patients, unenhanced CT densitometry al-
lows adrenal adenomas to be accurately differ-
entiated from nonadenomas because of the rela-
tively high lipid content in adenomas (60,61).
To our knowledge, this method has not been
has been proposed that tumor size greater than
6 cm in diameter and heterogeneity of the pri-
mary mass at imaging studies are useful indica-
tors of malignancy (55,62). However, Fishman
and colleagues (59) concluded that adrenocorti-
cal carcinoma may manifest as a well-margin-
ated, homogeneous mass 6 cm or less in diam-
eter on CT scans.
There are few descriptions of the MR imag-
ing appearance of hyperfunctioning adrenocor-
tical tumors occurring in childhood (51,53,58,
63). In one study of five pediatric patients with
pathologically proved adrenocortical tumors
(four adenomas, one of indeterminate histologic
characteristics) ranging in size from 1.0 to 7.5
cm, all lesions were of intermediate signal in-
tensity on T1-weighted MR images and of high
signal intensity relative to liver on T2-weighted
images (Figs 10, 11). There was no significant
difference in signal intensity characteristics be-
tween the larger indeterminate lesion and the
four adenomas. Similar MR imaging features in
two adenomas and one carcinoma were illus-
trated in a pictorial review by Westra et al (51).

July-August 1999 Agrons et al ■ RadioGraphics ■ 1001

 a. b.
Figure 12. Obstruction of the inferior vena cava by
tumor thrombus in a 15-year-old girl with a palpable ab-
dominal mass and bilateral lower extremity swelling.
(a) Contrast-enhanced chest CT scan (mediastinal win-
dow) shows the intrahepatic segment of the inferior
vena cava (black arrow) distended by heterogeneously
enhancing soft tissue. Dilated azygous (white arrow)
and hemiazygous (arrowhead) veins represent collateral
pathways of systemic venous return. A right pleural ef-
fusion is present. (b) CT scan obtained cephalad to a
demonstrates a lobulated, right atrial filling defect (ar-
row). (c) Coronal T2-weighted (4,000/102) MR image
reveals the primary tumor (arrowhead) in continuity
with inferior vena cava thrombus (arrow) that extends
into the right atrium.

The multiplanar capability of MR imaging fa-

cilitates detection of inferior vena cava tumor
thrombus (Fig 12) (53). The accuracy of op-
posed-phase chemical shift MR imaging of adre-
nal masses, which allows adrenal adenomas to
be differentiated from nonadenomas based on
their relative fat content, has been established
in adult patients (64–69). However, the utility
of this technique has not been studied in the
pediatric population.
Excess serum cortisol may produce interest-
ing secondary findings on cross-sectional imag-
ing studies that carry a high specificity for the
diagnosis of adrenocortical neoplasm. Of the
many causes of medullary nephrocalcinosis, hy-
percalcemia due to Cushing syndrome is un-
usual (70). Hyperechoic renal medullary pyra-
mids at sonography or high-attenuation medul-
lary areas on unenhanced CT images of patients
c.
with a suprarenal mass support the diagnosis of
adrenocortical neoplasm (Fig 13). Similarly, an
increase in the amount of retroperitoneal fat in
infants or young children, who normally have
little, is a useful anatomic clue to the nature of
an adrenal mass (Fig 13).
■ DIFFERENTIAL DIAGNOSIS
The combination of clinical features of hor-
mone overproduction in a child, supportive
laboratory findings, and a circumscribed adrenal
mass at cross-sectional imaging studies is virtu-
ally diagnostic of a functioning adrenocortical
neoplasm. Other pediatric adrenal masses in-
clude neuroblastoma, pheochromocytoma, ad-
renal hemorrhage, and, rarely, metastases.

1002 ■ Continuing Education Volume 19 Number 4

 a. b.

c. d.
Figure 13. Adrenocortical tumor in a 7-week-old girl with acne and hypertension. (a) Axial contrast-enhanced
CT scan of the abdomen shows a circumscribed, heterogeneous left suprarenal mass. (b, c) Longitudinal sono-
grams of the kidneys show the hypoechoic left suprarenal mass associated with hyperechoic renal pyramids
(arrow in c), representing medullary nephrocalcinosis. (d) Axial T1-weighted (600/15) MR image of the abdo-
men obtained inferior to the level of the mass reveals increased retroperitoneal fat (arrows) due to Cushing syn-
drome.

Neuroblastoma, a neoplasm of the adrenal
medulla and extraadrenal sympathetic tissue,
typically affects young children, and its age of
manifestation may overlap with that of child-
hood adrenocortical neoplasm. However, neu-
roblastoma often produces an increase in the
serum and urinary levels of catecholamines. In
addition, neuroblastoma is more commonly
metastatic at presentation than adrenocortical
carcinoma, and patients with this neoplasm of-
ten appear ill. Neuroblastoma may be associ-
ated with the paraneoplastic syndrome of myo-
clonic encephalopathy of infancy, and tumor
elaboration of vasoactive intestinal peptide may
produce watery diarrhea and hypokalemia. Al-
though the protean manifestations of neuroblas-
toma are well-recognized (71), cross-sectional
imaging studies of patients with neuroblastoma
typically demonstrate a large, irregular, retro-
peritoneal mass that frequently encases vascular
structures, often contains characteristic punc-
tate calcifications, and may extend into the ex-
tradural spinal canal through neural foramina.
Nevertheless, neuroblastoma may also mani-
fest as a circumscribed suprarenal mass that is

July-August 1999 Agrons et al ■ RadioGraphics ■ 1003

 a. b.
Figure 14. Metastatic adrenocortical carcinoma in
a 17-year-old girl with fatigue, weight gain, and hy-
pertension. (a) Unenhanced axial CT scan of the
abdomen shows a large, lobulated, right flank mass
containing punctate calcifications (arrow). (b) On a
CT scan obtained after intravenous contrast material
administration, the mass enhances heterogeneously
and displays low-attenuation regions representing
hemorrhage and necrosis. The inferior vena cava
(arrow) is compressed, displaced, and possibly in-
vaded. (c) Axial contrast-enhanced CT scan through
the upper abdomen shows a low-attenuation me-
tastasis (arrow) in the liver dome.

indistinguishable from an adrenocortical tumor,

and adrenocortical tumors may also be associ-
ated with calcifications (Fig 14), retroperitoneal
adenopathy, and vascular encasement (Figs 3,
8). Unlike adrenocortical neoplasms, neuroblas-
toma accumulates iodine-labeled metaiodoben-
zoguanidine (MIBG) at scintigraphy.
Although rare in children, pheochromocy-
toma, like neuroblastoma, is a neoplasm derived
from neural crest tissue, and also secretes cat-
echolamines and concentrates I-123 MIBG at
scintigraphy. However, pheochromocytoma
usually occurs in older children (6–14 years)
than does neuroblastoma or adrenocortical tu-
mors. Children with pheochromocytoma typi-
cally present with constant or paroxysmal head-
aches caused by hypertension (71,72). Pheo-
chromocytoma may occur in patients with
neurofibromatosis, von Hippel–Lindau disease,
c.
Sturge Weber syndrome, and multiple endo-
crine neoplasia types IIA and IIB. Less than 10%
of pheochromocytomas in children are malig-
nant (73). Pheochromocytoma characteristi-
cally appears as a rounded, circumscribed mass
at imaging studies. On T1-weighted MR images,
the lesion has lower signal intensity than that of
the liver but shows higher signal intensity than
does adrenocortical tumor on T2-weighted im-
ages (72).
Adrenal hemorrhage typically occurs in neo-
nates, which is an unusual age of presentation
for adrenocortical tumors. Hemorrhage can usu-
ally be distinguished from a neoplastic adrenal
mass by temporal evolution over serial sono-
grams, which typically depict liquefaction, clot
retraction, and shrinkage of the hematoma.

1004 ■ Continuing Education Volume 19 Number 4


Large adrenocortical neoplasms may appear
to invade or arise from the upper pole of the
kidney at cross-sectional imaging studies and
thus may mimic a primary renal tumor (Fig 10).
Solid renal neoplasms of childhood include
Wilms tumor (nephroblastoma), mesoblastic
nephroma, renal cell carcinoma, clear cell sar-
coma, and rhabdoid tumor of the kidney. Meso-
blastic nephroma may be congenital and is typi-
cally discovered in the neonatal period or early
infancy. Nevertheless, the age of presentation
of adrenocortical tumor in childhood may over-
lap that of mesoblastic nephroma and other pe-
diatric renal tumors. None of the renal tumors
produces the clinical findings of hormonally ac-
tive adrenocortical neoplasms. However, rhab-
doid tumor and mesoblastic nephroma may be
associated with hypercalcemia.
■ TREATMENT AND PROGNOSIS
Following characterization of the primary tu-
mor mass and possible metastases by means of
cross-sectional imaging studies, surgery is the
mainstay of treatment for adrenocortical neo-
plasms. Evaluation of the renal veins and infe-
rior vena cava with US, CT, or MR imaging is
critical, because tumor thrombus in the intrahe-
patic segment of the inferior vena cava or right
atrium requires a thoracoabdominal surgical ap-
proach. The optimal role of adjuvant chemo-
therapy in children who develop recurrent dis-
ease, who have metastases at diagnosis, or who
July-August 1999
Figure 15. Pulmonary metastases
from adrenocortical carcinoma in an
8-year-old girl with a 1-year history of
precocious puberty. Frontal radio-
graph of the chest demonstrates mul-
tiple spherical peripheral nodules.
The right adrenal mass (not shown)
measured 4 cm in diameter.
have persistent elevation of hormone levels fol-
lowing surgery continues to be investigated
(74). There have been limited reports of the
palliative use of radiation therapy (75).
In the older literature, a relatively poor prog-
nosis had been assigned to children with adre-
nocortical neoplasms (2). In a compendium of
adrenocortical tumors in children reported up
to 1962, only 23 of 222 patients survived 2 or
more years after treatment (76). However, the
rarity of adrenocortical neoplasms in the pedi-
atric age group limits the experience of many
pathologists, and a tendency to overdiagnose
the lesions as carcinoma has been recognized
(2,20,35). Unless metastases are present (Figs
14, 15), this difficulty in distinguishing ad-
enoma from carcinoma is compounded by the
absence of universally accepted histologic prog-
nostic factors. The study by Cagle et al (35)
concluded that pediatric adrenocortical neo-
plasms were more likely to be benign than had
previously been thought. Improved survival
rates may reflect the benefits of earlier detec-
tion because of the advent of cross-sectional im-
aging, the availability of cortisone replacement
therapy, and refinements in surgical techniques
and postoperative clinical care (1).
The prognosis for children with a pathologic
diagnosis of adrenocortical carcinoma is strati-
fied by age of presentation. In an analysis by
Agrons et al ■ RadioGraphics ■ 1005
 Lack and colleagues (2), the survival rate for pa-
tients older than 5 years was only 13%, com-
pared with 70% for children 5 years or younger.
A review by Humphrey et al (77) showed an
overall survival rate of 53% for infants with
adrenocortical carcinoma compared with 17%
for adolescents. Most deaths caused by adreno-
cortical carcinoma occur within 1–2 years after
diagnosis (2,77).
■ SUMMARY
Adrenocortical neoplasms are rare in children
but differ substantially in clinical characteris-
tics and biologic behavior from similar tumors
found in adults. Although the older literature as-
signed a relatively poor prognosis to pediatric
patients with adrenocortical neoplasms, recent
work suggests that these tumors are more likely
to be benign than previously thought. Never-
theless, it is often difficult to distinguish benign
from malignant lesions in the absence of meta-
static disease. The survival rate for patients
with aggressive adrenocortical tumors is age
stratified, with children younger than 5 years
faring significantly better. Use of cross-sectional
imaging studies, particularly CT and MR imag-
ing, has streamlined the evaluation of a child
with pseudoprecocious puberty or Cushing
syndrome, and the combination of finding an
adrenal mass and clinical features of adrenocor-
tical hyperfunction is diagnostic of adrenocorti-
cal neoplasm.
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