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W70 6 AJR:196, June 2011

Cause

Most ACCs are sporadic; however, they

also can be associated with several complex

genetic syndromes.

Li-Fraumeni Cancer Syndrome

Li-Fraumeni cancer syndrome results in

a familial susceptibility to a variety of can-

cers including adrenocortical tumors (car-

cinomas, adenomas), sarcomas, leukemias,

breast, brain, lung, and laryngeal cancers be-

cause of a germline TP53 mutation.

Carney Complex

Carney complex consists of primary pig-

mented nodular adrenal dysplasia, cardiac

myxomas, cutaneous myxomas, testicular

tumors, and other endocrine neoplasms.

Beckwith-Wiedemann Syndrome

Beckwith-Wiedemann syndrome is a con-

genital disorder characterized by pre- andpostnatal overgrowth, macroglossia, and an-

terior abdominal wall defects (most com-

monly exomphalos).

Familial Adenomatous Polyposis Coli

Familial adenomatous polyposis coli causes

multiple adenomatous polyps and cancer of

the colon and rectum, thyroid tumors, hepa-

toblastoma, and adrenocortical tumors (car-

cinomas, adenomas).

Adrenocortical Carcinoma:The Range of Appearanceson CT and MRI

Nishat Bharwani1

Andrea G. Rockall1

Anju Sahdev1

Maria Gueorguiev2

William Drake2

Ashley B. Grossman2

Rodney H. Reznek1

Bharwani N, Rockall AG, Sahdev A, et al.

1Imaging Department, St. Bartholomew’s Hospital,

King George V Wing, Ground Fl, Room 3 West Smithfield,

London EC1A 7BE, United Kingdom. Address correspon-

dence to N. Bharwani.

2Department of Endocrinology, Barts & The London NHS

Trust, London, United Kingdom.

Genitour inary Imaging • Review

CME

This article is available for CME credit.

See www.arrs.org for more information.

WEB

This is a Web exclusive article.

AJR 2011; 196:W706–W714

0361–803X/11/1966–W706

© American Roentgen Ray Society

Adrenocortical carcinoma (ACC)

is a rare, aggressive tumor aris-

ing from the adrenal cortex that

typically presents late with a

large mass. The increased use of cross-sec-

tional imaging for unrelated reasons has led

to a greater number of ACCs being detected

incidentally at an earlier stage. Recognition

of the typical clinical, biochemical, and im-

aging findings is imperative for rapid diagno-

sis, prompt intervention, and early use of the

appropriate therapy. Cross-sectional imag-

ing with CT and MRI is essential for deter-

mining the extent of local and distant tumor

spread. Complete surgical resection is cur-

rently the only potentially curative treatment

of ACC, and the information attained from

CT and MRI is important to guide surgery

and further patient treatment.

Epidemiology

ACCs account for only 0.05–0.2% of allcancers [1, 2] or 1–2 patients per 1 million

population per year [3]. The age distribution

of the affected population is bimodal, with

an increased incidence in infants and chil-

dren younger than 5 years old and in indi-

viduals in their fourth and fifth decades of

life [4, 5]. A female preponderance has been

noted [2, 3], and women are more likely than

men to present with more well-differentiated

tumors that tend to be functional [6].

Keywords: adrenal gland, adrenal neoplasms,

adrenocortical carcinoma, CT, MRI

DOI:10.2214/AJR.10.5540

Received August 11, 2010; accepted after revision

November 2, 2010.

OBJECTIVE. Adrenocortical carcinoma (ACC) is a rare, aggressive tumor arising from

the adrenal cortex that typically presents late with a large mass. The increased use of cross-

sectional imaging for unrelated reasons has led to a greater number of ACCs being detected

incidentally at an earlier stage. Recognition of the typical clinical, biochemical, and imaging

findings is imperative for rapid diagnosis, prompt intervention, and early use of the appropri-

ate therapy.

CONCLUSION. Cross-sectional imaging with CT and MRI is essential for determiningthe extent of local and distant tumor spread. Complete surgical resection is currently the only

potentially curative treatment of ACC, and the information attained from CT and MRI is im-

portant to guide surgery and further patient management.

Bharwani et al.CT and MRI of Adrenocortical Carcinoma

Genitourinary ImagingReview

F O C U S O N :


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CT and MRI of Adrenocortical Carcinoma

Multiple Endocrine Neoplasia, Type 1

Multiple endocrine neoplasia, type 1, causes

pituitary, parathyroid, and pancreatic tumors;

adrenocortical adenomas or hyperplasia; and,

very rarely, adrenocortical carcinomas.

Clinical and Biochemical Features

ACCs are functional in approximately60% of cases [4, 5, 7, 8], more commonly in

children (? 85%) than in adults (15–30%)

[4, 7]. Unlike adrenal adenomas that pre-

dominantly secrete cortisol, ACCs secrete

a variety of hormones including androgens,

cortisol, estrogens, and aldosterone [1]. In

adult patients with functioning tumors, 30%

present with Cushing syndrome, 20% with

virilization, and 10–20% with a combina-

tion of the two [1, 8]. Feminization and hy-

peraldosteronism are much rarer, each ac-

counting for approximately 2% of ACC

cases [8]. The rapid onset of Cushing syn-

drome, often with virilizing features, is

characteristic of ACC in adults [9]. Al-

though benign adrenocortical tumors tend

to secrete a single class of steroid, ACC can

secrete various types; cosecretion of corti-

sol with androgens is a frequent combina-

tion and is highly suggestive of malignancy

[3, 10–12]. In children, ACCs can present

with virilization, Cushing syndrome, femi-

nization, or Conn syndrome [1].

Approximately 65–85% of ACCs in adults

are nonfunctioning, and patients present

with a large mass and symptoms related to

mass effect (e.g., abdominal or flank pain in55%) [7] or with a palpable mass (40–50%)

[1, 7]. Some ACCs are discovered inciden-

tally (0–25%) when they tend to be smaller

[2, 13]. Due to the late presentation of non-

functioning tumors, a significant proportion

(~ 30% of ACC cases) presents with meta-

static disease to the regional and paraaortic

lymph nodes, lung, liver, and bone [7, 12].

Pathologic Features

Separating benign from malignant adrenal

cortical neoplasms is not always possible on

the basis of histologic findings alone, partic-

ularly from biopsy specimens [2, 14]; how-ever, there are macroscopic and microscopic

criteria that favor malignancy [14–17].

Macroscopic Criteria

The macroscopic criteria that favor malig-

nancy are tumor wet weight of greater than

500 g; a tumor with a grossly lobulated cut

surface; and the presence of necrotic areas,

calcification, or hemorrhage in the tumor.

Microscopic Criteria

The microscopic criteria that favor malig-

nancy are architectural disarray, mitotic rate,

marked nuclear pleomorphism, nuclear atyp-

ia, hyperchromasia, capsular invasion, and

venous or sinusoidal invasion. The mitotic

rate is also important for predicting tumor

aggressiveness.

Staging

The most widely used staging system for

ACC was proposed by the American Joint

Committee on Cancer and the International

Union Against Cancer (UICC) and uses the

TNM principle [18, 19] (Tables 1 and 2).

This system is based largely on earlier clas-

sification systems proposed by MacFarlane

[20] and modified by Sullivan and colleagues

[21]. In recent evaluations, authors have sug-

gested that there are significant limitations in

the prognostic accuracy of the UICC system

[22, 23], and a new system, the European

Network for the Study of Adrenal Tumors

(ENSAT) classification, has been proposed

[22]. According to the ENSAT system, stage

III disease is defined as the presence of posi-

tive lymph nodes, infiltration of the surround-

ing tissues, or venous tumor thrombus, and

stage IV disease is restricted to patients with

distant metastases.

Imaging Appearances

The presence of metastatic disease is defini-

tive of malignancy [24]. However, several im-

aging features should increase the suspicion of

ACC within an adrenal mass [1, 25–27]: tumor

size greater than 4 cm, irregular tumor mar-

gins, central intratumoral necrosis or hemor-

rhage, heterogeneous enhancement, invasion

into adjacent structures, venous extension

(renal vein or inferior vena cava [IVC]),

and calcification. Using a logistic regression

model, Hussain et al. [25] found tumor sizeof greater than 4 cm and heterogeneous en-

hancement to be the most important discrim-

inators of malignancy.

ACCs are usually large at presentation,

ranging from 2 to 25 cm (average size, approx-

imately 9 cm). Approximately 70% of ACCs

are larger than 6 cm [24] (Figs. 1–3). They are

bilateral in 2–10% of cases [2] and are slightly

more common on the left than on the right [4].

Tumors are frequently hemorrhagic (Fig. 1)

and necrotic [24, 28] (Fig. 2) and may con-

tain small areas of intracytoplasmic lipid or

fatty regions [29, 30] (Fig. 3). The existence

of intracytoplasmic fat in ACCs has been at-

tributed to the presence of cortisol and relat-

ed fatty precursors in hormonally active tu-

mors [30]. On occasion, pockets of fat may

be seen within the mass, indicating coexis-

tent myelolipomatous tissue.

IVC invasion has been reported in 9–19% of

ACC cases at presentation [5] (Figs. 4 and 5).

CT

The typical appearance of ACC on unen-

hanced CT is of a large, inhomogeneous but

well-defined suprarenal mass that displaces

adjacent structures as it grows [24]. Regions

TABLE 1: TNM Staging of Adrenocortical Carcinoma [18–21]

TNM Stage Description

Primary tumor (T)

Tx Primary tumor cannot be assessed

T0 No evidence of primary tumor

T1 ≤ 5 cm in greatest dimension, ex traadrenal invasion absent

T2 > 5 cm in greatest dimension, extraadrenal invasion absent

T3 Tumor of any size with local invasion, but not invading adjacent organsa

T4 Tumor of any size with invasion of adjacent organsa

Regional lymph nodesb (N)

Nx Regional lymph nodes cannot be assessed

N0 No regional lymph node metastasis

N1 Positive regional lymph nodes

Distant metastases (M)

M0 No distant metastases

M1 Distant metastases present

aAdjacent organs include kidney, diaphragm, great vessels, pancreas, and liver.bThe regional lymph nodes are hilar, abdominal paraaortic, and paracaval nodes.


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Bharwani et al.

of low attenuation correspond to necrosis

pathologically (Fig. 2); in one reported se-

ries, necrosis was invariably present when

tumors reached 6 cm in size [24]. However,

smaller lesions may be homogeneous on un-

enhanced CT [31]. After the administration

of IV contrast material, there is inhomoge-neous enhancement of the tumor, typically

with greater enhancement seen peripherally

and relatively little enhancement seen cen-

trally, because of central necrosis [24, 32].

Measurement of the attenuation of adrenal

lesions on unenhanced CT is of great value

in distinguishing between benign and ma-

lignant masses. Cumulative data obtained

for the identification of adrenal adenomas

indicate that ACCs rarely have an attenu-

ation value of less than 10 HU. The speci-

ficity of this threshold for the identification

of benign adenomas is approximately 98%

[33]. Equally, ACCs retain IV contrast ma-

terial and have absolute and relative percent-

age washout of less than 60% and less than

40%, respectively, at 15 minutes after con-

trast administration [26, 34] or less than 50%

and less than 40%, respectively, at 10 min-

utes [34–36] (Fig. 6).

Calcification, either microcalcification or

coarse calcification, is seen on CT in approxi-

mately 30% of patients with ACC and is usu-

ally centrally located [24, 32] (Fig. 3). Cal-

cification is rare in adenomas, although it is

present in approximately 10% of pheochro-

mocytomas [37].

Tumor thrombus extending into the IVC at

presentation is not rare [28] and is more fre-

quently seen in right-sided tumors. A tumorthrombus within a vein is usually well encap-

sulated and can often be withdrawn intact from

the vein [38]. The presence and cephalad ex-

tent of tumor thrombus can be identified on

contrast-enhanced CT or MRI (Fig. 4 and 5).

CT is also of value in showing the local

and distant spread of an ACC. Preservation

of fat planes around the tumor indicates that

there is no local invasion. Where there is a

paucity of retroperitoneal fat, it may be im-

possible to determine whether tumor has in-

vaded adjacent organs.

Metastases are frequently found at presen-tation: Regional and paraaortic lymph nodes

(25–46%), lungs (45–97%), liver (48–96%),

and bone (11–33%) are the common sites [1,

5, 28]. Hepatic metastases tend to be hyper-

vascular and are best seen on arterial phase

imaging after IV contrast administration.

MRI

ACC is typically heterogeneous in signal

intensity on MRI because of the presence

of hemorrhage and/or necrosis [30]. On T1-

weighted imaging, ACC is typically isoin-

tense or slightly hypointense to normal liver

parenchyma. However, high T1 signal inten-sity is often seen because of the presence of

hemorrhage (Fig. 1). On T2-weighted imag-

TABLE 2: Surgical Staging of Adrenocortical Carcinoma [8, 18, 20, 21, 73]

Surgical Stage Imaging Feature Percentage at Presentation 5-year Survival (%)

I Tumor ≤ 5 cm without local invasion, nodal or distant metastases 2.2–6.3 65

II Tumor > 5 cm without local invasion, nodal or distant metastases 21.7–49.8 65

III Tumor with local invasion or positive lymph nodes 17.9–22.5 40

IV Tumor with local invasion and positive lymph nodes or distant metastases 21.3–34.7a 10

aPresence of metastases.

A

A

Fig. 1—32-year-old woman with cortisol-secreting right adrenocortical carcinoma resulting in Cushing syn-drome. (Reprinted with permission from [27])A, Unenhanced CT scan shows large low-attenuation suprarenal mass (arrowheads ), with internal areas of highattenuation (arrows ) consistent with hemorrhage.B, Axial T1-weighted MR image shows high signal intensity (arrows ) within right adrenal mass (arrowheads )consistent with hemorrhage.

Fig. 2—35-year-old woman with adrenocortical carcinoma.A, Portal venous phase CT scan shows large heterogeneously enhancing left suprarenal mass that displacesleft kidney inferiorly. Regions of nonenhancing tissue (arrows ) are consistent with necrosis.B, Axial T2-weighted MR image shows high signal intensity (arrows ) consistent with necrosis.

B

B


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ing, ACC is usually hyperintense to liver pa-

renchyma and has a heterogeneous texture

because of the presence of intratumoral cys-

tic regions and hemorrhage [39] (Fig. 2).

A functioning ACC can contain small re-

gions of intracytoplasmic lipid resulting in

small nonuniform areas of loss of signal on

chemical shift imaging (< 30% of the lesion)[26, 29, 30] (Fig. 7). Although similar small

nonuniform loss can occur in lipid-poor ad-

enomas, the significant uniform signal loss

seen in lipid-rich adenomas does not occur.

Schlund et al. [30] described the presence

of peripheral mural-based enhancing nod-

ules in seven of eight ACCs reviewed. This

feature has not been described elsewhere in

the medical literature.

Enhancement after the administration of

IV contrast material is generally avid with

slow washout [3].

MRI has been shown to be superior to CT

in the delineation of the presence and extent

of IVC invasion [40, 41] (Fig. 5).

The results of early studies suggested that

proton MR spectroscopy may be useful indifferentiating adrenal adenomas and pheo-

chromocytomas from adrenal metastases and

ACC [42]. Faria et al. [42] looked at the spec-

tral traces obtained from 60 patients with ad-

renal masses. Adenomas and pheochromo-

cytomas could be differentiated from ACCs

and metastases using choline-creatine ratios

of greater than 1.20 (92% sensitivity and 96%

specificity) and choline-lipid ratios of great-

er than 0.38 (92% sensitivity and 90% speci-

ficity). ACCs and pheochromocytomas could

be differentiated from adenomas and metasta-

ses using a 4.0–4.3 ppm/creatine ratio greater

than 1.50 (87% sensitivity and 98% specific-

ity). By combining these two spectral analy-

ses, they were able to divide adrenal mass

lesions into one of four distinct groups: adeno-

ma, pheochromocytoma, ACC, or metastasis

[42]. Although some criticisms of this study

have been raised, the technique appears to of-fer potential in helping to distinguish among

adrenal mass lesions [43].

Functional Imaging

FDG PET can identify some malignant adre-

nal masses by virtue of their increased metabol-

ic activity; however, when FDG uptake is only

modest, the likelihood of benign versus malig-

nant is about equal [44]. FDG PET combined

with contrast-enhanced CT has a sensitivity of

100% and specificity of 87–97% for identify-

ing malignant adrenal masses. The lower speci-

ficity is because a small number of adenomas

and other benign lesions mimic malignancy[45, 46]. The novel PET tracer 11C metomidate,

a marker of 11β-hydroxylase, is used as tracer

for adrenocortical tissue and is taken up by ad-

enomas and ACCs. This marker differentiates

adrenal cortical lesions from pheochromocyto-

mas and metastases, which are uptake-negative

[47]. However, the most valuable aspect of PET

is its ability to detect distant metastases (Fig. 8);

it is important to remember that one third of pa-

A

A

Fig. 3—20-year-old woman with adrenocortical carcinoma.A and B, Axial (A) and sagittal (B) contrast-enhanced images show large right suprarenal mass that is displacinginferior vena cava medially and right kidney inferiorly. Tumor contains flecks of coarse calcification ( blackarrows , B) and macroscopic fat (–20 HU) (white arrows ) centrally.

Fig. 5—60-year-old woman with adrenocortical carcinoma (ACC).A and B, Axial T2-weighted (A) and coronal MR venography (B) images show large heterogeneous left-sidedACC (asterisk ) with tumor thrombus extending into adrenal vein (arrow , B) and inferior vena cava (arrow , A).

Fig. 4—52-year-old man with metastatic adreno-cortical carcinoma. Portal venous phase CT scanshows large, irregular right suprarenal mass (blackarrow ) with enhancing tumor thrombus (arrowheads )extending into right renal vein and inferior venacava resulting in luminal filling defect. At least twometastatic deposits (white arrows ) are shown withinadjacent liver.

B

B


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Bharwani et al.

tients with ACC will have metastatic disease at

presentation [7, 12, 48].

Differential Diagnosis and

Distinguishing Features

Adenoma

Adenomas may be diagnosed with a sensi-

tivity of 75–98% and specificity of 92–100%

using CT washout characteristics [49] and

chemical shift imaging [50]. However, in some

cases, it can be difficult to distinguish benign

from malignant lesions. If they measure 3–4

cm in diameter, the pathologic label of “in-

determinate malignant potential” is often ap-plied, and if they are larger than 4 cm, they are

generally managed as malignant lesions.

Pheochromocytoma

Pheochromocytomas may be benign or

malignant. Small pheochromocytomas are

usually homogeneous in appearance with

a density of 40–50 HU on unenhanced CT

[26], whereas larger pheochromocytomas

can be inhomogeneous with areas of hemor-

rhage and necrosis [51] (Fig. 9A). There is

no correlation between tumor size and ma-

lignancy [52]. On MRI, pheochromocyto-

mas are typically described as isointense orhyperintense to liver on T1 and hyperintense

to fat on T2 [51]. However, appearances can

be variable. For example, Jacques et al. [52]

reported in 2008 that only 11% of pheochro-

mocytomas showed “typical” T2 hyperinten-

sity and that pheochromocytomas that were

only mildly hyperintense to the spleen (34%)

or that were heterogeneous on T2 (39%) were

more common. Increasing heterogeneity was

seen to correlate with increasing amounts of

hemorrhage, necrosis, and fibrosis.

After IV contrast administration, pheochro-

mocytomas enhance avidly and have a pro-

longed washout phase, although exceptions do

exist [53]. Ninety-one percent of pheochromo-

cytomas are functioning and biochemical mark-

ers are important in establishing the diagno-

sis [54]. Nonfunctioning pheochromocytomas

(9%) pose more of a diagnostic dilemma; al-

though many will be differentiated from ACCusing 123I-metaiodobenzylguanidine (MIBG)

scintigraphy, some nonfunctioning pheochro-

mocytomas will not be MIBG-avid [54].

Dominantly inherited succinate dehydro-

genase (SDH) gene mutations account for

most familial paraganglioma syndromes in

which patients have an increased incidence

of adrenal and extraadrenal paragangliomas.

In patients with SDH-B gene mutations,

there is an increased risk of malignancy,

which is reported as between 34% and 97%,

and paragangliomas usually show intense

uptake on FDG PET [55, 56].

Lymphoma

The primary pathologic type that involves the

adrenal glands is non-Hodgkin diffuse large B-

cell lymphoma [57, 58]. Disease is usually bilat-

eral with enlarged adrenal glands [57, 58] that

maintain their normal “adeniform” shape.

Metastases

Adrenal metastases are found in up to 27%

of patients with malignant epithelial tumors at

autopsy [59]. This diagnosis should be consid-

ered when bilateral adrenal lesions are present

and there is a known primary malignancy else-

where or there is evidence of other metastases.

The most common primary site is the lung.

A

Fig. 7—38-year-old man with right-sided adrenocortical carcinoma (ACC).A and B, Chemical shift imaging shows large irregular right-s ided lesion (arrow ) that does not show signal lossbetween in-phase (A) and out-of-phase (B) images. Tumor was confirmed to be ACC on histology.

B

A

Fig. 6—50-year-old woman with right-sided adrenocortical carcinoma (ACC). Dedicated adrenal CT with washout studies shows 2.5-cm right suprarenal mass.A, Unenhanced CT image shows that lesion (arrow ) has attenuation of 29 HU and therefore requires further characterization.B and C, CT images obtained 60 seconds (B) and 15 minutes (C) after administration of IV contrast material show lesion (arrow ) has attenuation values of 54 and 43 HU,respectively. These attenuation values result in absolute percentage washout of 43% at 15 minutes, making lesion indeterminate by CT washout criteria. Mass was con-firmed to be ACC on histology.

CB


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Composite or Collision Tumors

Composite or collision tumors are rare tu-

mors that consist of two contiguous but his-

tologically different tissues within a single

mass [60]. A collision tumor is composed of

independently coexisting neoplasms without

significant tissue admixture, whereas a com-

posite tumor contains coexisting neoplasms

with considerable admixture of the two dif-ferent cell types such as a ganglioneuroma

and pheochromocytoma (Fig. 9B) or myelo-

lipoma and Cushing adenoma.

Ganglioneuroma

Ganglioneuromas are benign neoplasms

arising from the sympathetic ganglia. Gen-

erally, they are large solid lesions on CT

with homogeneous to mildly heterogeneous

enhancement after IV contrast administra-

tion (Fig. 9C). On MRI they are typically

hypointense on T1 and heterogeneously hy-

perintense on T2 depending on the content

of their myxoid stroma [61, 62].

Infection

The imaging appearances of infection

within the adrenal gland are generally non-

specific and can be seen as soft-tissue masses

and cystic changes with or without calcifica-

tion. Tuberculosis and histoplasmosis tend to

be bilateral but can be asymmetric and give

the appearance of unilateral disease [63].

Neuroblastoma

Neuroblastomas occur most frequently in

children and are rare in the adult population.

Calcification is a hallmark of neuroblasto-

ma in children but is rarely seen in adults.

Adults with neuroblastoma tend to show a

higher rate of metastatic disease at presenta-

tion than children do [64, 65].

Adrenal Hemangioma

Adrenal hemangiomas are well-defined

soft-tissue masses with inhomogeneous en-

hancement after contrast administration.

They are often calcified because of either in-tratumoral phleboliths or previous hemor-

rhage. On MRI, adrenal hemangiomas are

typically hypointense to liver on T1 and may

exhibit central hyperintensity due to hem-

orrhage. On T2, lesions are hyperintense.

Foci of low signal intensity on T1 and T2 are

caused by calcification. Characteristically,

they show persistent peripheral enhancement

on delayed imaging [66, 67].

All the diagnoses discussed can present a

diagnostic challenge in trying to differenti-

ate them from an ACC. In practice, however,

it is most frequently adenomas that can pres-

ent the greatest difficulty, partly because of

the frequency with which they occur. Indeed,

on occasion, pathologists also find it difficult

to make this distinction [2, 14, 15]. Thus, al-though most benign cortical adenomas can

now be confidently diagnosed on the basis of

the criteria mentioned, some lesions remain

indeterminate. When biochemical testing

shows these lesions to be functioning, most

endocrinologists would advocate removal of

the mass. Surgery may also be indicated if

doubt exists about the true nature of a non-

functioning lesion.

A detailed clinical history and biochemi-

cal testing can often distinguish between a

pheochromocytoma and an ACC without the

need for diagnostic imaging tests, although

imaging is still often required for surgical

planning. The distinction between large non-

functioning pheochromocytomas and ACCs

can be problematic, particularly when the le-

sion is not 123I-MIBG-avid. Once again, sur-

gery is sometimes required to resolve the di-

agnostic dilemma.

Treatment Planning

Role of Biopsy

There is controversy concerning the role

of biopsy in indeterminate adrenal lesions.

On one hand, percutaneous biopsies of sus-

pected ACC may not be justified in light ofthe risks of inducing tumor capsule break-

down and tumor spread along the needle

track [68]. The difficulty arises with suspect-

ed adrenocortical lesions that are borderline

in size (3–4 cm) in patients at high surgical

risk. In some of these cases, biopsy may be

justified, but decisions need to be made for

each patient individually.

Surgery

The definitive treatment of all stages of

ACC is en bloc resection of the tumor with or

without adjacent invaded organs. If en bloc re-

section is not possible because of local exten-sion into adjacent structures, maximal tumor

debulking surgery is indicated. This surgery

decreases the amount of hormone-secreting

tissue present and also reduces complications

due to mass effect.

IVC invasion is not rare, and surgery is

performed even when tumor extends the en-

tire length of the IVC and into the right atri-

um; cardiac bypass techniques may be used

A

Fig. 8—52-year-old man with metastatic adrenocortical carcinoma (ACC).A–C, Axial diagnostic contrast-enhanced CT image(A) and axial (B) and coronal (C) fused PET/CT imagesshow large right adrenal mass (arrow ) with avid FDGuptake and multiple metabolically active hepaticmetastases (arrowheads ).

B

C


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Bharwani et al.

in these cases. Delineation of IV tumor is par-

amount in surgical planning because venous

control must be established distal to the tumor

thrombus and may require bypass procedures

for venotomy or tumor thrombectomy.

Surgery can be open or laparoscopic for

small tumors without local invasion or tumor

thrombus depending on the tumor extent and

the expertise of the local surgical team [69].

However, open adrenalectomy is currently the

preferred option because of the high rate of re-currence and peritoneal carcinomatosis asso-

ciated with laparoscopic procedures [70, 71].

There are no published guidelines regard-

ing postsurgical imaging follow-up of patients

with ACC. At our institution, the interval and

modality are decided on an individual patient

basis and include CT, MRI, and PET/CT.

More recently, less invasive techniques

have been introduced whereby both adreno-

cortical tumors and adrenal metastases have

been treated successfully by radiofrequency

ablation [72].

Chemotherapy

Treatment with the adrenolytic drug mito-

tane may improve survival or at least control

symptoms [3, 7, 12] and is used in both pri-

mary and adjuvant therapy. It also plays a role

in metastatic and recurrent disease. There is

currently no agreement about the possible role

of other forms of cytotoxic chemotherapy, but

large-scale trials are under way to assess dif-

ferent chemotherapeutic regimens.

Radiotherapy

Radiotherapy is indicated in patients with

a high risk for local recurrence including

those with advanced locoregional disease

and incomplete or indeterminate resection;

radiotherapy may be helpful in treating the

symptoms from bone metastases [73].

Prognosis

Patients with unresectable stage IV ACC

have a median survival of 3 months [20].When treated aggressively with surgery, pa-

tients with stage I and II tumors have an ap-

proximately 65% 5-year survival, whereas

patients with stage III and IV disease have

40% and 10% 5-year survival, respectively

[74]. The overall 5-year survival rate for all

patients with ACC is 38% [8, 75].

Recurrence (Fig. 10) and metastatic dis-

ease (Figs. 4 and 8) are common in patients

with ACC. Of the patients undergoing appar-

ent complete resection, 35–85% will develop

recurrent or metastatic disease [5, 76].

Conclusions

The imaging appearances of ACC are di-

verse because of the variable presence of ne-

crosis, hemorrhage, calcification, and intra-

cellular lipid content. As illustrated, other

diseases can simulate ACC, and familiarity

with both typical and atypical appearances on

cross-sectional imaging taken in conjunction

with clinical information helps to suggest the

accurate diagnosis and appropriate treatment.

Imaging is also used to plan the extent of and

surgical approach for ACC resection.

References

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A

Fig. 9—Examples of some differential diagnoses.A, 36-year-old man who presented with hypertension and flushing. Arterial phase CT image shows large heterogeneous enhancing left suprarenal mass, which alsoshowed intense metaiodobenzylguanidine uptake (not shown). Mass was confirmed to be pheochromocytoma after surgery.B, 27-year-old woman who presented with left flank pain and palpitations. Portal venous phase CT image shows large left suprarenal mass with regions of necrosis (arrows ) seen centrally. Mass was confirmed to be collision or composite tumor on pathologic examination and contained both pheochromocytoma and ganglioneuroma

tissue.C, 42-year-old man who presented with refractory hyper tension. Portal venous phase image through upper abdomen shows lobulated infiltrative lef t suprarenal mass.Mass was confirmed to be benign ganglioneuroma on pathologic examination.

Fig. 10—45-year-old man with recurrent disease inright adrenalectomy bed. Surgical clips (arrows ) areengulfed by abnormal enhancing soft tissue in rightadrenalectomy bed. Infiltrative recurrent tumor (arrowheads ) invades liver and inferior vena cava.

CB


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