Jurnal Ane 7
Transcript of Jurnal Ane 7
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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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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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CT and MRI of Adrenocortical Carcinoma
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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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 adrenocor- tical 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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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.
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