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AJR Integrative Imaging

LIFELONG LEARNING
FOR RADIOLOGY

Imaging Features of Sarcoidosis on MDCT, FDG PET,


and PET/CT
Hima B. Prabhakar1, Chad B. Rabinowitz1, Fiona K. Gibbons2, Walter J. O’Donnell2, Jo-Anne O. Shepard3, and Suzanne L. Aquino3

Objective The imaging features of sarcoidosis are protean and can


The objectives of this article are to discuss the epidemiology be shown with a variety of imaging techniques. Diagnostic
and natural history of sarcoidosis; to review the classic imaging imaging can not only help suggest a diagnosis in asymptom-
features of sarcoidosis on radiography, CT, and 67Ga nuclear atic patients, but can also help in monitoring therapeutic
medicine scans; and to present clinical examples of sarcoidosis response in symptomatic patients. FDG uptake on PET in
as seen on PET and PET/CT in the chest, abdomen and pelvis, patients with sarcoidosis is nonspecific and can mimic that
and bones. in malignancies such as lymphoma and diffuse metastatic
disease [2].
Conclusion
The imaging features of sarcoidosis are diverse and can be Epidemiology
seen on a variety of imaging techniques. It is important for ra- Sarcoidosis has a worldwide distribution and typically af-
diologists and nuclear medicine physicians to recognize the com- fects young to middle-aged adults. The highest prevalence of
mon imaging features and patterns of sarcoidosis in order to the disease is found in African-Americans, Swedes, and Danes.
raise the possibility in the appropriate clinical setting. In the United States, the incidence rate of sarcoidosis is 35.5
cases per 100,000 in blacks and 10.9 cases per 100,000 in
Introduction whites. Additionally, the disease incidence is slightly higher in
Sarcoidosis is a multiorgan granulomatous disease with a women than in men [3].
wide variety of imaging features. Imaging abnormalities can
commonly be seen on chest radiography, MDCT, 67Ga scans, Clinical Presentation and Natural History
FDG PET, and PET/CT. FDG uptake from sarcoidosis is non- Because sarcoidosis affects multiple organ systems, presen-
specific and can mimic other disease processes, including lym- tation varies from nonspecific constitutional symptoms to
phoma and diffuse metastatic disease. When combined with those related to specific organ involvement. Symptoms related
imaging features on other techniques, such as MDCT, FDG to lung involvement (dyspnea and cough) can lead to chest
uptake can be useful in monitoring therapeutic response in radiographs that eventually yield the diagnosis of sarcoid.
patients with known sarcoidosis. Because imaging features of One third of patients have peripheral lymphadenopathy, most
sarcoidosis can overlap considerably with those of malignant commonly involving the cervical, axillary, and inguinal lymph
disorders, it is important for both radiologists and nuclear nodes. One quarter of patients show characteristic skin le-
medicine specialists to be aware of the many varied presenta- sions, including erythema nodosum and lupus pernio [3].
tions of sarcoidosis in order to suggest the diagnosis in the The natural history of sarcoidosis varies significantly
appropriate clinical setting. from patient to patient. The disease spontaneously remits in
Sarcoidosis is a systemic and chronic disease of unknown up to one third of patients, but is chronic and progressive in
cause [1]. The characteristic histologic lesion, a noncaseating up to 30%. There is a 1–5% fatality rate from the disease,
granuloma, has been described as affecting all organ systems, most commonly resulting from severe respiratory or cardiac
although they are most frequently seen affecting the lungs [2]. involvement [3].

Keywords: CT, FDG PET, MDCT, PET/CT, sarcoidosis


DOI:10.2214/AJR.07.7001
Received March 8, 2007; accepted after revision June 11, 2007.
1
Abdominal Imaging and Interventional Radiology, Department of Radiology, Massachusetts General Hospital, 55 Fruit St., FND 270, Boston, MA 02114. Address correspondence to
H. B. Prabhakar (himaprab@gmail.com).
Department of Pulmonary/Critical Care Medicine, Massachusetts General Hospital, Boston, MA.
2

Thoracic Radiology, Department of Radiology, Massachusetts General Hospital, Boston, MA.


3

AJR 2008;190:S1–S6 0361–803X/08/1903–S1 © American Roentgen Ray Society

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Prabhakar et al.

Fig. 1—Stage 1 sarcoidosis in 54-year-old man with biopsy-proven sarcoidosis. Fig. 2—Pulmonary nodules in peribronchovascular distribution in 44-year-old
Frontal chest radiograph shows right paratracheal and bilateral hilar lymphade- woman with sarcoidosis. High-resolution chest CT image shows multiple tiny pul-
nopathy (arrows) and clear lungs. monary nodules centered in peribronchovascular distribution (upper arrow). Small
pulmonary nodules can also be seen lining right major fissure (lower arrow).

Fig. 3—Abdominal lymphadenopathy in 38-year-old man with biopsy-proven Fig. 4—Lambda (λ) sign on 67Ga scan in 26-year-old man with biopsy-proven sar-
sarcoidosis. Contrast-enhanced axial CT image of upper abdomen shows mul- coidosis. Anterior image of chest shows increased tracer uptake in right paratra-
tiple periaortic lymph nodes (arrows). cheal and bilateral hilar lymph nodes, in configuration known as “lambda sign.”

Classic Imaging Features of Sarcoidosis MDCT


Radiography Lymphadenopathy and parenchymal involvement in the
Chest radiographic features include mediastinal and bi- neck and chest are more readily shown on MDCT. In the
lateral hilar lymphadenopathy, parenchymal opacities, and, neck, palpable cervical lymphadenopathy is identified in
in more advanced cases, parenchymal fibrosis. A clinical one third of patients, usually in the posterior triangle. In
staging system based on the chest radiograph has been de- the chest, paratracheal, mediastinal, and bilateral hilar
vised to monitor disease in patients with sarcoidosis as well lymphadenopathy are most commonly identified. Charac-
as to predict patient prognosis. The five-part staging sys- teristic parenchymal lesions include pulmonary nodules,
tem ranges from stage 0 (no radiographic abnormality) to typically in a peribronchovascular distribution or along fis-
stage 4 (pulmonary fibrosis), with varying degrees of sures [2] (Fig. 2). Less commonly, alveolar consolidation can
lymphadenopathy and pulmonary parenchymal abnormal- be seen with air bronchograms, cavitation, and fibrosis [4].
ities in between. Spontaneous remission is more commonly In the abdomen, lesions are less characteristic, mimick-
seen in patients with stage 1 disease (Fig. 1) than in patients ing systemic diseases such as lymphoma, diffuse metastatic
with more advanced stages [3]. disease, or granulomatous or mycobacterial infection. In

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Imaging Features of Sarcoidosis

is nonspecific in both intensity and pattern, and is not gener-


ally useful in making an initial diagnosis. Additionally, marked
FDG uptake in lymph nodes and parenchymal organs can be
an important mimic of malignancy, specifically lymphoma
and diffuse metastatic disease. Despite this, FDG uptake can
decrease when sarcoidosis is treated, and PET can be useful in
monitoring the effectiveness of therapy [8, 9].
Although FDG uptake is nonspecific in sarcoidosis, com-
bining the imaging features of sarcoidosis on CT with up-
take on PET can make combined FDG PET/CT a useful
technique in monitoring disease progression or remission.
Additionally, if characteristic patterns of chest CT lesions
are identified (as described previously), along with typical
patterns of lymphadenopathy, the disease can be suggested
on the basis of FDG PET/CT findings. Histologic proof,
however, often is still required because of the importance of
excluding malignancies, particularly lymphoma [10].

Clinical Examples on FDG PET and PET/CT


Head and Neck
Head and neck involvement by sarcoidosis is usually iden-
tified as cervical lymphadenopathy, seen in approximately
one third of patients [2]. On FDG PET, increased uptake has
Fig. 5—Palpable submental lymph node with FDG uptake in 56-year-old woman been described in these lymph nodes (Fig. 5), as well as in the
with palpable submental lymph node. Axial fused contrast-enhanced PET/CT im- parotid glands, in a similar distribution to that seen with 67Ga
age shows enlarged left submental lymph node (arrow) with increased FDG up-
take. Lesion was biopsied and was consistent with sarcoidosis. scanning [7].

addition to diffuse lymphadenopathy (Fig. 3), nonspecific Chest


parenchymal lesions have been described, usually in the Although the radiographic and CT features of sarcoidosis
spleen and liver [4]. Diffuse hepatic involvement can prog- have been well described in the chest, few articles have spe-
ress in some cases to confluent hepatic fibrosis [2]. cifically addressed patterns of FDG uptake in the lungs. Me-
diastinal and hilar lymphadenopathy from sarcoidosis shows
Gallium-67 Scanning increased FDG uptake, as in other parts of the body (Fig. 6).
Gallium-67 imaging has been widely used in the diagnosis Lung parenchymal involvement and FDG uptake is less well
of sarcoidosis. Gallium-67 is taken up in lesions with in- described; however, it has been shown that FDG PET can
creased blood flow, typically in lesions having an inflamma- detect lung involvement by sarcoidosis in patients after trans-
tory or infectious cause. In sarcoidosis, a characteristic pat- plantation [9].
tern of uptake in the chest has been described as the “lambda
sign:” paratracheal and bilateral hilar uptake [5] (Fig. 4). An- Abdomen
other pattern of uptake is called the “panda sign,” caused by Again, as elsewhere in the body, abdominal lymph nodes
uptake in the lacrimal and parotid glands. Although this pat- secondary to sarcoidosis can show increased FDG activity
tern can be seen in other entities, such as lymphoma and [7]. Parenchymal lesions in the abdomen have also been de-
HIV, the bilateral symmetric involvement of the glands is scribed as showing increased FDG uptake. For example,
more typical of sarcoidosis [6]. Additionally, when the panda sarcoidosis is known to cause splenomegaly and low-density
sign is seen in conjunction with the lambda sign, it is highly focal lesions in the spleen that have been reported to have
specific for sarcoidosis [5]. increased FDG uptake on PET [11] (Fig. 7).

FDG PET and PET/CT Musculoskeletal


FDG PET is an important clinical tool in the evaluation of Bone involvement in sarcoidosis can be seen in up to one
known or suspected malignancy. Uptake of the tracer is non- third of patients, usually in the hands and feet. Less com-
specific, however, and is related to tissue metabolism. Thus, monly, axial skeletal involvement can be seen. In both cases,
the agent is also readily taken up in some infectious and in- lesions of sarcoid can be either osteolytic or osteosclerotic,
flammatory conditions. Prior studies show increased FDG and their nonspecific appearance can make diagnosis diffi-
uptake in active sarcoidosis [7, 8]. FDG uptake in sarcoidosis cult. Increased activity can be seen on bone scintigraphy.

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Prabhakar et al.

A B

Fig. 6—Confluent parenchymal lung nodules and mediastinal and bilateral hilar
lymphadenopathy with increased FDG uptake in 56-year-old woman with biopsy-
­proven sarcoidosis.
A–C, Axial CT image (A) shows confluent parenchymal lung nodules (yellow ar-
rows) and mediastinal and bilateral hilar lymphadenopathy (blue arrows). These
abnormalities show increased FDG uptake on fused PET/CT (B) and unfused
PET (C) images.

A B C

Fig. 7—Splenic lesions with uptake from sarcoidosis in 43-year-old woman with history of Hodgkin’s lymphoma.
A–C, Images from combined PET/CT show low-density lesions (arrows, A and C) in spleen on coronal CT image (A). Lesions show increased FDG uptake on fused PET/
CT (B) and unfused PET (C) images. Because of patient’s history of lymphoma, she underwent splenectomy to assess cause of lesion, and pathology revealed nonca-
seating granulomas consistent with sarcoidosis. Sarcoidosis is known to cause splenomegaly and low-density focal lesions in the spleen and has been reported to
have increased FDG uptake on PET scans [11].

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Imaging Features of Sarcoidosis

A B

Fig. 8—Skeletal uptake in 56-year-old woman with known sarcoidosis in neck,


who presented with pelvic bone pain.
A–C, Images from combined PET/CT scan show multiple subtle sclerotic lesions
(arrows) in bilateral iliac bones on axial CT image (A). These lesions show in-
creased FDG uptake on fused PET/CT (B) and unfused PET (C) images. Biopsy of
left iliac bone lesion was consistent with sarcoidosis.

Fig. 9—Follicular lymphoma and asymptomatic pulmonary sarcoidosis in 44-year-old woman


with history of grade 3 follicular lymphoma that is now in remission. Patient underwent trans-
bronchial biopsy to evaluate small lymph nodes in chest, which revealed noncaseating granulo-
mas consistent with sarcoidosis. Whole-body PET image shows marked FDG uptake in bilateral
axillae and left paratracheal regions (upper arrows), as well as in abdomen (lower arrows). Distri-
bution of adenopathy is more consistent with lymphoma than with sarcoidosis, especially be-
cause of lack of significant hilar or mediastinal lymphadenopathy. Biopsy of left axillary lymph
node revealed follicular lymphoma.

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Prabhakar et al.

Additionally, case reports have described increased FDG tures and patterns of sarcoidosis in order to raise the pos-
uptake in skeletal sarcoidosis (Fig. 8). In conjunction with sibility in the appropriate clinical setting.
the more characteristic findings of sarcoidosis, such as me-
diastinal lymphadenopathy, bone involvement from sarcoid References
can be suggested in patients with increased focal bone FDG 1. Cox CE, Davis-Allen A, Judson MA. Sarcoidosis. Med Clin North Am 2005;
89:817–828
uptake rather than diffuse metastatic disease [12, 13]. 2. Koyama T, Ueda H, Togashi K, Umeoka S, Kataoka M, Nagai S. Radiologic man-
ifestations of sarcoidosis in various organs. RadioGraphics 2004; 24:87–104
Sarcoidosis as a Mimic of Malignancy 3. Statement on sarcoidosis. Joint Statement of the American Thoracic Society
The most common radiologic finding in sarcoidosis is in- (ATS), the European Respiratory Society (ERS), and the World Association of
trathoracic lymphadenopathy, seen in up to 85% of patients Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the
ATS Board of Directors and by the ERS Executive Committee, February 1999.
[2]. Abdominal lymphadenopathy is seen 30% of cases, with Am J Respir Crit Care Med 1999; 160:736–755
massive lymphadenopathy (lymph nodes > 2 cm) seen in 10% 4. Warshauer DM, Lee JK. Imaging manifestations of abdominal sarcoidosis.
of patients [4]. Given the presence of lymphadenopathy in AJR 2004; 182:15–28
such a large percentage of patients with sarcoidosis, it is not 5. Sulavik SB, Spencer RP, Weed DA, Shapiro HR, Shiue ST, Castriotta RJ. Rec-
ognition of distinctive patterns of gallium-67 distribution in sarcoidosis. J Nucl
surprising that one of the more common differential consider-
Med 1990; 31:1909–1914
ations in these patients is lymphoma. Additionally, as de- 6. Kurdziel KA. The panda sign. Radiology 2000; 215:884–885
scribed previously, musculoskeletal involvement in sarcoidosis 7. Lewis PJ, Salama A. Uptake of fluorine-18-fluorodeoxyglucose in sarcoidosis.
can manifest as increased focal uptake throughout the skele- J Nucl Med 1994; 35:1647–1649
ton, which can mimic diffuse metastatic disease [12, 13]. 8. Nishiyama Y, Yamamoto Y, Fukunaga K, et al. Comparative evaluation of 18F–
FDG PET and 67Ga scintigraphy in patients with sarcoidosis. J Nucl Med 2006;
To further complicate matters, a known association exists
47:1571–1576
between sarcoidosis and lymphoma, described in 1986 by 9. Love C, Tomas MB, Tronco GG, Palestro CJ. FDG PET of infection and in-
Brinker and called “sarcoidosis–lymphoma syndrome” [14]. flammation. RadioGraphics 2005; 25:1357–1368
Several cases studies have been published describing the asso- 10. Hollister D Jr, Lee MS, Eisen RN, Fey C, Portlock CS. Variable problems in
ciation of chronic active sarcoidosis and systemic lymphoma, lymphomas: Case 2. Sarcoidosis mimicking progressive lymphoma. J Clin On-
col 2005; 23:8113–8116
both Hodgkin’s and non-Hodgkin’s lymphoma [15, 16] (Fig.
11. Vento JA, Arici M, Spencer RP, Sood R. F-18 FDG PET: mottled splenomega-
9). Using data from patients with respiratory sarcoidosis who ly with remission of symptoms after splenectomy in sarcoidosis. Clin Nucl
had registered with the Danish Institute of Clinical Epidemi- Med 2004; 29:103–104
ology, Brinker determined that patients with sarcoidosis are 12. Aberg C, Ponzo F, Raphael B, Amorosi E, Moran V, Kramer E. FDG positron
emission tomography of bone involvement in sarcoidosis. AJR 2004;
at 5.5 times increased risk of developing a lymphoprolifera-
182:975–977
tive disorder as other patients in the same age group [14]. 13. Ludwig V, Fordice S, Lamar R, Martin WH, Delbeke D. Unsuspected skeletal
sarcoidosis mimicking metastatic disease on FDG positron emission tomogra-
Conclusion phy and bone scintigraphy. Clin Nucl Med 2003; 28:176–179
The imaging features of sarcoidosis are diverse and can 14. Brinker H. The sarcoidosis-lymphoma syndrome. Br J Cancer 1986; 54:467–473
be shown on a variety of imaging techniques. FDG uptake 15. Schmuth M, Prior C, Illersperger B, Topar G, Fritsch P, Sepp N. Systemic sar-
on PET in patients with sarcoidosis is variable and can coidosis and cutaneous lymphoma: is the association fortuitous? Br J Dermatol
1999; 140:952–955
mimic malignancies such as lymphoma and diffuse meta- 16. Dunphy CH, Panella MJ, Grosso LE. Low-grade B-cell lymphoma and con-
static disease. It is important for radiologists and nuclear comitant extensive sarcoidlike granulomas: a case report and review of the lit-
medicine physicians to recognize the common imaging fea- erature. Arch Pathol Lab Med 2000; 124:152–156

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