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Journal of Radiology Case Reports

Retroperitoneal Fibrosis as a Cause of Positive FDG PET/CT

Case Report

Amar Mehta1, Todd M. Blodgett2*

Radiology Case. 2011 July; 5(7):35-41 :: DOI: 10.3941/jrcr.v5i7.722

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1. Department of Radiology, University of Pittsburgh, Pittsburgh, PA, USA
2. Foundation Radiology Group, Pittsburgh, PA, USA

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  ABSTRACT
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This article educates the reader on idiopathic retroperitoneal fibrosis and emphasizes the importance of considering conditions that mimic this disease on PET/CT through an illustrative case of a 58 year-old man presenting with intermittent abdominal, back, and scrotal pain undergoing successive PET/CT scans, both for diagnosis and following treatment.








  CASE REPORT
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This is a case of a 58 year-old man who presented with approximately two weeks of intermittent abdominal, left lower back, and left scrotal pain. The patient was initially evaluated with a CT scan of the abdomen and pelvis, which showed circumferential soft-tissue thickening primarily around the left common iliac artery and vein causing minimal external compression of the vessels. The scan was also significant for left-sided hydronephrosis and slight medial deviation of the left ureter, for which the patient was treated with insertion of a double-J ureteral stent. Biopsy taken at this time showed dense fibrous tissue containing lymphocytes, plasma cells, neutrophils and eosinophils, findings which, though supporting the clinical impression of retroperitoneal fibrosis, were deemed non-specific; malignancy could not entirely be ruled out, though biopsy showed no evidence of neoplasm. The patient was prescribed steroids for approximately three months with resolution of his symptoms.

The patient was referred for an initial PET/CT scan in July of 2008 to evaluate for potential lymphoma (Fig. 1 Preview this figure

Figure 1: Nuclear Medicine
(left) PET coronal, (upper right) CT axial, and (lower right) PET/CT axial views of a 58 year-old man with (unknown) retroperitoneal fibrosis, undergoing initial PET/CT to evaluate for potential lymphoma. Diffuse moderate FDG activity corresponding to CT soft tissue abnormality is seen as an infiltrative soft tissue mass surrounding left common iliac artery and vein causing minimal compression of the vessels. (Performed 60 minutes after injection of FDG tracer, dose of 17.53 mCi, with 5 mm slice thickness, CT-based attenuation correction algorithm using two iterations and 8 subsets, 120 kVp, 112 mAs.)
). This study showed diffuse moderate FDG activity corresponding to the CT soft tissue abnormality also found on CT scan 1 month prior. A follow-up PET/CT scan performed 3 months later (4 months after onset of symptoms) demonstrated progression of the soft-tissue infiltration with involvement of the distal aorta and right common iliac vessels (Fig. 2 Preview this figure
Figure 2: Nuclear Medicine
(left) PET coronal, (upper right) CT axial, and (lower right) PET/CT axial views of a 58 year-old man with retroperitoneal fibrosis at diagnosis. Seen is circumferential soft-tissue thickening around the distal aorta and common iliac vessels, as well as associated diffuse moderate FDG activity within the soft tissue. (Performed 60 minutes after injection of FDG tracer, dose of 17.2 mCi, with 5 mm slice thickness, CT-based attenuation correction algorithm using two iterations and 8 subsets, 120 kVp, 280 mAs.)
). The metabolic activity within the soft tissue also increased slightly with a SUVmax of 5.9 compared to the initial scan SUVmax of 3.2.

The characteristic extension of the mass, the pattern of FDG uptake, and lack of lymph node involvement led to interpretation of the scan as suspicious for retroperitoneal fibrosis and less likely as lymphoma. The patient was placed on steroids for 3 months, when a third PET/CT scan was performed (Fig. 3 Preview this figure
Figure 3: Nuclear Medicine
(left) PET coronal, (upper right) CT axial, and (lower right) PET/CT axial views of a 58 year-old man with retroperitoneal fibrosis following steroid therapy. There is complete resolution of the soft tissue infiltration around the aorta and iliac vessels and complete resolution of the abnormal FDG activity. (Performed 60 minutes after injection of FDG tracer, dose of 16.7 mCi, with 5 mm slice thickness, CT-based attenuation correction algorithm using two iterations and 8 subsets, 120 kVp, 294 mAs.)
), demonstrating complete resolution of the soft tissue infiltration around the aorta and iliac vessels and complete resolution of the abnormal FDG activity.

  DISCUSSION
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Retroperitoneal fibrosis (RPF) is a rare condition characterized by chronic inflammatory tissue with appreciable fibrosis in the retroperitoneum which can expand to entrap various abdominal organs. The inflammatory component consists of lymphocytes, plasma cells, macrophages, and scattered eosinophils, while fibroblasts and myofibroblasts compose a sclerotic matrix of type I collagen (1). Though the true incidence of RPF is unknown, estimates range from 1 in 200,000 to 500,000 (2,3). The condition occurs in men two to three times as much as in women, and peak incidence occurs in patients between 40 to 60 years of age (4-6). However, at least 23 cases have been reported in children under 18 (7). Over two-thirds of RPF cases are considered idiopathic, and though its etiology remains unclear, it is thought to involve an immune-mediated response to severe atherosclerosis (1,5). One third of cases of RPF occur secondary to other causes including neoplasms, infections, hemorrhage, trauma, radiotherapy, surgery, and use of certain drugs (8,9). There is no evidence of an ethnic predilection or a familial pattern of this disease.

Patients typically present with insidious dull abdominal, lumbar, back or flank pain that becomes acute and colicky with ureteral involvement. Constitutional and non-specific GI symptoms are common, as is scrotal pathology (swelling, pain, hydrocele, varicocele) and finally deep vein thrombosis (1,10,11). Physical examination is generally unremarkable with the exception of hypertension, probably of renal origin. Due to non-specific findings, patients can present in renal failure secondary to obstructive uropathy and long-standing hydronephrosis before the correct diagnosis is made (11,12). Ureteral involvement is reported in 80 - 100% of cases (12), and indeed the late stage of RPF is characterized by progressive ureteral obstruction, bilateral reportedly in over half of RPF cases (13,14). Diagnosis usually depends on radiographic evaluation, CT and MRI being the modalities of choice (see later). Retroperitoneal biopsy is usually performed only in cases with atypical localizations or when clinical or laboratory findings suggest an underlying infection or malignancy (1).

Primary retroperitoneal neoplasms, such as lymphoma or sarcoma, and metastatic retroperitoneal tumors, such as breast and colon carcinomas, can also produce a desmoplastic reaction and thus mimic RPF (1). There are several case reports in the literature concerning the similar appearance of lymphoma and idiopathic RPF on CT and MRI (15-18); however, to our knowledge, the PET/CT findings of retroperitoneal fibrosis have been described in only one other case (19). Differentiating between lymphoma and RPF remains crucial, as implications for treatment differ drastically. Similarly, wherever retroperitoneal fibrosis is suspected, it is important to discern idiopathic from secondary forms of RPF, again due to treatment implications. Of particular importance is RPF secondary to malignancy, which is caused by infiltration of the retroperitoneum by malignant cells producing a pronounced desmoplastic and sclerotic reaction. Malignant RPF is estimated to account for8% of cases of RPF (5). Due to the poor prognosis of malignant RPF (20), an appreciation for its potential and an appreciation of the radiographic features are vital to the assessment of affected patients. By contrast, cases of idiopathic RPF portend a very favorable prognosis, though relapse is common after withdrawal of glucocorticoids, even several years after treatment, and therefore requires close monitoring (1).

As mentioned previously, diagnosis relies heavily on imaging. CT characteristically shows a paraspinal, well-demarcated retroperitoneal mass of varying thickness with irregular borders, isodense with muscle (21). It most commonly surrounds the anterior and lateral sides of the abdominal aorta and iliac arteries, often appearing at the level of L4-L5, spreading cranially towards the renal hila (1,10). Ureteral encasement, medial deviation, and obstruction are frequent sequelae, and other abdominal structures such as the IVC can similarly become enveloped by the retroperitoneal mass (1,8,10). The mass shows pronounced enhancement with contrast, a finding that varies with activity of the fibrotic process; that is, in early stages of the disease, contrast enhancement is striking, though in a chronic, advanced setting, little or no enhancement may be seen (1,21). Similar to other fibrotic processes, RPF manifests as a hypointensity on T1-weighted images. T2 signal, however, again varies with degree of disease activity, reflected by inflammation and edema. Chronic, inactive fibrosis will contain little edema and thus displays low-intensity signal on both T1 and T2. PET imaging has emerged as potentially useful in diagnosis, shown in a recent small study to correlate with mass thickness and CRP levels, though proper interpretation of increased FDG uptake in most clinical situations associated with RPF remains unclear (1,22,23). Additionally, several authors have also recently described the relevance of FDG PET in monitoring response to corticosteroid therapy in cases of RPF, with diminishing avidity correlating with decreases in inflammatory markers and subsequent increased radiotracer uptake signalling relapse of disease activity (21,23,24).

Characterizing differentiating features of idiopathic RPF from underlying malignancy by radiographic evidence has proven challenging. However, several findings that have been described to support the malignant RPF include a more cephalad distribution of a larger, bulkier mass, more likely to display mass effect, pushing the aorta and IVC anteriorly and the ureters laterally (25,26). Finally, underlying lymphoma tends to be homogenous and hypovascular with less contrast enhancement on CT, is associated with lymphadenopathy, and shows speckled enhancement on T1, relatively lower intensity signal on T2, and delayed enhancement on dynamic MR (27,28). It is important to note that in cases of lymphoma, PET often has discrete FDG avid nodes, but can also atypically appear as a diffuse soft tissue mantle, mimicking the findings of RPF.

In conclusion, upon suspicion of idiopathic retroperitoneal fibrosis, one must be cognizant that the imaging findings of this process are often mimicked by lymphoma and by RPF due to secondary causes, malignancy in particular. These entities must be ruled out before the diagnosis is made.

  TEACHING POINT
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In patients undergoing PET/CT evaluation for suspected lymphoma, particularly those that display imaging characteristics such as pronounced enhancement, non-bulky nature, and periaortic location, retroperitoneal fibrosis should be entertained as a potential diagnosis, as this process may mimic other imaging findings of neoplastic disease.








  FIGURES
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Display figure 1 in original size

Figure 1: (left) PET coronal, (upper right) CT axial, and (lower right) PET/CT axial views of a 58 year-old man with (unknown) retroperitoneal fibrosis, undergoing initial PET/CT to evaluate for potential lymphoma. Diffuse moderate FDG activity corresponding to CT soft tissue abnormality is seen as an infiltrative soft tissue mass surrounding left common iliac artery and vein causing minimal compression of the vessels. (Performed 60 minutes after injection of FDG tracer, dose of 17.53 mCi, with 5 mm slice thickness, CT-based attenuation correction algorithm using two iterations and 8 subsets, 120 kVp, 112 mAs.)

Figure 1: Nuclear Medicine (Open in original size)
(left) PET coronal, (upper right) CT axial, and (lower right) PET/CT axial views of a 58 year-old man with (unknown) retroperitoneal fibrosis, undergoing initial PET/CT to evaluate for potential lymphoma. Diffuse moderate FDG activity corresponding to CT soft tissue abnormality is seen as an infiltrative soft tissue mass surrounding left common iliac artery and vein causing minimal compression of the vessels. (Performed 60 minutes after injection of FDG tracer, dose of 17.53 mCi, with 5 mm slice thickness, CT-based attenuation correction algorithm using two iterations and 8 subsets, 120 kVp, 112 mAs.)

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Display figure 2 in original size
Figure 2: (left) PET coronal, (upper right) CT axial, and (lower right) PET/CT axial views of a 58 year-old man with retroperitoneal fibrosis at diagnosis. Seen is circumferential soft-tissue thickening around the distal aorta and common iliac vessels, as well as associated diffuse moderate FDG activity within the soft tissue. (Performed 60 minutes after injection of FDG tracer, dose of 17.2 mCi, with 5 mm slice thickness, CT-based attenuation correction algorithm using two iterations and 8 subsets, 120 kVp, 280 mAs.)

Figure 2: Nuclear Medicine (Open in original size)
(left) PET coronal, (upper right) CT axial, and (lower right) PET/CT axial views of a 58 year-old man with retroperitoneal fibrosis at diagnosis. Seen is circumferential soft-tissue thickening around the distal aorta and common iliac vessels, as well as associated diffuse moderate FDG activity within the soft tissue. (Performed 60 minutes after injection of FDG tracer, dose of 17.2 mCi, with 5 mm slice thickness, CT-based attenuation correction algorithm using two iterations and 8 subsets, 120 kVp, 280 mAs.)

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Display figure 3 in original size
Figure 3: (left) PET coronal, (upper right) CT axial, and (lower right) PET/CT axial views of a 58 year-old man with retroperitoneal fibrosis following steroid therapy. There is complete resolution of the soft tissue infiltration around the aorta and iliac vessels and complete resolution of the abnormal FDG activity. (Performed 60 minutes after injection of FDG tracer, dose of 16.7 mCi, with 5 mm slice thickness, CT-based attenuation correction algorithm using two iterations and 8 subsets, 120 kVp, 294 mAs.)

Figure 3: Nuclear Medicine (Open in original size)
(left) PET coronal, (upper right) CT axial, and (lower right) PET/CT axial views of a 58 year-old man with retroperitoneal fibrosis following steroid therapy. There is complete resolution of the soft tissue infiltration around the aorta and iliac vessels and complete resolution of the abnormal FDG activity. (Performed 60 minutes after injection of FDG tracer, dose of 16.7 mCi, with 5 mm slice thickness, CT-based attenuation correction algorithm using two iterations and 8 subsets, 120 kVp, 294 mAs.)

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Display figure 4 in original size
Figure 4: Summary table for retroperitoneal fibrosis

Figure 4: Table (Open in original size)
Summary table for retroperitoneal fibrosis

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Display figure 5 in original size
Figure 5: Differential diagnosis table for retroperitoneal fibrosis

Figure 5: Table (Open in original size)
Differential diagnosis table for retroperitoneal fibrosis

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  REFERENCES
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  ABBREVIATIONS
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CT - Computed Tomography
FDG - Fluorodeoxyglucose
RPF - Retroperitoneal Fibrosis
SUVmax - Standardized uptake value (maximum value)









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