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DOI:10.2214/AJR.05.0985
AJR 2007; 188:W44-W48
© American Roentgen Ray Society

Clinical Observations

Biliary Inflammatory Pseudotumor: Imaging Features in Seven Patients

Mitchell E. Tublin1, A. James Moser2, J. Wallis Marsh2 and Thomas Clark Gamblin2

1 Department of Radiology (AI), University of Pittsburgh Medical Center, Presbyterian-Shadyside (Presbyterian Campus), 200 Lothrop St., Pittsburgh, PA 15213.
2 Department of Surgery, University of Pittsburgh Medical Center, Presbyterian-Shadyside (Presbyterian Campus), Pittsburgh, PA 15213.

Received June 9, 2005; accepted after revision August 2, 2005.

 
Address correspondence to M. E. Tublin (tublinme{at}upmc.edu).

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Abstract
Top
 Abstract
Introduction
 Materials and Methods
 Results
 Discussion
 References
 
OBJECTIVE. Hepatic biliary pseudotumor is a benign, rare, and poorly understood tumor that is typically diagnosed after aggressive surgical intervention. The purpose of this report is to describe the clinical and imaging features of this tumor in seven patients.

CONCLUSION. Although secondary clinical features may rarely suggest inflammatory pseudotumor, the typical presenting symptom—painless obstructive jaundice—is indicative of malignancy. The imaging appearance of hepatic hilar biliary pseudotumor is also indistinguishable from that of cholangiocarcinoma.

Keywords: biliary disease • cholangiocarcinoma • hepatic biliary pseudotumor • hepatobiliary imaging • MRCP • pseudotumor


Introduction
Top
 Abstract
 Introduction
Materials and Methods
 Results
 Discussion
 References
 
Inflammatory pseudotumor is a rare benign tumor characterized by an admixture of fibrovascular tissue and a cellular infiltrate of plasma cells, eosinophils, and histiocytes. Although the clinical course and histology of these lesions are benign, their appearance throughout the body mimics that of an aggressive malignancy. The lack of specificity of the imaging appearance of hepatic parenchymal inflammatory pseudotumor has been described in several reports [1-5]. The diagnosis of hepatic parenchymal pseudotumor, however, is often possible after imaging-directed percutaneous core biopsy [6-9]. This has not been the case with hepatic biliary pseudotumors.

Although the histology of biliary inflammatory pseudotumors is identical to that of pseudotumors found throughout the body, the infiltrating growth pattern of biliary pseudotumor typically precludes percutaneous biopsy. Moreover, the primary presenting symptom of this tumor (obstructive jaundice), its ERCP appearance (biliary stricture), and the cellular atypia that is frequently documented at preoperative biliary brushing strongly suggest cholangiocarcinoma. Indeed, in sporadic small case series and case reports in the surgery and pathology literature, biliary pseudotumors have been diagnosed only after laparotomy and aggressive surgical resection [9-12]. To our knowledge, the imaging appearance of isolated hepatic biliary pseudotumor has not been reported in the radiology literature.

The purpose of this study was to report the appearance and clinical features of hepatic hilar pseudotumor in seven patients; the ultimate goal of the review is to determine whether there are distinct features of this inflammatory tumor that might allow it to be differentiated from cholangiocarcinoma before aggressive surgery.


Materials and Methods
Top
 Abstract
 Introduction
 Materials and Methods
Results
 Discussion
 References
 
Patients with hilar biliary pseudotumors were identified after a computerized search of a pathology database and an informal review of personal surgical logs. The computer search of all pathology records of inflammatory pseudotumor specimens was restricted to the PACS era at the University of Pittsburgh Medical Center (1998 to the present). Seven patients with hilar pseudotumor (five women, two men) ranging in age from 29 to 68 years (mean, 41 years) were eventually identified. Computerized clinical records were reviewed; imaging studies were retrospectively reviewed by a fellowship-trained radiologist with extensive experience in biliary disease.

All patients were evaluated with contrast-enhanced helical CT. Scanning protocols had evolved over the preceding 6 years, although all studies were performed during the rapid administration (4 mL/s) of nonionic contrast media using a 5-mm collimation. Unenhanced, portal venous inflow, and hepatic parenchymal phase image sets were obtained in all patients. Five-minute delayed image sets of the liver were also acquired in three patients (Figs. 1A, 1B, 1C, 1D, 1E, 1F, 2A, 2B, 2C, 3A, 3B, and 3C).


Figure 1
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  		Fig. 1A —48-year-old woman with jaundice and suspected cholangiocarcinoma. Biliary inflammatory pseudotumor confirmed at right trisegmentectomy. Sonogram shows subtle isoechoic lesion at duct bifurcation (arrows).

 

Figure 2
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  		Fig. 1B —48-year-old woman with jaundice and suspected cholangiocarcinoma. Biliary inflammatory pseudotumor confirmed at right trisegmentectomy. Portal venous inflow phase image of triphasic contrast-enhanced CT shows poorly defined enhancing lesion at duct bifurcation (arrow) and moderate intrahepatic biliary duct dilatation.

 

Figure 3
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  		Fig. 1C —48-year-old woman with jaundice and suspected cholangiocarcinoma. Biliary inflammatory pseudotumor confirmed at right trisegmentectomy. Hepatic phase CT image shows enhancing lesion at duct bifurcation (arrow).

 

Figure 4
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  		Fig. 1D —48-year-old woman with jaundice and suspected cholangiocarcinoma. Biliary inflammatory pseudotumor confirmed at right trisegmentectomy. Delayed phase CT image shows contrast retention within hilar lesion (arrow).

 

Figure 5
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  		Fig. 1E —48-year-old woman with jaundice and suspected cholangiocarcinoma. Biliary inflammatory pseudotumor confirmed at right trisegmentectomy. Thick-slab MR cholangiopancreatography image shows biliary obstruction at bifurcation (arrows).

 

Figure 6
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  		Fig. 1F —48-year-old woman with jaundice and suspected cholangiocarcinoma. Biliary inflammatory pseudotumor confirmed at right trisegmentectomy. ERCP image, which corresponds to E, obtained during stent placement shows focal hilar biliary stricture.

 

Figure 7
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  		Fig. 2A —42-year-old woman with presumed Klatskin's tumor. Infiltrating biliary pseudotumor was diagnosed at surgical resection after adjuvant chemotherapy. Hepatic phase CT image shows infiltrating tumor along encased and compressed right portal vein (arrows).

 

Figure 8
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  		Fig. 2B —42-year-old woman with presumed Klatskin's tumor. Infiltrating biliary pseudotumor was diagnosed at surgical resection after adjuvant chemotherapy. Axial PET/CT image shows 18F-FDG activity along course of right portal vein (arrows). Left percutaneous biliary catheter (arrowhead) is in place.

 

Figure 9
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  		Fig. 2C —42-year-old woman with presumed Klatskin's tumor. Infiltrating biliary pseudotumor was diagnosed at surgical resection after adjuvant chemotherapy. Coronal PET/CT image shows linear 18F-FDG uptake within right lobe (arrows). Internal biliary stent (arrowhead) is also in place.

 

Figure 10
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  		Fig. 3A —68-year-old man with jaundice and abdominal pain. Brushing cytology during ERCP showed cellular atypia. Inflammatory pseudotumor was diagnosed after right hepatectomy and biliary diversion. Hepatic phase image of triphasic CT examination shows isoenhancing biliary hilar soft tissue (arrow) and intrahepatic duct dilatation after stent placement.

 

Figure 11
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  		Fig. 3B —68-year-old man with jaundice and abdominal pain. Brushing cytology during ERCP showed cellular atypia. Inflammatory pseudotumor was diagnosed after right hepatectomy and biliary diversion. Delayed phase image shows mild retention of contrast material within hilar soft tissue (arrow).

 

Figure 12
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  		Fig. 3C —68-year-old man with jaundice and abdominal pain. Brushing cytology during ERCP showed cellular atypia. Inflammatory pseudotumor was diagnosed after right hepatectomy and biliary diversion. ERCP image shows short-segment malignant-appearing hilar stricture (arrows).

 
Additional cross-sectional imaging was performed in four of the seven patients. Sonography, using a variety of platforms and 3- to 4-MHz probes, was performed in four patients. MR cholangiopancreatography (MRCP) was also performed in one of these four patients; one other patient also underwent two combined PET/CT examinations for tumor staging. Preoperative pre-biliary stent ERCP examinations of five patients were available for direct review. The ERCP report of a sixth patient was also reviewed.


Results
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 Abstract
 Introduction
 Materials and Methods
 Results
Discussion
 References
 
Clinical Data
All patients presented with severe jaundice. Three of the seven patients also complained of right upper quadrant pain. One patient had a long-standing history of steroid-dependent Crohn's colitis; one patient presented with Crohn's colitis 1 year after resection of hilar biliary inflammatory pseudotumor—he was also thought at that time to have an undefined autoimmune disorder based on markedly elevated antinuclear antibody titers. One patient was diagnosed with scleroderma 1 year before she presented with jaundice. None of the patients had a history of primary sclerosing cholangitis.

Imaging Findings
Endoscopic cholangiography with brushings and stent placement were performed initially in all patients. All brushings yielded inconclusive results. Cholangiography showed short-segment, high-grade strictures in five patients (Figs. 1F and 3C); in one patient, a short-segment stricture was shown just proximal to the bifurcation. MR cholangiopancreatography (MRCP) in one patient showed marked intrahepatic biliary obstruction, but the obstructing lesion was not visualized (Fig. 1E).

Sonography examination showed a subtle isoechoic hilar lesion in one patient (Fig. 1A); isolated intrahepatic biliary duct dilatation without extrahepatic duct dilatation was identified in two patients. An obliterated right portal vein within an otherwise normal-appearing liver was the only sonographic manifestation of hilar and right biliary inflammatory pseudotumor in one patient. The PET/CT examination in that patient showed 18F-FDG-avid linear infiltrating soft tissue within the posterior segment (Figs. 2A, 2B, and 2C). This finding was erroneously thought to be malignant tumor thrombus. After adjuvant chemotherapy, 18F-FDG avidity decreased for what was presumed to be central and peripheral cholangiocarcinoma on the basis of cholangiographic findings, atypical brushings, and initial metabolic activity at PET.

CT examination depicted subtle infiltrating hilar soft tissue in six of the seven patients (Figs. 1B, 1C, and 3A). Soft tissue appeared to retain contrast material in the three CT examinations in which delayed images were obtained (Fig. 1D). In one patient, hilar soft tissue was inseparable from a gallbladder fossa mass. Infiltrating soft tissue extended into the posterior segment in one patient (subsequently imaged at PET/CT), anterior segment in one patient, and lateral segment in one patient.

Surgical Findings
All patients were subsequently diagnosed with infiltrating biliary or peribiliary pseudotumor at laparotomy. Extrahepatic biliary and hepatic resections with Roux-en-Y reconstruction were performed in four patients. Isolated extrahepatic biliary resection and biliary diversion were performed in two patients. Long-term biliary stents were placed in one patient in whom frozen sectioning of multiple intraoperative Tru-Cut needle (Baxter Healthcare) biopsies of an infiltrating pancreatic and hilar mass revealed no malignancy.

Five patients remain disease-free 1-6 years after resection. Follow-up imaging showed decreased hilar and pancreatic soft tissue in one patient on corticosteroid and azothioprine therapy. Two patients had unexplained progressive liver dysfunction after trisegmentectomy. Liver biopsies performed 1 year after resection showed mild fibrosis: one of these patients died as a complication of the liver biopsy, and the second patient died after a myocardial infarction.


Discussion
Top
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
References
 
Inflammatory pseudotumors are rare lesions that are often mistaken for aggressive malignancy. Although originally described in the lung, a variety of extrapulmonary sites have been reported, primarily in the pathology literature. Hepatic parenchymal pseudotumors have been reported in limited radiology series [1-5]; although the appearance is nonspecific, a diagnosis may be made at imaging-directed core biopsy [6-9]. On the other hand, an extremely uncommon subtype—hepatic biliary pseudotumor— has rarely, if ever, been diagnosed before laparotomy [10-12].

In our series of seven patients with biliary pseudotumor, no patient was, in fact, thought to have a nonneoplastic lesion before biliary diversion. The clinical features of biliary inflammatory pseudotumor (jaundice, rarely pain, fever, or anemia) mimicked those of biliary neoplasm. Prospective preoperative interpretation of multiple imaging studies in these patients was also misleading: Intrahepatic biliary duct dilatation and subtle infiltrating hilar tumor were thought to be pathognomonic features of cholangiocarcinoma.

Unfortunately, no particularly distinctive clinical feature was identified in any of the patients in our series even at retrospective review. A link between chronic local inflammation— within the context of primary sclerosing cholangitis or recurrent pyogenic cholangitis—has been reported in the past [13, 14]. None of the patients in our series had a documented history of cholangitis before surgery. Although this association may still exist, it is certainly not a helpful clinical feature: A history of cholangitis should increase the index of suspicion of superimposed cholangiocarcinoma. In retrospect, the most helpful indicator of benign disease was the repeatedly negative frozen resection margins that invariably were reported during tedious exploration of infiltrating porta hepatis tumor. Aggressive hepatic resection and biliary diversions were still performed, however, because of the initial high clinical suspicion of malignancy.

All of the imaging features of this benign entity were identical to those of hilar cholangiocarcinoma as well. In the three patients who underwent delayed imaging, hilar soft tissue retained contrast material—a feature that gave the interpreting radiologist more confidence in the diagnosis of cholangiocarcinoma. Delayed contrast retention has been previously reported in cases of hepatic parenchymal pseudotumor: Retention of contrast material is likely a reflection of the fibrotic component of pseudotumor [6]. The PET/CT features of inflammatory pseudotumor in one patient also erroneously indicated malignancy. Indeed, the 18F-FDG uptake noted along the right bile duct in this patient was used as a justification for adjuvant chemoembolization therapy for "imaging-positive" (and brushing inconclusive) infiltrating cholangiocarcinoma. Likewise, in our series, MRCP and sonography offered no useful differentiating information: Isolated intrahepatic biliary duct dilatation was invariably considered a secondary finding of extrahepatic biliary tumor.

In conclusion, although hilar biliary pseudotumor might be considered in the differential diagnosis of an obstructing extrahepatic biliary lesion, there are no distinctive clinical or imaging features that allow it to be differentiated from cholangiocarcinoma before laparotomy. Awareness of this rare but benign entity, however, might prompt a more limited operative approach when intraoperative resection margins of palpable disease are repeatedly negative for tumor.


References
Top
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 

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