Xanthogranulomatous Cholecystitis

Published on 18/07/2015 by admin

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 XGC cannot be confidently distinguished from gallbladder carcinoma radiologically

• CT: GB wall may be focally or diffusely thickened 

image Low-attenuation intramural nodules and bands (corresponding to foamy cell infiltrate and abscesses)
image Pericholecystic fluid and inflammatory change ± fistulous tracts, abscesses, contained perforation, etc.
image Inflammation can extend to involve adjacent liver and mimic GB cancer with hepatic invasion
• MR: Gallbladder wall thickening (diffuse > focal) with intramural T2 hyperintensity

image Discrete intramural nodules appear T2 hyperintense and hypointense on T1WI and T1WI C+
image Thickened gallbladder wall and intramural nodules may show signal drop-out on out-of-phase images
image Thickened wall often demonstrates delayed enhancement on T1WI C+
• US: Hypoechoic nodules or bands within thickened GB wall

image Gallstones, sludge, echogenic intraluminal debris

PATHOLOGY

• Mucosal ulceration or rupture of Rokitansky-Aschoff sinuses → extravasation of bile into GB wall → phagocytosis of bile lipids → inflammation and xanthoma cell formation
• Gallstones always present and may play causative role

CLINICAL ISSUES

• Symptoms most often similar to acute cholecystitis, but can be chronic and mimic malignancy
• More common in females during 6th or 7th decade of life
• Only definitive treatment is cholecystectomy
image
(Left) Resected GB shows marked wall thickening image and an intramural abscess image. A portion of the adjacent liver image was resected because of the high intraoperative suspicion of GB cancer. Pathology revealed xanthogranulomatous cholecystitis (XGC).

image
(Right) Axial CECT shows a thickened wall of the gallbladder, especially the fundus image, with an indistinct border with the liver. While the appearance was concerning for gallbladder cancer, this was found to be XGC at cholecystectomy.
image
(Left) Axial CECT of a patient with RUQ pain shows marked irregular GB wall thickening image, intramural low attenuation image, and several pericholecystic collections image. Low-attenuation intramural nodules in XGC are due to either abscesses or xanthogranulomas.

image
(Right) Axial NECT in a patient with chronic abdominal pain shows a distended, thick-walled gallbladder exhibiting indistinct margins with the liver image. While this was worrisome for carcinoma, XGC was confirmed at surgery.

TERMINOLOGY

Abbreviations

• Xanthogranulomatous cholecystitis (XGC)

Synonyms

• Fibroxanthogranulomatous cholecystitis; xanthogranulomatous cholangitis

Definitions

• Rare inflammatory disorder of gallbladder (GB) characterized by accumulation of lipid-laden macrophages and fibrous tissue

IMAGING

General Features

• Best diagnostic clue

image GB wall thickening with intramural low-attenuation/hypoechoic nodules or bands corresponding to foamy cell infiltrates or abscesses
• Location

image GB wall
image Pericholecystic space
image Main lobar hepatic fissure

Imaging Recommendations

• Best imaging tool

image CECT or MR

CT Findings

• Imaging findings overlap with acute cholecystitis, chronic cholecystitis, and gallbladder carcinoma

image GB wall may be focally or diffusely thickened (± loss of normal wall definition)

– GB wall may demonstrate poor enhancement
image Low-attenuation intramural nodules and bands corresponding to foamy cell infiltrate and areas of necrosis/abscess

– Intramural nodule may occupy > 60% of wall surface
– Most unique imaging feature for XGC
image Pericholecystic fluid, inflammatory change, and induration ± fistulous tracts, abscesses, contained perforation, etc.

– Inflammation can extend to involve adjacent liver (blurring margin between GB and liver), and thus mimic GB cancer with hepatic invasion
image Adjacent focal hyperperfusion of liver/transient hepatic attenuation difference (THAD)
image Gallstones almost always present but not always visible on CT
image Local lymphadenopathy frequent (usually reactive due to inflammation)
• Helpful findings to differentiate XGC from GB carcinoma

image More commonly diffuse wall thickening (rather than focal)
image Continuous mucosal line of enhancement
image Intramural low-attenuation nodules
image Absence of hepatic invasion
image Absence of biliary dilatation
image Presence of 3 findings leads to 83% sensitivity, 100% specificity, and 91% accuracy for differentiation of XGC from GB carcinoma

MR Findings

• Gallbladder wall thickening (diffuse > focal) with intramural T2 hyperintensity

image Areas of xanthogranulomatosis are iso- or slightly T2 hyperintense
image Areas of necrosis and abscess are highly T2 hyperintense
image Discrete intramural nodules (akin to CT) appear T2 hyperintense and hypointense on T1WI and T1WI C+
image Thickened gallbladder wall (and intramural nodules) may show subtle signal drop-out on out-of-phase images as a result of intracellular lipid
• Thickened wall often demonstrates delayed enhancement on T1WI C+ with preservation of continuous mucosal enhancement

image Gallbladder cancer typically demonstrates earlier peak enhancement
• Both gallbladder cancer and XGC typically demonstrate restricted diffusion on DWI, but malignancy typically demonstrates lower ADC values
• Gallstones almost always present (low signal on all pulse sequences)
• Surrounding infiltration into pericholecystic fat and liver (usually high signal on T2WI)

Ultrasonographic Findings

• GB wall thickening (may rarely appear focal and mass-like, mimicking carcinoma)
• Hypoechoic nodules or bands within GB wall
• Echogenic pericholecystic fat (reflecting pericholecystic inflammation)
• Gallstones, sludge, echogenic intraluminal debris
• Pericholecystic fluid collections
• Hypoechoic areas of inflammation in liver adjacent to gallbladder fossa

DIFFERENTIAL DIAGNOSIS

Gallbladder Carcinoma

• Soft tissue mass extending from GB wall with invasion of liver and porta hepatis

image May appear as diffuse or focal wall thickening or discrete polypoid mass
• Unlike XGC, frequently results in obstruction of common duct, direct invasion of liver, and bulky periportal lymphadenopathy
• In most cases cannot be distinguished from XGC based on imaging alone, and distinction only possible after cholecystectomy
• More likely than XGC to present with anorexia, weight loss, palpable mass, and jaundice

Gallbladder Adenomyomatosis

• Mural GB wall thickening secondary to exaggeration of Rokitansky-Aschoff sinuses and smooth muscle proliferation

image Can result in focal/diffuse wall thickening or discrete mass (usually at GB fundus)
image Echogenic foci in GB wall with “comet tail” artifacts on ultrasound
image Could theoretically mimic XGC with discrete intramural nodules on CT or MR
• Unlike XGC, patients with adenomyomatosis are asymptomatic

Gangrenous Cholecystitis

• Asymmetrically thickened gallbladder wall on US with pericholecystic fluid, ulceration or irregularity of wall, intraluminal linear sloughed membranes, or frank perforation

image Fat stranding and inflammation of pericholecystic fat on CT/MR with echogenic pericholecystic fat on US
• XGC may not be readily distinguishable from acute cholecystitis (either uncomplicated or gangrenous), with both entities often exhibiting similar clinical symptoms

PATHOLOGY

General Features

• Etiology

image Pathogenesis

– Mucosal ulceration or rupture of Rokitansky-Aschoff sinuses → extravasation of bile into GB wall → phagocytosis of bile lipids → inflammation and xanthoma cell formation
image Gallstones present in almost all patients and likely plays a causative role

– Gallstone → obstruction and stasis → increased intraluminal pressure → mucosal ulceration or rupture of Rokitansky-Aschoff sinuses
• Associated abnormalities

image Proposed association and relationship with GB carcinoma (uncertain)

– Gallbladder cancer present in about 6% of cases of XGC
image Questionable association with cholangiocarcinoma
image Biliary obstruction (less common than GB cancer)

Staging, Grading, & Classification

• Stage I

image Confined to GB and pericholecystic space
• Stage II

image Involvement of lower biliary tree, duodenum, or colon

Gross Pathologic & Surgical Features

• GB wall thickening

image Xanthogranulomatous foci in GB wall
image Ulcerated mucosa
• Serosa covered with dense fibrous adhesion
• Frequent infiltration of surrounding pericholecystic fat and liver
• Gallstones (seen in all cases)

Microscopic Features

• Abundant lipid-laden macrophages, fibroblasts, acute and chronic inflammatory cells

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Symptoms often acute and similar to acute calculous cholecystitis: RUQ pain, fever, leukocytosis
image Symptoms can also mimic malignancy, including obstructive jaundice
• Other signs/symptoms

image Symptoms may be chronic and persist for years
image Biliary colic
image Jaundice (secondary to biliary obstruction)
• Laboratory findings

image ↑ WBC
image ↑ bilirubin and alkaline phosphatase (secondary to choledocholithiasis or Mirizzi syndrome)

Demographics

• Age

image Usually 6th or 7th decade of life
• Gender

image F > M
• Epidemiology

image Incidence: 0.7% of symptomatic GB diseases in USA

– Reported incidence as high as 10% in Japan and India

Natural History & Prognosis

• Active and destructive process that may persist for years and can lead to significant morbidity
• High rate of complications (as high as 30%)

image Perforation
image Pericholecystic/hepatic abscess
image Fistula formation to adjacent organs (e.g., colon or duodenum) or skin surface
image Mirizzi syndrome
• Prospective diagnosis is uncommon, and in many cases, diagnosis only made at cholecystectomy, with preoperative diagnosis of either acute cholecystitis or gallbladder carcinoma

Treatment

• Only definitive treatment is cholecystectomy

image Open cholecystectomy is preferred due to extent of inflammation and frequent preoperative suspicion of gallbladder carcinoma
image Requires complete resection of adjacent xanthogranulomatous tissue and liver (if involved)
image Requires intraoperative frozen section to differentiate from GB carcinoma

DIAGNOSTIC CHECKLIST

Consider

• XGC cannot be confidently distinguished from GB carcinoma radiologically and definitive diagnosis is almost always only possible after cholecystectomy

Image Interpretation Pearls

• Consider XGC if gallbladder is diffusely thickened with low-attenuation nodules or bands in gallbladder wall (representing xanthogranulomatous inflammation or microabscesses)
image
(Left) Axial CECT of an asymptomatic 75-year-old man shows irregular GB wall thickening image and intramural low attenuation image.

image
(Right) Ultrasound of the same patient shows asymmetric wall thickening image, sludge image, and a stone within the GB neck image (and focal fatty sparing image). Preoperative differentiation between GB carcinoma and xanthogranulomatous cholecystitis (XGC) is often difficult, but the absence of ductal dilatation may suggest a chronic inflammatory process rather than neoplasia.
image
(Left) Axial CECT of a 64-year-old woman with chronic right upper quadrant pain shows gallbladder wall thickening image as well as focal mural low attenuation image. At pathology, the low attenuation corresponded to an area of necrosis filled with foamy macrophages. Note the increased hepatic perfusion, representing a THAD image.

image
(Right) Ultrasound of the same patient shows GB wall thickening, multiple small cystic areas of mural necrosis along the anterior wall image, and sludge filling the GB lumen image.
image
(Left) Axial CECT of an asymptomatic 88-year-old man performed for aortic aneurysm evaluation shows incidental irregular GB wall thickening image and intramural low attenuation image.

image
(Right) US of the same patient shows wall thickening image, sludge image, and intramural hypoechogenicity image. Laparoscopic cholecystectomy confirmed chronic XGC. Note that the extensive adhesions and wall thickening associated with chronic inflammation often necessitate open cholecystectomy.

SELECTED REFERENCES

1. Lee, NK, et al. Diffusion-weighted magnetic resonance imaging for non-neoplastic conditions in the hepatobiliary and pancreatic regions: pearls and potential pitfalls in imaging interpretation. Abdom Imaging. 2014. [ePub].

 

Revzin, MV, et al. The gallbladder: uncommon gallbladder conditions and unusual presentations of the common gallbladder pathological processes. Abdom Imaging. 2014. [Epub ahead of print].

Kang, TW, et al. Differentiating xanthogranulomatous cholecystitis from wall-thickening type of gallbladder cancer: added value of diffusion-weighted MRI. Clin Radiol. 2013; 68(10):992–1001.

Zhao, F, et al. CT and MR features of xanthogranulomatous cholecystitis: an analysis of consecutive 49 cases. Eur J Radiol. 2013; 82(9):1391–1397.

Arora, A, et al. Xanthogranulomatous cholecystitis: characteristic CT findings. Indian J Gastroenterol. 2012; 31(2):85.

Shetty, GS, et al. Xanthogranulomatous cholecystitis: sonographic and CT features and differentiation from gallbladder carcinoma: a pictorial essay. Jpn J Radiol. 2012; 30(6):480–485.

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Klarenbeek, BR, et al. Percutaneous gallbladder drainage for xanthogranulomatous cholecystitis. Surg Laparosc Endosc Percutan Tech. 2008; 18(5):506–507.

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Iannicelli, E, et al. A case of xanthogranulomatous cholecystitis: integrated US-CT imaging. Radiol Med. 2002; 104(1–2):106–110.

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Kim, PN, et al. Xanthogranulomatous cholecystitis: radiologic findings with histologic correlation that focuses on intramural nodules. AJR Am J Roentgenol. 1999; 172(4):949–953.

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