A 76-year-old woman presented to the emergency department with intractable nausea and vomiting. Initial evaluation included an abdominal computed tomographic (CT) scan that demonstrated nonspecific intrahepatic ductal dilatation and a normal hepatobiliary scan.
Hootan C. Roozrokh, MD
, Department of General Surgery,
Kurt R. Stahlfeld, MD, FACS
, Program Director, Department of General Surgery,
Amy Maszkiewicz, MD
, Department of Radiology, and
Beatrice A. Carlin, MD
, Program Director, Department of Radiology, Mercy Hospital of
Pittsburgh, Pittsburgh, PA.
A 76-year-old woman presented to the emergency department with
intractable nausea and vomiting. Initial evaluation included an
abdominal computed tomographic (CT) scan that demonstrated
nonspecific intrahepatic ductal dilatation (Figure 1) and a normal
hepatobiliary scan (not shown). The patient was treated
conservatively. A few months later, she developed vague abdominal
pain, fever, leukocytosis, and elevated pancreatic enzymes.
Abdominal sonography was interpreted as normal. Repeat abdominal CT
demonstrated a 4.5-cm irregularly enhancing cystic mass within the
gallbladder fundus, persistent intrahepatic ductal dilatation, and
common bile duct dilatation (Figure 2). A preliminary diagnosis of
gallbladder carcinoma was made. Pathology following open
cholecystectomy found no evidence of malignancy.
Xanthogranulomatous cholecystitis (XGC)
In October 2000, an emergent abdominal CT scan showed mild
nonspecific intrahepatic ductal dilatation. Retrospectively, an
eccentric gallbladder mass was evident at this time, but the mass
mimicked contrast within the hepatic flexure of the colon (Figure
1). When the patient was re-evaluated in January 2001 due to
worsening symptoms, abdominal sonography was interpreted initially
as normal, but repeat abdominal CT revealed a complex cystic mass
within the gallbladder fundus as well as mild intrahepatic and
extrahepatic dilatation (Figure 2). After a surgical diagnosis was
made, review of the earlier ultrasound determined that what was
originally thought to be debris within the adjacent stomach was
actually an atypically appearing fundal gallbladder mass (Figure
Gross pathologic examination showed a large, chronically
inflamed gallbladder measuring 5 * 4 cm and cholelithiasis (Figure
4). Histologic analysis found xanthogranulomatous cholecystitis and
malacoplakia, with focal microscopic sinus formation (Figure
Christensen and Ishak
first coined the pathologic diagnosis of XGC, describing seven
patients with pseudotumors of the gallbladder. Many pseudonyms such
as ceroid granulomas, ceroid-like histiocytosis, and
fibroxanthogranulomatous inflammation existed before the Armed
Forces Institute of Pathology formally characterized XGC.
The reported incidence of XGC varies from 0.66% to 1.8%.
Preoperative diagnosis is rarely made. Frequently, the patients are
misdiagnosed as having cholelithiasis or a primary cancer.
Xanthogranulomatous cholecystitis was initially thought to be a
malignant disease process; many studies now show that this disease
behaves in a similar fashion to another benign disorder,
xanthogranulomatous pyleonephritis (XGP).
Both of these disease processes are associated with chronic
infection and calculi that induce histiocytes to begin the
first speculated that gallbladder calculi creating mechanical
trauma along the mucosa incite the inflammatory response. The
inflammation causes lechitin in the bile to react with free fatty
acids, producing lysolecithin. This results in further damage to
the gallbladder mucosa, initiating a destructive cascade.
This inflammatory process progresses from cholecystitis to XGC when
the Rokitansky-Aschoff sinuses become occluded and inspissated with
bile and mucin.
When the wall tension of the distended gallbladder exceeds its
natural compliance, the Rokitansky-Aschoff sinuses rupture,
spreading inflammation to the adjacent tissues.
The degradation of bile results in phagocytosis of insoluble
cholesterol and bile salts by histiocytes, giving the
characteristic XGC appearance of macrophage-laden and foamy
Xanthogranulomatous cholecystitis has many similarities to its
original counterpart, but it is generally more severe. Similar
pathogens found in both XGC and cholecystitis include
Since XGC is usually not suspected preoperatively, neither are its
sequelae. Complications such as gallbladder perforation, hepatic
abscess, biliary ductal stricture and obstruction, ascending
cholangitis, and biliary fistula are associated more commonly with
Although imaging modalities
such as CT, ultrasound, endoscopic retrograde
cholangiopancreatography, and percutaneous transhepatic
cholangiography aid in diagnosing the complications of XGC, only
histologic examination of a surgical specimen will accurately give
In addition, there is an increased association of underlying
carcinoma in patients with XGC than in those with ordinary
Due to the high prevalence of primary fistulas, cholecystectomy
is accompanied by excision of all surrounding xanthogranulomatous
tissue to assure removal of any fistula.
Since dissection in Calot's triangle is difficult due to the dense
fibrosis, laparoscopic surgery is rarely successful.
Some have advocated subtotal cholecystectomy with drainage of the
subhepatic space to decrease the likelihood of iatrogenic injury.
Also, due to the high incidence of associated fistulas and
malignancies, many advocate intraoperative cholangiography.
Xanthogranulomatous cholecystitis is an uncommon variant of
cholecystitis that is rarely diagnosed preoperatively. It is
generally more virulant than ordinary cholecystitis, but the same
pathogens are isolated from both, implicating a primarily
histiocytic driven inflammatory mechanism as the cause of