Ampullary Xanthogranulomatous Inflammation

Biju Pottakkat
, Rajan Saxena
, Hirdaya Hulas Nag
, Neeraj Kumari
, Narendra Krishnani
Department of Surgical Gastroenterology and
Department of Pathology,
Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGIMS). Lucknow, India
Context Xanthogranulomatous inflammation
commonly affects the gallbladder. To date,
there have been no reports of xanthogranul-
omatous inflammation of the ampulla.
Case report A 48-year-old female presented
to us with fever, jaundice and a palpable
gallbladder. Evaluation revealed features of
periampullary malignancy. The patient
underwent a Whipple’s pancreaticoduoden-
ectomy. Histopathology revealed a
xanthogranulomatous inflammation affecting
the ampulla and the gallbladder.
Conclusion Xanthogranulomatous inflam-
mation should be added to the differential
diagnosis of patients presenting with a
suspected periampullary lesion accompanied
by a thick-walled gallbladder.
Xanthogranulomatous inflammation can
affect the gallbladder, kidney, bone, ovary,
pancreas, lymph nodes and soft tissues, the
gallbladder being the commonest site among
these. Commonly, the disease is correctly
identified on histopathological examination.
Fine needle aspiration cytology can also
diagnose the condition. Patients with
xanthogranulomatous cholecystitis can
present with obstructive jaundice which is
seen in up to 23 % of cases [1]. The causes of
obstructive jaundice include common duct
stones, extrinsic compression of the common
bile duct or associated malignancy. We report
a case of obstructive jaundice due to
xanthogranulomatous inflammation affecting
the ampulla of Vater, a clinical situation
hitherto undescribed.
A 48-year-old female was referred to our
centre complaining of fever and jaundice. Six
weeks before presenting to us, she had
experienced recurrent attacks of high-grade
fever up to 38.9°C associated with jaundice,
dark urine, pale stools and pruritus. She was
initially treated by a local physician with
antibiotics and her fever subsided
temporarily, but the jaundice gradually
progressed. She did not experience any pain,
weight loss or anorexia. Clinical examination
showed deep jaundice and scratch marks all
over the body. Abdominal examination
revealed a 3 cm firm hepatomegaly and a
tense palpable gallbladder. She had an
ultrasonogram which showed bilobar
intrahepatic biliary dilatation with dilatation
of the common bile duct extending to the
ampullary region. The gallbladder was
distended with a single calculus of 28 mm and
the gallbladder wall was 5 mm thick. No mass
was seen at the lower common bile duct or the

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JOP. Journal of the Pancreas - - Vol. 7, No. 2 - March 2006. [ISSN 1590-8577]
pancreas on ultrasonogram. At the time of
referral, she had a bilirubin level of 7.9 mg/dL
(reference range: 0.10-1.30 mg/dL), alkaline
phosphatase of 1,901 IU/L (reference range:
35-150 IU/L), SGOT of 187 IU/L (reference
range: 5-40 IU/L), and SGPT of 284 IU/ L
(reference range: 5-40 IU/L). With a
diagnosis of malignant biliary obstruction, she
was referred to us for further management.
She underwent a contrast-enhanced CT scan
(CECT) of the abdomen. It revealed bilobar
intrahepatic biliary radical dilatation and a
dilated common bile duct extending to the
lower portion. The pancreatic duct was also
dilated. No mass was seen on CECT (Figure
1). A periampullary malignancy was
suspected. During this period, she had another
attack of cholangitis which did not respond to
broad spectrum antibiotics, and her bilirubin
level rose to 14.8 mg/dL. Her cholangitis was
managed with an endoscopic papillotomy and
drainage. Endoscopy did not reveal any
ampullary lesion (endoscopic picture not
available). A common bile duct bulge was
seen at the medial wall of the second part of
the duodenum. Multiple biopsies were taken
from the ampullary region which did not
show malignant cells or any other specific
pathology. With a strongly suspected but
unproven diagnosis of periampullary
pancreaticoduodenectomy (PD) two weeks
At laparotomy, a hard growth was palpable at
the ampullary region. A few soft
retropancreatic lymph nodes (the largest
measuring 2 cm) were also present. The
gallbladder was distended and thick-walled
and there was a 30 mm calculus in the lumen.
There was also intense pericholedochitis
involving the first part of duodenum. The
head and uncinate process of the pancreas
were firm and fibrotic. A classical Whipple’s
PD was carried out. Postoperative recovery
was uneventful and the patient was
discharged on the 8
postoperative day.
Gross examination of the resected PD
specimen revealed an irregularly thick-walled
gallbladder with a maximum thickness of 10
mm. There was a necrotic area of 30x25 mm
at the fundus of the gallbladder. A grayish
white area of thickening measuring 20x5 mm
was seen at the ampulla of Vater. The
common bile duct wall was 3 mm thick.
Microscopy of the ampulla revealed
ulceration and dense panmural inflammatory
cell infiltrate comprised of plasma cells,
lymphocytes and sheets of foamy histiocytes
with focal antral metaplasia of the lining
epithelium (Figure 2). These changes
extended from the gallbladder through the
common bile duct to the ampulla. At the
ampulla, the inflammatory cell infiltrates
Figure 1. Contrast-enhanced CT scan (CECT) showing
a dilated common bile duct, a dilated pancreatic duct,
and a thick walled gallbladder. There is no mass lesion.
Figure 2. Histopathological examination of the
ampullary region showing Brunner’s glands with
mixed inflammatory cell infiltrate (plasma cells,
lymphocytes and histiocytes) consistent with
xanthogranulomatous inflammation.

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JOP. Journal of the Pancreas - - Vol. 7, No. 2 - March 2006. [ISSN 1590-8577]
extended transmurally from the duodenal
mucosa to the superficial pancreas. No
evidence of malignancy was seen. All the
lymph nodes showed reactive hyperplasia. A
inflammation was made.
Xanthogranulomatous cholecystitis is a
destructive form of chronic cholecystitis
characterized by the presence of grayish
nodules or streaks in the gallbladder wall,
mainly caused by lipid laden macrophages.
The disease can mimic gallbladder cancer and
can be locally advanced with involvement of
adjacent organs which may necessitate an
enbloc resection because of the difficulty in
distinguishing it from a carcinoma by pre-
operative investigations [2]. Involvement of
the common bile duct by the inflammatory
process can result in obstructive jaundice.
Common causes of jaundice include the
Mirizzi syndrome due to extrinsic
compression of the common bile duct,
primary involvement of the common bile duct
by the inflammation (xanthogranulomatous
choledochitis) or associated gallbladder
malignancy [1, 3, 4, 5]. Xanthogranulomatous
inflammation presenting with obstructive
jaundice due to the involvement of the lower
bile duct or the head of pancreas is rarely
reported [5, 6]. As most periampullary lesions
are malignant, patients with this clinical
presentation are usually subjected to a
pancreaticoduodenal resection.
From January 1989 to December 2003, 4,800
cholecystectomies were performed at the
authors’ institution. A xanthogranulomatous
inflammation was seen in 453
cholecystectomy specimens (9%). Forty three
out of 453 (9%) patients had a total bilirubin
greater than 3 mg/dL. The predominant
causes for an elevated bilirubin level included
choledocholithiasis in 23 (53%) patients,
associated gallbladder cancer in 7 (16%) and
Mirizzi syndrome in 9 (21%). In 214/453
(47%) patients, the gallbladder wall was thick
(greater than 4 mm on ultrasonography).
Xanthogranulomatous inflammation of the
ampulla causing obstructive jaundice has not
previously been reported. In the experience
choledochitis and xanthogranulomatous
pancreatitis, pancreaticoduodenectomy was
performed [5, 6]. In our patient, it is the
involvement of the ampulla with this
inflammation which resulted in this peculiar
clinical presentation. Repeated biopsies taken
from the ampulla usually will fail to reveal
malignancy. It is probable that the use of
intraductal sonography, particularly in
combination with per-oral cholangioscopy,
could give a better anatomy of the lesion and
under their guidance, fine needle aspiration
cytology could accurately diagnose the
condition pre-operatively [7]. We do not have
any experience of this modality at our centre.
However, given a tissue diagnosis of
ampullary xanthogranulomatous inflam-
mation, the absence of malignancy could still
prompt the same surgical treatment because
of the known association between this
pathology and malignancy. Although the
disease is rare, it should be added to the
differential diagnosis of patients presenting
with a suspected periampullary lesion and a
thick-walled gallbladder.
Received January 18
, 2006 - Accepted
January 26
, 2006
Keywords Ampulla of Vater; Common Bile
Duct; Pancreaticoduodenectomy
Abbreviations PD: pancreaticoduodenectomy
Rajan Saxena
Department of Surgical Gastroenterology
Raebareli Road

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JOP. J Pancreas (Online) 2006; 7(2):222-225.
JOP. Journal of the Pancreas - - Vol. 7, No. 2 - March 2006. [ISSN 1590-8577]
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