BMJ Case Reports 2012; doi:10.1136/bcr.01.2012.5497
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Cystadenomas are uncommon benign cystic neoplasms of
the biliary system, of unknown aetiology and account for
approximately 5% of all the hepatobiliary cystic masses. 1
Most cystadenomas arise within the intrahepatic bile
ducts. 2 3 Very rarely these lesions are found in the extrahe-
patic biliary system and gallbladder. 4
Women are more commonly affected with a mean age
at presentation of 45 years. 4 Preoperative diagnosis is chal-
lenging, since the radiologic features are non-specifi c.
We present a rare case of biliary cystadenoma with both
intra and extrahepatic bile duct involvement. The literature
on the radiologic and pathologic features of biliary cysta-
denomas, as well as approach to management, will be
A 62-year-old woman with a remote history of breast
cancer and hypothyroidism presented to her primary care
physician with 1 week history of dysuria and hyperpig-
mentation of her urine. Her physical examination was
within the normal limits except for sclero-icterus. Thyroid
replacement hormone therapy was the only medication
she was on and her family and social history were non-
Her physical examination was unremarkable. Liver func-
tion tests were abnormal, with an alkaline phosphatase of
215 U/l (normal: 32–92 U/l), aspartate aminotransferase of
266 U/l (normal: 15–41 U/l), alanine aminotransferase of
433 U/l (normal: 14–54 U/l), γ-glutamyl transpeptidase of
524 U/l (normal: 7–50 U/l) and a total bilirubin of 19 μmol/l
(normal: 0–16 μmol/l). Carbohydrate antigen (CA19.9)
19–9 levels were within normal limits.
Contrast enhanced CT scan and MRI with pancreatog-
raphy (MRCP) were performed and demonstrated diltation
of the left intrahepatic, common hepatic and common bile
ducts. The presence of enhancing septations within these
ducts was demonstrated on MRI ( fi gure 1A–C ). In addi-
tion, the left hepatic lobe was atrophic and its parenchyma
was hyperattenuating on CT ( fi gure 2 ) with increased T2
signal on MRI.
An endoscopic retrograde cholangiopancreatography
(ERCP) did not demonstrate a communication between
the biliary system and the cystic tumour. Cytopathology
specimens obtained from brushings during ERCP were
negative for malignancy.
Differential diagnosis for an intraductal, multi-cystic mass
and intraductal papillary mucinous tumour (IPMT).
On laparotomy, atrophy of the left hepatic lobe and dila-
tation of the common hepatic duct were appreciated. An
extended left hepatectomy, with common bile duct exci-
sion and Roux-en-Y right intrahepatic biliary-enteric ansto-
mosis, was performed. 5
The gross examination of the surgical specimen demon-
strated a multi-cystic, mucin-containing mass arising in the
left hepatic duct that prolapsed into the common bile duct
( fi gure 3A,B ).
OUTCOME AND FOLLOW-UP
Microscopically, the cysts were lined with a single layer
of mucinous columnar to cuboidal epithelium ( fi gure 4 ).
The stroma proliferating beneath the epithelium was
composed of spindle cells, resembling ovarian stroma.
Immunohistochemical stains were positive for oestrogen
Intra and extra-hepatic cystadenoma of the biliary duct.
Review of literature and radiological and pathological
characteristics of a very rare case
Diana Soochan, 1 Valerie Keough, 1 Ian Wanless, 2 Michele Molinari 3
1 Radiology Department, Dalhousie University, Halifax, Canada ;
2 Surgical Pathology Department, Dalhousie University, Halifax, Canada ;
3 Surgery Department, Dalhousie University, Halifax, Canada
Correspondence to Dr Michele Molinari, firstname.lastname@example.org
Painless obstructive jaundice is often associated with a malignant disease of the common bile duct or head of the pancreas. The authors
present a unique case of a 62-year-old woman affected by an intrahepatic cystadenoma that extended into the common biliary duct. To our
knowledge no previous case reports have been published on similar cases. After undergoing an en-block hepatic and bile duct resection, this
patient is doing well without signs of recurrent disease.