2016, Volume 32, Number 1, Page(s) 060-062
A Problematic Case of Unclassified Multicystic Biliary Tumor with Adenofibroma Features
Gülsüm Özlem ELPEK, Betül ÜNAL, Cumhur İbrahim BAŞSORGUN, Erdem AYIK
Department of Pathology, Akdeniz University, School of Medicine, ANTALYA, TURKEY
To the Editor,
Biliary adenofibroma (BFA) was first described by Tsui
et al.1. Since then, few cases of BFA of the liver have
been reported in the literature1-8. It is characterized by
tubulocystic proliferation of variable-sized neoplastic ducts
embedded in abundant fibrous stroma. The typical case of
biliary adenofibroma shows a non-mucin secreting and
apocrine-like epithelium that expresses biliary cytokeratins
(CK7, CK19), suggesting a bile duct origin1,6. In this
report, we describe a case of biliary multicystic tumor
with adenofibroma features, which did not fit any current
classification of biliary tumor.
A 23-year-old man who had a past history of left orchiectomy
and chemotherapy for a testicular germ cell tumor was
admitted to our institution for routine follow-up controls.
Physical examination on admission was unremarkable.
However, a well-circumscribed multicystic mass lesion in
the liver containing solid areas was detected on abdominal
computerized tomography (CT). Although tumor markers
as well as hematologic and coagulation tests were within the
normal ranges, the patient underwent partial hepatectomy
with preoperative diagnosis of germ cell tumor metastasis
to the liver.
In the resection specimen, a mass measuring approximately
6x3 cm was detected in the liver parenchyma. In the cut
section, an unencapsulated but well-circumscribed multicystic
tumor containing solid areas was observed. The cysts
contained serous and hemorrhagic fluid. Solid areas were
white in color and composed of microcysts leading to a
Histopathological findings are presented in Figure 1A-G.
The cyst locules were not communicating and were lined
by columnar, cuboidal and flattened epithelium. Mucin
production was not present by mucicarmine histochemical
staining. A moderate to high epithelial atypia was observed
in some areas. However, atypical mitosis was not present.
In some areas, papillary growths and apocrine snouts
were also evident. The stroma beneath the epithelium was
fibrous and no ovarian-like stroma was detected. The solid
areas consisted of smaller tubules and glands embedded in
a denser collagenous background.
In the immunohistochemical examination, tumor cells
displayed positivity with CK7, CK19, CK 18 and EMA. AFP, PLAP, HCG, Hepatocyte, CK20, CD30, OCT4 and
MUC2 were negative (Figure 1A-G).
Click Here to Zoom
|Figure 1: A) The cut section of the tumor. A wellcircumscribed
multicystic tumor was observed, B) The
histopathology of the multicystic area. (H&E; x100), C)
The locules were lined by columnar and cuboidal tumor
cells showing various degrees of atypia (H&E; x200), D)
The tumor shows partial papillary growth and apocrine
snouts (H&E; x200), E) The stroma was fibrous but an
ovarian-like stroma was not observed (H&E; x100), F)
CK7 positivity in the tumor cells (CK7; x100), G) CK19
positivity in the tumor cells (CK19; x200).
Considering the past history of the patient, the possibility
of testicular germ cell tumor metastasis was first to
be excluded. This was not troublesome, because the
histopathological features and immunohistochemistry did
not support a metastatic germ cell tumor.
The gross multicystic appearance of the presented tumor
was reminiscent of an intrahepatic multicystic biliary
hamartoma. The tumor was composed of neoplastic
cysts and fibrous stroma only whereas multicystic biliary
hamartoma is composed of ducts, periductal glands and
The Von-Meyenberg complex with its smaller size and
periportal localization was easily excluded. The findings
of the present tumor were also completely different from a
ciliated foregut cyst8.
The presence of multiple cysts and papillary projections
together with solid areas leaded us to consider mucinous
cystic neoplasia (MCN) and intraductal papillary
neoplasms (IPN) of the biliary tree in the differential
diagnosis. However, the absence of ovarian like-stroma and
the non-communicant nature of the cysts did not fit the
diagnostic criteria of MCN and IPN, respectively9-11.
Furthermore, tumors cells were negative for both MUC-2
and mucicarmine. The tumor did not show the morphology
of the solid component of malignant MCN composed
of tubulopapillary carcinoma or cholangiocarcinoma.
Therefore, other current classifications of biliary cystic
tumors such as MCNs or IPNs seemed inappropriate for
In the tumor presented here, solid areas showed
characteristics of BFA such as dense fibrous stroma
and apocrine snouts1,6. On the other hand, marked
cellular atypia was not consistent with BFA, because it is
considered a benign tumor. However, the first report of
biliary adenofibroma documented that moderate atypia
was evident in some cysts similar to the present tumor1.
Additionally, two out of seven reported recent cases showed
malignant transformation2,3 and one of them showed
local recurrence and distant metastases3,8. That is why
our diagnosis was in the direction of biliary fibroadenoma
despite the presence of cellular atypia. The expression of CK7, CK19 and CK 18 also supported the bile duct origin
of this tumor. However, the present tumor displays two
features that are not consistent with BFA. Macroscopic
appearance of the tumor is not microcystic and solid like
BFA and the papillary component is less pronounced than
BFA as reported by Kai et al.8. Therefore, the tumor was
diagnosed as unclassified multicystic biliary tumor with
Although this tumor is extremely rare, we expect the
appropriate classification will be developed by the
accumulation of similar cases and studies that reveal the
nature of this kind of neoplasm.
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