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Diagnosis and Management of Intrahepatic Cholangiocarcinoma: A Comprehensive Update For The Radiologist
Diagnosis and Management of Intrahepatic Cholangiocarcinoma: A Comprehensive Update For The Radiologist
INTRAHEPATIC CHOLANGIOCARCINOMA:
A COMPREHENSIVE UPDATE
FOR THE RADIOLOGIST
A.D. Baheti, S.H. Tirumani, M.H. Rosenthal, A.B. Shinagare, N.H.
Ramaiya
Clinical Radiology 69 (2014) e463-e470
INTRODUCTION
Cholangiocarcinoma is the most common neoplasm of the biliary
tree.
Classified based on its location as intrahepatic or extrahepatic.
Extrahepatic cholangiocarcinoma is subdivided as perihilar
(Klatskins tumour) and distal extrahepatic cholangiocarcinoma.
IHCC is the least
cholangiocarcinoma.
common
of
the
three,
+/-
8-10%
of
AETIOPATHOGENESIS
Most common age group affected by IHCC is between 5575 years, with a slight male preponderance in both
incidence and mortality.
Risk factors include various disorders, which cause chronic
biliary inflammation :
Primary sclerosing cholangitis,
Parasitic infestation (endemic in Southeast Asia),
Hepatolithiasis,
hepatitis B and C, and cirrhosis,
Congenital abnormalities of the biliary tract :
choledochal cyst and fibrocystic liver disease.
AETIOPATHOGENESIS
Liver Cancer Group classified cholangiocarcinoma into three
types based on their morphological appearance and pattern of
spread :
Mass-forming (79-86%)
Periductal-infiltrating
Intraductal growing
Pathologically, IHCC has a tendency to develop abundant
desmoplastic response, particularly at its centre.
On histopathology, IHCC is an adenocarcinoma of bile ducts
with central fibrous stroma and foci of coagulative necrosis.
Mucinous degeneration may be present in the centre, and
rarely show calcification.
Immunohistochemistry considered suggestive : cytokeratin 7
(CK7), CK19, and anion exchanger (AE)1/3 and absence of
lack
of
lymph
node
metastases
Right
regional
lymph
nodes
:
hilar,
periduodenal,
and
peripancreatic nodes; whereas
left regional lymph nodes
include hilar and gastrohepatic
nodes; Coeliac, periaortic, and
pericaval
adenopathy
is
considered as M1 stage
IMAGING FEATURES
IMAGING FEATURES
IMAGING FEATURES
IMAGING FEATURES
IMAGING FEATURES
IMAGING FEATURES
IMAGING FEATURES
IMAGING FEATURES
Prognosis
Enhancement pattern
Asayama Y et al. : Intra tumoral enhancing
component > 2/3 on delayed-phase CT worsen
prognosis. Delayed enhancement correlated with
fibrous stroma and perineural invasion on
histopathology.
Kim SA et al. : Arterial enhancing tumor correlate
significantly tumor vascularity, chronic liver
disease, and improved survival. Histopathology :
less fibrotic and necrosis component and more
cellular area.
Overall accuracy of CT in evaluating tumour
IMAGING FEATURES
Prognosis
IMAGING FEATURES
Prognosis
IMAGING FEATURES
Potentially
curative
procedure
surgery
:
wedge/segmental resections, partial hepatectomy, or
extended hepatectomy
Portal lymphadenectomy for accurate N staging,
although its therapeutic benefit is uncertain
Optimal management strategy : resection margin, nodal
status, lymphovascular and perineural invasion
R0 resection followed-up with observation or treated with
chemotherapy or enrolled in a clinical trial. Imaging
surveillance at 6 months intervals for 2 years if clinically
indicated.
Patients with R1/ R2 resection are treated with standard
chemotherapy or chemoradiation. Ablative therapy may
FUTURE DIRECTIONS
Molecular targeted therapy (MTT) promising trial
further validation
VEGF (vascular endothelial growth factor) inhibitors
(bevacizumab, sunitinib, sorafenib),
EGFR (epidermal growth factor receptor) inhibitors
(erlotinib,cetuximab)
/
in
combination
with
conventional chemotherapy
Important role of chromatin remodelling in the
carcinogenesis in IHCC precision radiology and
genomic tumour evaluation extensive ongoing
research surrounding cholangiocarcinoma further
developments
in
its
diagnosis,
imaging,
and
management.
CONCLUSION
imaging plays an important role in the diagnosis,
prognostication, and follow-up of patients with IHCC
Radiologist must identify :
Newly detected hepatic mass : various imaging
features that indicate a diagnosis of IHCC.
Pre operative : surgical resectability and various
prognostic imaging features with respect to the
pattern of contrast enhancement and the subtype (I, II,
III)
Post surgical : intra- and extrahepatic sites of recurrent
disease, restaging scans with patients on treatment
/treatment response, identify drug-associated toxicities
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