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2017 ; 6
(1
): 22-34
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Pathology of intrahepatic cholangiocarcinoma
#MMPMID28261592
Vijgen S
; Terris B
; Rubbia-Brandt L
Hepatobiliary Surg Nutr
2017[Feb]; 6
(1
): 22-34
PMID28261592
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Intrahepatic cholangiocarcinoma (iCC) is a primary carcinoma of the liver with
increasing significance and major pathogenic, clinical and therapeutic
challenges. Classically, it arises from malignant transformation of
cholangiocytes bordering small portal bile duct (BD) to second-order segmental
large BDs. It has three major macroscopic growth pattern [mass-forming (MF),
periductal infiltrative (PI), and intraductal growth (IG)] and histologically is
a desmoplastic stroma-rich adenocarcinoma with cholangiocyte differentiation.
Recent data pointed out noteworthy degree of heterogeneity in regards of their
epidemiology and risk factors, pathological and molecular features, pathogenesis,
clinical behaviors and treatment. Notably, several histological variants are
described and can coexist within the same tumor. Several different cells of
origin have also been depicted in a fraction of iCCs, amongst which malignant
transformation of ductules, of hepatic stem/progenitor cells, of periductal
glands or through oncogenic reprogramming of adult hepatocytes. A degree of
pathological overlap with hepatocellular carcinoma (HCC) may be observed in a
portion of iCC. A series of precursor lesions are today characterized and
emphasize the existence of a multistep carcinogenesis process. Overall, these new
data have brought up in proposal of new histological or molecular
classifications, which could soon replace current anatomic-based classification
and could have major impact on establishment of prognosis and on development of
novel target treatment approaches.