The Diagnostic Conundrum and Liver Transplantation Outcome for Combined Hepatocellular‐Cholangiocarcinoma

Abstract

Combined hepatocellular-cholangiocarcinoma (cHCC-CC) is a rare primary liver malignancy with mixed hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC) histological features. It is almost impossible to obtain an accurate, preoperative noninvasive diagnosis of cHCC-CC with tumor markers or crosssectional abdominal imaging due to the mixed histological features. Despite these difficulties, accurate cHCC-CC diagnosis remains an important goal with prognostic significance. In our study, we retrospectively reviewed the tumor markers: AFP and CA 19-9, and cross-sectional liver imaging, in light of liver explant findings, to identify and characterize cHCC-CC features followed by liver transplantation (LT) outcome analysis. The results from this 12 patient cohort failed to identify characteristic features for cHCC-CC. None of the imaging features helped to identify the cHCC-CC tumor and they mimicked either HCC or CC, depending on the degree of glandular differentiation expressed histologically. In our cHCC-CC LT recipients, the 1-, 3-and 5-year cumulative survival probabilities were 79%, 66% and 16%, respectively with a 5-year survival comparable to or better than LT for intrahepatic CC but poorer than LT for HCC following the Milan criteria. Conceivably explained by its cholangiocarcinoma component the LT outcome for this rare and hard to diagnose tumor appears poor.

Mixed hepatocellular cholangiocarcinoma and intrahepatic cholangiocarcinoma in patients undergoing transplantation for hepatocellular carcinoma

“…This incidence might have been higher if only patients who had undergone transplantation for HCC had been considered. Panjala et al 6 recently reported that among patients who underwent LT for HCC, 12 patients were found to have mixed HCC-CC and I-CC in the explant. The tumor recurrence rate was 58%, and the 5-year overall patient survival rate was a dismal 16%.…”

“…6,7 CC tumors are divided into hilar/perihilar and intrahepatic subtypes. Intrahepatic cholangiocarcinoma (I-CC) is rare in patients with cirrhosis with etiologies other than primary sclerosing cholangitis (PSC).…”

Mixed hepatocellular and cholangiocarcinoma: a rare tumor with a mix of parent phenotypic characteristics

“…Both HCC and ICC have characteristic imaging features. [16][17][18][19] The imaging features of cHCC-ICC are often only subtly different 9,20 from HCC and ICC, precluding reliable imaging diagnosis. Moreover, even percutaneous biopsy of cHCC-ICC may be misleading because only the dominant HCC or ICC component of cHCC-ICCC is confirmed pathologically, and the true histopathology of cHCC-ICC can only be confirmed after evaluation of the resected surgical specimen.…”

“…Currently, clinical consensus opposes the use of transplantation as a therapeutic option for patients with these tumors. [8][9][10] However, data regarding optimum management of cHCC-ICC remain murky.…”

Current status of the organ replacement approach for malignancies and an overture for organ bioengineering and regenerative medicine

“…[59][60][61][62][63] One study that analyzed patients in the Surveillance, Epidemiology, and End Results database found that LT (19 patients) and resection (35 patients) for localized combined HCC-CCA had similar 3-y survival rates in the 50% range, which was inferior to LT for HCC (1447 patients, 78%). 63 At the moment, liver resection is preferred over LT for resectable combined HCC-CCA.…”