Torbenson, Michael S.

ABSTRACT Hepatoid adenocarcinoma (HAC) is a rare extrahepatic tumor of non‐germ cell origin that morphologically resembles hepatocellular carcinoma (HCC). HAC has a propensity to metastasize to the liver and therefore may be mistaken for HCC. There is a lack of standardized treatment protocols, and further studies are needed to evaluate the benefit of targeted therapy and immunotherapy. Recent studies have reported that tumor protein 53 ( TP53 ) gene mutations are associated with increased expression of programmed cell death ligand‐1 (PD‐L1), which may be a predictor of response to PD‐L1 targeted checkpoint inhibitors. This review provides a clinical guidance for the management of patients with HAC by summarizing the salient clinical features, risk factors, diagnostic criteria, differential diagnosis, new therapeutic approaches, and prognosis of this rare tumor. Furthermore, we reviewed the Mayo Clinic experience to describe the clinical characteristics of 15 patients diagnosed with HAC. HAC is usually diagnosed at an advanced stage with distant metastases. In patients diagnosed with liver lesions that have similar radiologic and histologic features to HCC, particularly in the absence of underlying chronic liver disease, further evaluation should be performed to rule out HAC. Communication between medical subspecialties is important to avoid misdiagnosis and prevent further disease progression. In our patient cohort TP53 was the most frequently mutated gene (5 out of 8, 62.5%) and PD‐L1 expression showed a positive score in 3 out of 6 patients (50%). However, only a few patients received immunotherapy (6 out of 14, 42.9%) suggesting that the numbers are too small to draw a conclusion about its efficacy in treating HAC.

In this paper, we present our preliminary findings regarding magnetic resonance elastography (MRE) on the livers of 10 patients with systemic amyloidosis. Mean liver stiffness measurements (LSM) and spleen stiffness measurements (SSM) were obtained. Magnetic resonance imaging (MRI) images were analyzed for the distribution pattern of amyloid deposition. Pearson correlation analysis was performed in order to study the correlation between LSM, SSM, liver span, liver volume, spleen span, spleen volume, serum alkaline phosphatase (ALP), N-terminal pro b-type natriuretic peptide (NT pro BNP), and the kappa and lambda free light chains. An increase in mean LSM was seen in all patients. Pearson correlation analysis showed a statistically significant correlation between LSM and liver volume (r = 0.78, p = 0.007) and kappa chain level (r = 0.65, p = 0.04). Interestingly, LSM did not correlate significantly with SSM (r = 0.45, p = 0.18), liver span (r = 0.57, p = 0.08), or serum ALP (r = 0.60, p = 0.07). However, LSM correlated significantly with serum ALP when corrected for liver volume (partial correlation, r = 0.71, p = 0.03) and NT pro BNP levels (partial correlation, r = 0.68, p = 0.04). MRI review revealed that amyloid deposition in the liver can be diffuse, lobar, or focal. MRE is useful for the evaluation of hepatic amyloidosis and shows increased stiffness in hepatic amyloidosis. MRE has the potential to be a non-invasive quantitative imaging marker for hepatic amyloidosis.