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      Hepatoid Adenocarcinoma Presenting as Pancreatitis

      case-report

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          ABSTRACT

          Hepatoid adenocarcinoma (HAC) is an uncommon and aggressive type of adenocarcinoma, typically affecting the middle-aged and elderly. The morphological features of the HAC resemble hepatocellular carcinoma. Presenting symptoms may include upper abdominal pain, hematemesis, back pain, and palpable abdominal mass. HAC has no proven therapy, and the prognosis is extremely poor. Early surgical removal with chemotherapy remains the standard of care. We describe one of the youngest patients in the literature with HAC who presented with acute pancreatitis. The diagnostic workup was confused by diffuse lymphadenopathy and elevated β-human chorionic gonadotropin making lymphoma and germ cell tumor likely possibilities until immunohistochemistry confirmed the diagnosis.

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          Most cited references20

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          Clinicopathological characteristics in the differential diagnosis of hepatoid adenocarcinoma: a literature review.

          Hepatoid adenocarcinoma (HAC) is a rare but important special type of extrahepatic adenocarcinoma with clinicopathological presentation mimicking hepatocellular carcinoma (HCC), and prompt and correct diagnosis can be a challenge, especially in endemic areas with a high incidence of HCC. To date, HAC has only been reported in case series or single case reports, so we aimed to review the clinicopathological characteristics of HAC to obtain a more complete picture of this rare form of extrahepatic adenocarcinoma. All the articles about HAC published from 2001 to 2011 were reviewed, and clinicopathological findings were extracted for analysis. A late middle-aged male with high serum α-fetoprotein and atypical image finding of HCC should raise the suspicion of HAC, and characteristic pathological immunohistochemical stains can help with the differential diagnosis. Novel immunohistochemical markers may be useful to clearly differentiate HAC from HCC. Once metastatic HAC is diagnosed, the primary tumor origin should be identified for adequate treatment. The majority of HAC originates from the stomach, so panendoscopy should be arranged first.
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            Hepatoid adenocarcinoma of the stomach. A clinicopathologic and immunohistochemical analysis.

            The clinicopathologic and immunohistochemical features of the primary gastric hepatoid adenocarcinomas still remain unclear. The authors evaluated 28 hepatoid adenocarcinomas (HC) of the stomach on the basis of characteristic histologic features resembling hepatocellular carcinoma, which were selected from the 7200 cases of primary gastric carcinoma in their files. Additionally, 22 alpha-fetoprotein (AFP)-positive adenocarcinomas without hepatoid features (APC) were also selected. The HC cases fell into the following two groups: HC with AFP-positive tumor cells (Group 1; 15 cases) and HC without AFP-positive cells (Group 2; 13 cases). Histologically, the glycogen granules and hyaline globules were common features in HC. The incidence of a venous invasion of HC (Groups 1 and 2) was higher than that of APC (Group 3). There were no significant differences among the three groups regarding clinical features, macroscopic features, and the incidence of lymphatic permeation. An immunohistochemical study showed that HC had differentiation into various directions. As for the advanced carcinomas, the 5-year survival rates of patients with HC (Groups 1 and 2) and those with APC (Group 3) were 11.9% (21.4%, 8.3%) and 38.2%, respectively. The prognosis of Group 1 was similar to that of Group 2, but was poorer than that of Group 3. The findings suggested that HC, as shown by its characteristic histologic features, had a poor prognosis whether producing AFP or not, and that HC should therefore be distinguished from AFP-positive carcinoma without hepatoid features.
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              Hepatoid adenocarcinoma of the stomach: a unique subgroup with distinct clinicopathological and molecular features

              Objectives Hepatoid adenocarcinoma of the stomach (HAS) is characterized by histological resemblance to hepatocellular carcinoma and a poor prognosis. The aim of this study is to elucidate the clinicopathological and molecular characteristics of HAS. Methods Forty-two patients with HAS who received gastrectomy were enrolled in this study. Based on a panel of 483 cancer-related genes, targeted sequencing of 24 HAS and 22 clinical parameter-matched common gastric cancer (CGC) samples was performed. Prognostic factors for overall survival (OS) and disease-free survival (DFS) were analysed with the Kaplan–Meier method. Results The most frequently mutated gene in both HAS and CGC was TP53, with a mutation rate of 30%. Additionally, CEBPA, RPTOR, WISP3, MARK1, and CD3EAP were identified as genes with high-frequency mutations in HAS (10–20%). Copy number gains (CNGs) at 20q11.21-13.12 occurred frequently in HAS, nearly 50% of HAS tumours harboured at least one gene with a CNG at 20q11.21-13.12. This CNG tended to be related to more adverse biobehaviour, including poorer differentiation, greater vascular and nerve invasion, and greater liver metastasis. Pathway enrichment analysis revealed that the HIF-1 signalling pathway and signalling pathways regulating stem cell pluripotency were specifically enriched in HAS. The survival analysis showed that a preoperative serum AFP level ≥ 500 ng/ml was significantly associated with poorer OS (p = 0.007) and tended to be associated with poorer DFS (p = 0.05). Conclusion CNGs at 20q11.21-13.12 happened frequently in HAS and tended to be related to more adverse biobehaviour. The preoperative serum AFP level was a sensitive prognostic biomarker for DFS and OS. Electronic supplementary material The online version of this article (10.1007/s10120-019-00965-5) contains supplementary material, which is available to authorized users.
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                Author and article information

                Journal
                ACG Case Rep J
                ACG Case Rep J
                ACGCRJ
                ACGCRJ
                AC9
                ACG Case Reports Journal
                Wolters Kluwer (Maryland, MD )
                2326-3253
                May 2020
                07 May 2020
                : 7
                : 5
                : e00381
                Affiliations
                [1 ]Department of Medicine, Pennsylvania Hospital of the University of Pennsylvania Health System, Philadelphia, PA
                [2 ]Department of Pathology, Pennsylvania Hospital of the University of Pennsylvania Health System, Philadelphia, PA
                Author notes
                Correspondence: Rahul Chaudhari, MD ( Rahul.chaudhari@ 123456pennmedicine.upenn.edu ).
                Article
                ACGCR-19-0804 00009
                10.14309/crj.0000000000000381
                7289282
                388e3575-af03-4c05-a681-28231e8e9685
                © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.

                This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 20 November 2019
                : 04 March 2020
                Categories
                Case Report
                Stomach
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