Hepatoid carcinoma of the pancreas: a case report.

Background Hepatoid carcinoma (HC) is an extremely rare neoplasm that is morphologically similar to hepatocellular carcinoma. HC has been described in various organs; however, HC of the pancreas is extremely rare. To our knowledge, only 38 cases have been reported. We present a case of HC of the pancreas in a 36-year-old male patient. Case summary A 36-year-old cachexic man with no significant past medical history was transferred to our hospital with a history of painless jaundice, elevated blood glucose and significant weight loss. Lab tests showed elevated serum transaminases, bilirubin and alpha-fetoprotein levels. Magnetic resonance imaging of the upper abdomen showed a diffusely enlarged pancreas, appearing "sausage-shaped". Magnetic resonance cholangiopancreatography showed upstream ductal dilation secondary to stricture of the main pancreatic duct and the common bile duct, which were not visible. Immunohistochemistry of biopsied tissue from a percutaneous pancreatic biopsy showed tumor cell positivity for HepPar1, polyclonal carcinoembryonic antigen and CK19, suggestive of HC of the pancreas. The characteristics of 39 patients with HC of the pancreas were reviewed. Conclusion HC of the pancreas is more prevalent in males, and patients have a median age of 57 years. It is most commonly asymptomatic or presents as abdominal back pain, and the pancreatic tail is the most common location. At the time of diagnosis, liver metastasis is often present.

Hepatoid carcinoma of the pancreas: A case report and review of the literature.

Hepatoid adenocarcinoma (HAC) is rare and was first reported as α-fetoprotein (AFP)-producing tumor. It is an important variant of extrahepatic adenocarcinoma with clinicopathological presentation mimicking hepatocellular carcinoma and carries exceedingly poor prognosis. HAC most commonly originates in the stomach, and less commonly in the ovary, esophagus, lung, among other organs. HAC originating in the colon is exceedingly rare. Here we report such a case of a 63-year-old man presented as decompensated liver failure with jaundice and weakness. Computed tomography (CT) imaging findings showed multiple lesions in the liver with ascites and descending colonic mass suspicious for malignancy. The flexible sigmoidoscopy showed a 1.5 cm mass in the descending colon, and biopsy showed superficial fragments of tubular adenoma, but could not exclude deep invasive carcinoma. A liver biopsy was performed and showed a carcinoma with morphologic features resembling hepatocellular carcinoma. The tumor cells were positive for glypican-3, MOC31, CDX2, SATB2 and CK20, negative for arginase-1, p63, synaptophysin and chromogranin. Ki-67 highlighted 80% of the tumor cells. The pathology diagnosis was liver with metastatic hepatoid adenocarcinoma consistent with colonic primary. The patient experienced a rapid worsening of his liver function and died 3 weeks later of hepatic failure without any surgery and chemotherapy. A subsequent literature review of the 17 reported cases of HAC showed that this type of cancer frequently metastasizes to the liver with an astonishingly poor prognosis with eight patients died of the disease in less than 5 months after the diagnosis was made. Radical surgery followed by adjuvant chemotherapy with chemotherapy regimen used for colorectal cancer or primary hepatocellular carcinoma may be the treatment option for colorectal HAC.

/pdf/liver-metastasis-of-hepatoid-colonic-adenocarcinoma-a-rare-2u7o949f4g.pdf

Liver Metastasis of Hepatoid Colonic Adenocarcinoma: A Rare and Unusual Entity With Poor Prognosis and Review of the Literature.

Background Hepatoid adenocarcinoma (HAC) is a kind of rare extrahepatic adenocarcinoma with hepatocyte differentiation. At present, the incidence and prognostic factors of HAC remain poorly discussed. Methods We extracted two cohorts from Surveillance, Epidemiology, and End Results (SEER) database by restricting the histology to HAC. One cohort of 258 eligible patients was from SEER 21 Regs Limited-Field Research Data + Hurricane Katrina Impacted Louisiana Cases [2000–2016] to compute incidence, and the other cohort of 121 eligible patients was from SEER 18 Regs Custom Data (with additional treatment fields) [1975–2016] to estimate survival and risk factors. The incidence wad computed by the Joinpoint Regression Program (Version 4.7.0.0). The overall survival (OS) and prognostic factors were analyzed by univariate and multivariate analyses in SPSS 24.0. Results The overall incidence of HAC between 2000 and 2016 was 0.014 per 100,000 people (95% CI: 0.012 to 0.015). The most common primary site was the lung, followed by the digestive, urinary and reproductive organs, but in the previously published case reports, the stomach was the most common site. The median age at diagnosis was 66 (range, 31–85) years. A total of 28.1% of patients underwent surgery, while 71.9% underwent radiotherapy or/and chemotherapy. The 1-year survival rate of these patients was 35.0%, and the 3-year survival rate was only 16.9%. The median survival time was 5 months (95% CI: 2.97 to 7.03 months). Multivariate analysis showed that age less than 60 years, no distant metastasis, surgery and chemotherapy were independent prognostic factors related to a better outcome. Conclusions This study suggests that HAC is a rare malignant tumor with poor prognosis, and its most common site is lung. Its incidence is increasing year by year. Age at diagnosis, distant metastases, surgery, and chemotherapy were independent prognostic factors.

/pdf/incidence-and-prognostic-factors-of-hepatoid-adenocarcinoma-5d7kexyz4v.pdf

Incidence and prognostic factors of hepatoid adenocarcinoma: a population-based analysis

Mimics of hepatocellular carcinoma: a review and an approach to avoiding histopathological diagnostic missteps.

Primary pancreatic hepatoid carcinoma (PHC) is very rare. Here, we reported 3 such cases with review of additional 31 cases in the literature. Our 3 patients were male (83, 72, and 54 years old, respectively). Serum α-fetoprotein (AFP) was elevated in 1 patient (case 3, 8338 ng/mL) and not measured in the other two. The PHC in patient 1 (pathological stage pT2N0M0) and patient 2 (pT3N0M0) showed pure hepatocellular carcinoma (HCC)-like morphology, whereas in case 3 it was a PHC with true glandular differentiation (pT4N0M0). The diagnosis of PHC was confirmed with positive immunohistochemical staining in the tumor cells for AFP (2/3), Hep Par 1 (3/3), glypican-3 (2/3), arginase-1 (2/3), and Sal-like protein 4 (1/3). CD10 and polyclonal carcinoembryonic antigen stains show focal canalicular pattern in 2/3 tumors. Patient 1 did not receive further treatment after resection and was alive with no evidence of disease at 107 months. Patient 2 died of postoperative complications, whereas patient 3 received postsurgical chemoradiation and died of disease at 29 months. Our findings and literature review indicate that PHCs can be divided into 4 histological subtypes: with pure HCC-like morphology (n = 22), with neuroendocrine differentiation (n = 8), with true glandular differentiation (n = 3), and with acinar cell differentiation (n = 1). On univariate analysis, pure HCC-like morphology was associated with better disease-specific survival (DSS; P = .04), whereas lymph node and distant metastases were associated with worse DSS ( P = .002 for both). Age, gender, presenting symptoms, serum AFP level, and T stage were not associated with DSS. On multivariate analysis, none of these parameters was significantly associated with DSS.

https://journals.sagepub.com/doi/pdf/10.1177/1066896918783468

Primary Hepatoid Carcinoma of the Pancreas: A Clinicopathological Study of 3 Cases With Review of Additional 31 Cases in the Literature.

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Primary Hepatoid Carcinoma of the Pancreas: A Clinicopathological Study of 3 Cases With Review of Additional 31 Cases in the Literature.