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

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.

Expression of RNA-binding protein LIN28 in classic gastric hepatoid carcinomas, gastric fetal type gastrointestinal adenocarcinomas, and hepatocellular carcinomas: An immunohistochemical study with comparison to SALL4, alpha-fetoprotein, glypican-3, and Hep Par1.

INTRODUCTION: Gastric hepatoid carcinomas (GHCs) include type I (classic) and type II (fetal type gastrointestinal adenocarcinoma). The classic type shows overlapping morphologic features with those of hepatocellular carcinoma (HCC). The aim of this study is to investigate expression of LIN28 in GHCs and explore its utility to distinguish classic GHC from HCC. METHODS: We investigated immunohistochemical expression of LIN28 in 93 primary GHCs (47 type I, 46 type II) and 60 HCCs with comparison to SALL4, AFP, glypican-3, Hep Par1, p-CEA and CK7. We also stained LIN28 and SALL4 in 52 conventional gastric adenocarcinomas to assess their specificity in gastric carcinomas. RESULTS: Classic GHCs and fetal type gastrointestinal adenocarcinomas showed positive LIN28 in 21/47 (45%) and 10/46 (22%), SALL4 in 41/47 (87%) and 36/46 (78%), AFP in 30/46 (65%) and 33/46 (72%), glypican-3 in 31/41 (76%) and 24/38 (63%), Hep Par1 in 27/41 (66%) and 28/37 (76%), and CK7 in 15/40 (38%) and 25/38 (66%), respectively. p-CEA staining was seen in 19/44 (43%) classic GHCs. Among HCCs, LIN28, SALL4, AFP, glypican-3, Hep Par1, p-CEA and CK7 was seen in 1/60 (2%), 0/60 (0%), 6/30 (20%), 23/30 (77%), 29/30 (97%), 28/30 (93%) and 21/30 (70%) cases, respectively. LIN28 and SALL4 staining was seen in 2/52 (4%) and 14/52 (27%) gastric conventional adenocarcinomas, respectively. The sensitivity and specificity of distinguishing classic GHCs from HCCs was 45% and 98% for LIN28, 87% and 100% for SALL4, 65% and 80% for AFP, 76% and 30% for glypican-3, 66% and 3% for Hep Par1, 43% and 7% for p-CEA, and 38% and 30% for CK7, respectively. Combining LIN28 and SALL4 increased the sensitivity to 96% with 98% specificity to distinguish classic GHCs from HCCs. CONCLUSIONS: LIN28 is a very specific marker (98% specificity) for distinguishing classic GHCs from HCCs though it is not as sensitive as SALL4. AFP, glypican-3, Hep Par1 and p-CEA are not useful in distinguishing classic GHCs from HCCs. Combining LIN28 and SALL4 increased the sensitivity to distinguish classic PHCs from HCCs.

Renal Cell Carcinoma, Unclassified with Medullary Phenotype and Synchronous Renal Clear Cell Carcinoma Present in a Patient with No Sickle Cell Trait/Disease: Diagnostic and Therapeutic Challenges.

Renal medullary carcinoma (RMC) is an aggressive high-grade renal cell carcinoma (RCC) associated almost exclusively with sickle cell trait or sickle cell disease. However, RCC with RMC features has rarely been reported in patients with no sickle cell trait or disease. Renal cell carcinoma unclassified with medullary phenotype (RCCU-MP) is a newly-coined term used by an international panel of experts to describe renal cell carcinoma showing morphologic and immunohistochemical features of renal medullary carcinoma in patients without sickle cell trait/disease. So far, only one study in the English literature has described five such cases. Here, we report a case with unique clinical and pathological features in a 76-year-old male patient without sickle cell trait. The patient had a history of colon cancer with liver and lung metastases and was found to have a new renal mass in his right kidney during the follow up. A right nephrectomy was performed and showed two separate masses (tumor 1 and tumor 2). Tumor 1 had histologic features of RMC and the tumor cells were positive for CK7, Pax8, and OCT4 and showed loss of nuclear INI1 expression. Tumor 1 was diagnosed as RCCU-MP (6.3 cm, pT3aNx, WHO/ISUP nuclear grade 3). Tumor 2 showed features of clear cell type of RCC (0.6 cm, pT1aNx, WHO/ISUP grade 2) with intact nuclear INI1 expression. Three-months post-nephrectomy, the patient developed lung metastasis of RCCU-MP. To the best of our knowledge, this was the first documented case with synchronous RCCU-MP and clear cell RCC presenting in a patient without sickle cell trait. Careful histologic assessment with a panel of immunohistochemical biomarkers was helpful to render a correct diagnosis for early aggressive treatment.

Synchronous Pancreatic Ductal Adenocarcinoma and Hepatocellular Carcinoma: Report of a Case and Review of the Literature.

Two or more histologically distinct malignancies diagnosed during the same hospital admission are uncommon, but they do exist. Cases with synchronous primary pancreatic ductal adenocarcinoma and hepatocellular carcinoma are rarely seen. This is a case report of a 56 years old Caucasian female with the chief complaint of jaundice over a duration of 10 days. CT imaging findings revealed a 3.5 cm ill-defined pancreatic head mass and a 1.5 cm liver mass in the segment 5. EUS-FNA cytology showed pancreatic head ductal adenocarcinoma (PDAC). Liver biopsy revealed a well differentiated hepatocellular carcinoma (HCC). The patient underwent a pancreaticoduodenectomy and the pathology revealed a pancreatic ductal adenocarcinoma extending into peripancreatic soft tissue, portal vein and vascular groove with perineural invasion. This is a unique and challenging case with the coexistence of a primary PDAC and a primary HCC. To the best of our knowledge, this is the first documented case of synchronous PDAC and HCC in the English literature. The diagnosis and treatment of the two entities are discussed.