Letný bioptický seminár SD-IAP Senec MUDr. Juraj Marcinek, PhD. Prípad SD-IAP 480 Ústav patologickej anatómie a Konzultačné centrum hematopatológie UNM a JLF UK

Letný bioptický seminár SD-IAP Prípad SD-IAP 480  68 yer old man – Ca capitis pancreatis (cT3 N1 MX)  makroskopic finding: vhite tumorous mas of 18mm rising aroud pancreatic duct in the head of pancreas head of pancreas under duodenal serosa visible tumorous mass corresponding to LN under duodenal serosa visible tumorous mass corresponding to LN

Letný bioptický seminár SD-IAP 21 – Prípad SD-IAP 480

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PASPAS PASPAS-K

CK20CK7 CK17CK19

CEApCEAm trypsinechymotrypsine

HepPar1 villinAFP CD10

TTF1 Β-catenin glypican 3 Schmorl ? hepatoid carcinoma of pancreas with focal ductal differentiation

Letný bioptický seminár SD-IAP 21 – Prípad SD-IAP 480  hepatoid carcinomas – heterogenous neoplasms with morphologic and phenotypic resemblance to hepatocellular carcinoma (HCC) resemblance to hepatocellular carcinoma (HCC) - most common arise in stomach (since Ishikura et al. 1985) - most common arise in stomach (since Ishikura et al. 1985) - less common in ovary, testis, esophagus, duodenum, pancreas, - less common in ovary, testis, esophagus, duodenum, pancreas, colon, rectum, lung, gallblader, urinary tract, uterus, vagina colon, rectum, lung, gallblader, urinary tract, uterus, vagina - morphology: trabecular / pseudoacinar / solid growth pattern - morphology: trabecular / pseudoacinar / solid growth pattern polygonal cells with abundant cytoplasm polygonal cells with abundant cytoplasm central vesicular nuclei with prominent nucleoli central vesicular nuclei with prominent nucleoli pleomorphic cells, sinusoidal vascularisation pleomorphic cells, sinusoidal vascularisation intracytoplasmic PAS+ hyalinne globules intracytoplasmic PAS+ hyalinne globules occasional bile production occasional bile production - usually at least partial tubular/tubulopapilar - usually at least partial tubular/tubulopapilar growth pattern growth pattern - immunohistochemistry: markers of hepatocellular differetiation: - immunohistochemistry: markers of hepatocellular differetiation: AFP, HepPar1, polyklonal CEA, CD10, glypican 3, albumin mRNA AFP, HepPar1, polyklonal CEA, CD10, glypican 3, albumin mRNA - usually not all markers are present - usually not all markers are present - coexpression of cytokeratins atypical for HCC - coexpression of cytokeratins atypical for HCC

Letný bioptický seminár SD-IAP 21 – Prípad SD-IAP 480  hepatoid carcinoma of pancreas – rare neoplasm - larges study contains 15 cases (Kai et al., Pathology - larges study contains 15 cases (Kai et al., Pathology International 2012) International 2012) - clinically unspecific symptoms - clinically unspecific symptoms - can have elevated serum AFP (7/15) - can have elevated serum AFP (7/15) - can differentiate form acinar/ductal/islet cells of - can differentiate form acinar/ductal/islet cells of pancreas pancreas - common associated with other cellular carcinoma - common associated with other cellular carcinoma component (endocrine features 5/15, ductal component (endocrine features 5/15, ductal carcinoma 3/15, acinar carcinoma 1/15) carcinoma 3/15, acinar carcinoma 1/15)  prognosis – hepatoid carcinomas have generally poor prognosis - most tumors are aggressive with metastasis at time of diagnosis - most tumors are aggressive with metastasis at time of diagnosis - mainly hematogenous metastases into liver, vascular permeation (veins) - mainly hematogenous metastases into liver, vascular permeation (veins) - pancreatic hepatoid carcinomas – variable prognosis depending on stage at - pancreatic hepatoid carcinomas – variable prognosis depending on stage at the time of diagnosis the time of diagnosis

Letný bioptický seminár SD-IAP 21 – Prípad SD-IAP 480  differential diagnosis of hepatoid carcinomas from: 1. hepatocellular carcinoma with extrahepatic spreading – problematic to impossible hepatoid carcinomas - morphologically similar, but often contain variabile amounth hepatoid carcinomas - morphologically similar, but often contain variabile amounth glandular or tubulopapilary adenocarcinomatous component glandular or tubulopapilary adenocarcinomatous component - immunohistochemical pattern of HCC usually incomplete with - immunohistochemical pattern of HCC usually incomplete with strong coexpression of cytokeratins atypical fo HCC (CK19, strong coexpression of cytokeratins atypical fo HCC (CK19, CK17, CK20) CK17, CK20) - CEAm+ in adenocarcinomatous component - CEAm+ in adenocarcinomatous component - CEAp+ in both components (luminal/canalicular in hepatoid c.) - CEAp+ in both components (luminal/canalicular in hepatoid c.) - most important are clinical informations of liver finding - most important are clinical informations of liver finding - HCC spread into pancreas is a rare (2,7-5,6%) and late finding - HCC spread into pancreas is a rare (2,7-5,6%) and late finding - multiple liver nodules without chronic liver disease associated - multiple liver nodules without chronic liver disease associated with other large large gastrointestinal tumor are prone to be with other large large gastrointestinal tumor are prone to be metastases metastases

Letný bioptický seminár SD-IAP 21 – Prípad SD-IAP 480  differential diagnosis of hepatoid carcinoma of pancreas 2. acinar cell carcinoma (solid variant) – morphologically similar cells, granular PAS+ cytoplasm, uniform, basal oriented nuclei – morphologically similar cells, granular PAS+ cytoplasm, uniform, basal oriented nuclei and „palisading“ of nuclei around vessels, distinct nucleoli and „palisading“ of nuclei around vessels, distinct nucleoli - absence of dezmoplastic stroma - absence of dezmoplastic stroma - immunohistochemically: lipase+, trypsin+, chymotripsin+ - immunohistochemically: lipase+, trypsin+, chymotripsin+ CK19-, CK7-, CK20-, AFP (10% positive) CK19-, CK7-, CK20-, AFP (10% positive)

Letný bioptický seminár SD-IAP 21 – Prípad SD-IAP 480  differential diagnosis of hepatoid carcinoma of pancreas 3. pancreatoblastoma - mainly in children (1st. decade of live) - mainly in children (1st. decade of live) - trabecular and solid growth pattern with/without other pancreatic structures - trabecular and solid growth pattern with/without other pancreatic structures (acinar, ductal, endocrine, stromal) (acinar, ductal, endocrine, stromal) - presence of atypical squamoid corpusles - presence of atypical squamoid corpusles - a mesenchymal component with stromal hyalinisation may be present - a mesenchymal component with stromal hyalinisation may be present - immunohistochemically: lipase+, trypsin+, chymotripsin+, AFP+, HMW-CK+ - immunohistochemically: lipase+, trypsin+, chymotripsin+, AFP+, HMW-CK+

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