Choledochal Cyst Laura Webb Lamps, MD Key Facts Terminology Cystic dilatation of biliary tract, usually extrahepatic Clinical Issues Usually presents in childhood; most patients female Most common in Japan Jaundice,…

Nesidioblastosis Vikram Deshpande, MD Key Facts Terminology Morphologic findings associated with functional disorders of β cells (hyperinsulinemic hypoglycemia) in absence of insulinoma Etiology/Pathogenesis Abnormalities in potassium channels Clinical Issues Persistent…

Cystic Fibrosis, Pancreas Mari Mino-Kenudson, MD Key Facts Etiology/Pathogenesis Cystic fibrosis transmembrane conductance regulator (CFTR) gene on chromosome 7q31.2 Clinical Issues Exocrine pancreatic insufficiency occurs in majority of patients Macroscopic…

Congenital Pancreatic Cyst Grace E. Kim, MD Key Facts Terminology Rare benign congenital epithelial-lined cyst that is not associated with ductal obstruction or other pancreatic anomalies Does not communicate with…

Langerhans Cell Histiocytosis Joseph Misdraji, MD Key Facts Etiology/Pathogenesis Clonal proliferation of Langerhans dendritic cells Clinical Issues Affects infants and children Multisystem LCH can involve “risk” organs: Liver, spleen, hematopoietic…

Intrahepatic Bile Duct Cancer Protocol Laura Webb Lamps, MD Intrahepatic Bile Ducts Surgical Pathology Cancer Case Summary (Checklist) Specimen ___ Liver ___ Gallbladder ___ Other (specify): ______________________________ Procedure (select all…

Hepatocellular Carcinoma Cancer Protocol Laura Webb Lamps, MD Hepatocellular Carcinoma: Hepatic Resection Surgical Pathology Cancer Case Summary (Checklist) Specimen (select all that apply) ___Liver ___Gallbladder ___Other (specify):____________________ ___Not specified Procedure…

Hepatectomy Specimen Handling Laura Webb Lamps, MD In order to orient the liver, it is important to identify the right lobe lateral to the falciform ligament and the left lobe…

Undifferentiated Embryonal Sarcoma Hanlin L. Wang, MD, PhD Key Facts Clinical Issues 6-13% of all primary hepatic tumors in childhood > 50% occurring from 6-10 years of age Rarely seen…

Mesenchymal Hamartoma Hanlin L. Wang, MD, PhD Key Facts Etiology/Pathogenesis Developmental anomaly vs. neoplasm Recurrent balanced translocation involving chromosome band 19q13.4 or 19q13.3 Clinical Issues Typically occurring in 1st 2…