Congenital Pancreatic Cyst
Presumably due to failure of embryonic pancreatic ducts to regress, when they are replaced by permanent ones, that leads to their obstruction and cyst formation in utero May be associated…
Presumably due to failure of embryonic pancreatic ducts to regress, when they are replaced by permanent ones, that leads to their obstruction and cyst formation in utero May be associated…
Most common lethal autosomal recessive inherited disorder in Caucasian population • CFTR mutation on chromosome 7 Abnormal chloride transport in apical membrane of epithelial cells Clinical Issues • 1 in 2,000-2,500 live…
Progenitor neural crest cells Embryonic celiac ganglion Endodermally derived epithelial cells originating from ventral primordium of pancreas (hamartomatous proliferation) Pancreatic tumor composed of ganglion-islet cell complexes Clinical Issues • Uncommon • 3rd-9th…
Nuclear staining with CAMTA1 immunohistochemistry • Tumors with YAP1-TFE3 Well-formed vascular channels, tumor cells with voluminous eosinophilic cytoplasm Nuclear staining with TFE3 immunohistochemistry Top Differential Diagnoses • Dense myxoid stroma and intracytoplasmic…
Cestode (tapeworm) with wide geographic distribution • E. granulosus (cystic form) and E. multilocularis (alveolar form) most commonly infect humans Etiology/Pathogenesis • Humans infected by exposure to contaminated feces of primary or…
Large areas of fat necrosis along with variable parenchymal necrosis Saponification Hemorrhage, vascular thrombosis • Mild acute pancreatitis is most often clinical (rather than morphologic) diagnosis Spotty peripancreatic or perilobular fat…
Most often seen in children Classic syndrome involves combination of resolving viral illness and salicylate therapy Etiology/Pathogenesis • Pathogenesis unknown • Mitochondrial injury is a key feature • Frequent antecedent viral infection • Salicylate…
Glandular component may contain conventional ductal-type, clear cell, or signet ring cell components Ancillary Tests • p63 is positive in squamous component • Mucin stain, CK7, CK20, CEA, and CA19-9 highlight adenocarcinoma…
Slightly dilated and irregular in shape Lined by single layer of bland endothelial cells • Secondary involutional changes in central portion • Peripheral cellular areas show proliferation of vascular channels in loose…
Varices, ascites if portal hypertension present Jaundice variably present • Cutaneous, gastrointestinal, neuroophthalmic, musculoskeletal, renal, hematological manifestations also common • Nonspecific elevations in transaminases, alkaline phosphatase • Plasma vitamin A levels may be…