Polyarteritis Nodosa and Other Vasculitides
Isolated form rarely progresses to systemic form, but systemic form may develop up to 1 year later • Liver and gallbladder rarely involved in vasculitides other than PAN Churg-Strauss syndrome Rheumatoid…
Pyogenic Abscess
Occurs via portal vein, arterial system, or bile ducts Etiology/Pathogenesis • Bacterial pathogens most common in Western countries Klebsiella pneumoniae is now most common pathogen, followed by Escherichia coli Anaerobes are…
Embryonal Rhabdomyosarcoma
1% of all rhabdomyosarcomas occurring in children Most common malignant neoplasm of extrahepatic biliary tree in children • Occasionally seen in adults, usually in gallbladder • Long-term survival can be achieved with…
Ampullary Adenoma
Most are sporadic • Seen in 50-100% of patients with familial adenomatous polyposis and Gardner syndrome Most common site of extracolonic polyps in syndromic patients • Signs and symptoms of biliary or…
Hemophagocytic Syndromes
Autosomal recessive inheritance 1 in 50,000 live births • Reactive hemophagocytic syndrome (secondary HLH) Infections, malignancies, autoimmune diseases • Hyperproduction of cytokines and chemokines due to T-cell dysregulation that causes “cytokine storm”…
Primary Biliary Cholangitis
> 90% of patients are female Most common in individuals of North European descent • Insidious onset with pruritus, fatigue, jaundice Often other associated autoimmune disorders • AMA(+) Minority of cases are…
Cat-Scratch Disease
Zoonotic infection caused by small, weakly gram-negative coccobacillus Most cases are attributed to B. henselae Clinical Issues • Most cases are children and young adults with history of cat exposure Usually…
Caroli Disease
May fill with bile sludge, stones, &/or pus May affect entire liver or limited to 1 lobe, more commonly left Microscopic • Dilated ducts may show periductal fibrosis, acute and chronic…
Mucinous Cystic Neoplasm
Lined by mucinous columnar epithelium with focal cuboidal, flattened, or papillary areas May have gastric or intestinal metaplasia Varying degrees of dysplasia may be present Densely cellular ovarian-like stroma positive…
Wilson Disease
Inability to excrete copper in bile leads to its accumulation in liver and other tissues Clinical Issues • Variable presentation Acute liver failure, chronic liver disease with fibrosis/cirrhosis, neurologic/neuropsychiatric signs ±…
