Diagnostic Ultrasound - Abdomen and Pelvis

Biliary Cystadenoma/Carcinoma
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Diagnoses: Liver
○ Considered to have congenital origin, but exact
mechanism unknown
○ Theories: Ectopic rests of embryonal gallbladder tissue
or ectopic ovarian stroma
Gross Pathologic & Surgical Features
• Solitary, multiloculated cystic tumor with thick, welldefined,
fibrous capsule and septations
○ Surface is shiny, smooth, or bosselated
○ Large, polypoid excrescences in wall generally indicate
malignant transformation
Microscopic Features
• Biliary cystadenoma
○ Single layer of benign simple cuboidal or columnar
epithelial cells
– Basally oriented nuclei with prominent nucleolus and
thick chromatin
– Pale acidophil cytoplasm with mucin-filled vacuoles
• Biliary cystadenocarcinoma
○ Multilayered epithelium with many papillary projections
and invasion of stroma
○ Lose epithelial nuclear stratification and tubulopapillary
architecture, nuclear pleomorphism, atypia
• Stromal types
○ Dense, hypercellular, spindle cell ovarian-like stroma (>
85% cases)
– Exclusively seen in women
– Estrogen receptor and progesterone receptor positive
○ Nonovarian-type stroma
– Seen in both sexes
• Cystic fluid: Mucinous, serous, bilious, hemorrhagic, mixed
fluid
Demographics
• Age
○ Predominantly occurs in middle-aged women (40-60
years old)
• Gender
○ Biliary cystadenoma: > 85% of cases occur in women
○ Biliary cystadenocarcinoma: ~ 63% of cases occur in
women
– Higher suspicion for malignancy if detected in men
• Ethnicity
○ Primarily seen in Caucasians
• Epidemiology
○ Biliary cystic neoplasms account for < 5% of all reported
intrahepatic cysts
Natural History & Prognosis
• Complications: Secondary infection, rupture into
peritoneum or retroperitoneum, intracystic hemorrhage
• Recurrence: Inevitable if tumor is not completely resected
• Malignant transformation of biliary cystadenoma occurs in
up to 20-30%
○ Thought to be determined by presence of intestinal
metaplasia, characterized by numerous goblet cells
• Prognosis of biliary cystadenocarcinoma
○ Ovarian-type stromal tumors: Indolent course with
favorable prognosis
○ Nonovarian-type stromal tumors: More aggressive with
poorer prognosis
– Rapid dissemination or distant metastasis
Treatment
• Complete surgical resection
○ Liver resection with negative surgical margins preferred
○ Enucleation: Maximizes preservation of hepatic
parenchyma by using dissection plane between tumor
and liver tissue
– Appropriate for benign lesions to prevent recurrence,
but cannot definitively rule out malignancy
CLINICAL ISSUES
Presentation
• Most common signs/symptoms
○ Found incidentally if too small to be symptomatic
○ Abdominal pain (most common), palpable mass, early
satiety, anorexia, nausea
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○ May cause jaundice or cholangitis with compression of
common bile duct
• Diagnosis:Mostly based on imaging and resection with final
pathology
○ Labs: Normal ALT/AST, bilirubin, and ALP (except with
biliary obstruction)
– Generally normal Ca19-9 and CEA tumor markers (may
be elevated)
○ Fine-needle aspiration
– Generally avoided due to risk of peritoneal
dissemination if malignant
– Cytology inconclusive (sensitivity 50%, specificity
97.6%)
□ Malignant cells not always recovered in cases of
carcinoma
– Frequently show markedly ↑ Ca19-9 and mildly ↑ CEA
□ Unclear whether distinguishes it from simple cysts
○ Intraoperative frozen section analysis
– Cannot exclude malignancy
□ May have undetectable malignant foci or
synchronous carcinoma at borders
http://radiologyebook.com/
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