Biliary Cystadenocarcinoma

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[level-membership-for-radiology-category] Tumor is encapsulated

image Recurs after incomplete excision
image Variable MR signal intensity locules depending on content of cystic fluid
• Malignant potential to develop into cystadenocarcinoma even after years of stability

TOP DIFFERENTIAL DIAGNOSES

• Hemorrhagic or infected hepatic cyst

image No enhancement of nodules or septa
• Hepatic pyogenic abscess

image Cluster sign: Small abscesses aggregate, sometimes coalesce into single large septate cavity
image Rim of abscess locules will enhance
• Cystic metastases

image Show debris and mural nodularity
• Hydatid (echinococcal) disease

CLINICAL ISSUES

• Usually occurs in middle-aged women

image > 90% in women

DIAGNOSTIC CHECKLIST

• Large, well-defined, homogeneous or heterogeneous, complex cystic mass with septations and nodularity

image Almost always solitary
image Enhancing mural nodules suggest malignancy
• May mimic hemorrhagic or infected hepatic cyst
image
(Left) Graphic shows a lobulated complex cystic mass with a vascularized wall and septa.

image
(Right) Axial CECT in a middle-aged woman shows a complex cystic mass with lobulated margins and an enhancing wall and septa image. These findings in a patient with no other known tumor could be considered sufficiently diagnostic of a biliary cystadenoma to warrant resection without further evaluation.
image
(Left) Axial T1WI MR in a middle-aged woman shows a classic multiseptate, cystic, hepatic mass image with cyst contents having features characteristic of fairly simple fluid. There is slight heterogeneity of the signal that varies between some of the cyst compartments.

image
(Right) Axial T2WI MR in the same patient shows the bright signal of fluid content within the large septate mass.

TERMINOLOGY

Synonyms

• Bile duct cystadenocarcinoma, hepatobiliary cystadenoma

Definitions

• Rare, malignant or premalignant, unilocular or multilocular, cystic tumor

image May arise from intrahepatic bile ducts (IHBDs) within liver (common site)
image Very rarely from extrahepatic biliary tree or gallbladder

IMAGING

General Features

• Best diagnostic clue

image Complex, multiloculated, cystic mass in liver, often with septations and mural calcifications
• Location

image Right lobe (55%), left lobe (29%), both lobes (16%)
image Arising from

– Intrahepatic biliary ducts (83%)
– Extrahepatic bile ducts (13%)
– Gallbladder (0.02%)
• Size

image 1.5-25 cm in diameter

– Usually large at time of diagnosis if symptomatic
• Key concepts

image Biliary cystadenocarcinoma

– Malignant transformation of benign biliary cystadenoma
– Typically solitary tumor; usually multilocular, but sometimes unilocular
– Tumor is encapsulated
– Usually seen in middle-aged women
– Recurs after incomplete excision
image Benign biliary cystadenoma

– Probably congenital in origin due to presence of aberrant bile ducts
– Recurs after incomplete excision
– Malignant potential to develop into cystadenocarcinoma even after years of stability
image Benign and malignant lesions together account for only 5% of all intrahepatic lesions of bile duct origin
image Microcystic cystadenoma variant

– Composed of multiple small cysts
– Glycogen-rich cystadenoma
– Typical papillary and mesenchymal stromal features are not seen
– Lined by single layer of cuboidal epithelial cells
– Resembles serous microcystic adenoma of pancreas in pathology and on imaging

CT Findings

• NECT

image Large, well-defined, homogeneous, hypodense, water-density mass

– Some are heterogeneous (cystic and hemorrhagic areas)
image Cystadenocarcinoma: Septations and nodularity
image Cystadenoma: Septations without nodularity
image Mural or septal calcifications are common
image Biliary dilatation (due to pressure effect) of IHBDs

– Biliary obstruction considered to favor malignant tumor
• CECT

image Multilocular tumor

– Nonenhancing cystic spaces
– Enhancement of internal septa, capsule, and nodules
– Enhancement of papillary excrescences
– Mural or septal calcifications

image Less commonly, “honeycomb” or “sponge” appearance (microcystic variant)
– Uncommonly has metastases or adenopathy at initial diagnosis
image Unilocular tumor

– Large or small nonenhancing cystic space
– Enhancement of outer capsule and papillary excrescences
– Fine mural calcifications

MR Findings

• T1WI

image Variable signal intensity locules depending on content of cystic fluid
image High signal intensity (mucoid or hemorrhagic fluid)
image Low signal intensity (serous fluid)
image Septal or mural calcifications: Hypointense
• T2WI

image High signal intensity (serous fluid)
image Low signal intensity (mucoid fluid)
image Septa are well delineated
image Septal or mural calcifications: Hypointense
• T1WI C+

image Enhancement of capsule and septa

Ultrasonographic Findings

• Grayscale ultrasound

image Large, well-defined, multiloculated, anechoic mass
image Highly echogenic septa
image Tumor nodules or papillary growths
image Mural or septal calcifications or fluid levels
image Complex fluid: Areas of anechoic and internal echoes (cystic and hemorrhagic)

Angiographic Findings

• Conventional

image Avascular mass with small clusters of peripheral abnormal vessels in wall and septa
image Stretching and displacement of vessels

Imaging Recommendations

• Best imaging tool

image Multiplanar CECT or MR

DIFFERENTIAL DIAGNOSIS

Hemorrhagic or Infected Hepatic Cyst

• Complex heterogeneous cystic mass
• Multiple thick or thin septations
• May show mural nodularity and fluid level
• Calcification may be seen
• No enhancement of nodules or septa
• Hepatic cysts are usually multiple

Hepatic Pyogenic Abscess

• Simple pyogenic abscess

image Well-defined, round, hypodense mass (0-45 HU)
• Cluster sign: Small abscesses aggregate, sometimes coalesce into single large septate cavity

image Rim of abscess locules will enhance
image Contents > water density, no enhancement, ± gas
• Often associated with diaphragmatic elevation, atelectasis, and right-side pleural effusion

Cystic Metastases

• Usually from ovarian cystadenocarcinoma or metastatic sarcoma
• Show debris and mural nodularity
• May have thick septa and wall enhancement
• Usually multiple

Hydatid (Echinococcal) Disease

• Large, well-defined, cystic liver mass
• Often has numerous peripheral “daughter” cysts or scolices of different density or intensity
• May show curvilinear or ring-like pericyst calcification
• Occasionally dilated intrahepatic bile ducts

image Due to pressure effect or rupture into ducts
• May have extrahepatic foci of disease

PATHOLOGY

General Features

• Etiology

image Malignant transformation of benign biliary cystadenoma by invasion of capsule
image Biliary cystadenoma

– Probably derived from ectopic nests of primitive biliary tissue

Gross Pathologic & Surgical Features

• Solitary, multiloculated cystic tumor with well-defined thick capsule

image Contents: Serous, mucinous, bilious, hemorrhagic, or mixed fluid
• Surface is shiny, smooth, or bosselated
• Polypoid excrescences and septations may be seen

Microscopic Features

• Single layer of cuboidal or tall columnar biliary type epithelium with papillary projections
• Malignant epithelial cells may line cysts
• Subepithelial stroma resembles that of ovary

image Common feature of cystic tumors throughout abdomen (e.g., mucinous cystic tumor of pancreas)
• Usually mucinous, but serous type is also seen
• Goblet cells, Paneth cells, and argyrophilic endocrine cells may be seen

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Abdominal pain, obstructive jaundice, nausea, vomiting
image Abdominal swelling with palpable mass (90%)
• Diagnosis: Fine-needle aspiration may yield fluid with ↑ CA19-9

image Results are not definitive, and cytology is inconclusive
image Diagnosis is based mostly on imaging and resection with final pathology

Demographics

• Age

image Peak incidence in 5th decade
• Gender

image Usually occurs in middle-aged women
image Rare in men (< 10%)
• Ethnicity

image Predominantly seen in Caucasians
• Epidemiology

image Incidence: 5% of all intrahepatic cystic masses

Natural History & Prognosis

• Complications

image Rupture into peritoneum or retroperitoneum
image Recurrence common

– Almost inevitable unless entire rim of tumor is excised (often impossible due to adherence to vital hepatic vessels or inferior vena cava)
• Prognosis

image Tumors with ovarian stroma found in women have indolent course and good prognosis
image Tumors without ovarian stroma found in both sexes have aggressive clinical course and poor prognosis

Treatment

• Complete surgical resection

DIAGNOSTIC CHECKLIST

Consider

• Rule out other complex cystic masses of liver

Image Interpretation Pearls

• Large, well-defined, homogeneous or heterogeneous, complex cystic mass with septations and nodularity

image Enhancing mural nodules suggest malignancy
• May mimic hemorrhagic or infected hepatic cyst
image
(Left) Sonography in a 69-year-old woman with RUQ fullness and discomfort shows a complex cystic hepatic mass with fluid contents of slightly variable nature and multiple discrete septa. Mural nodularity image within 1 of the larger cystic spaces strongly suggests a neoplastic etiology.

image
(Right) Sagittal US in the same patient shows coarse septa image within the cystic mass.
image
(Left) Axial T2WI MR of the same patient shows a complex cystic hepatic mass with fluid contents of a slightly variable nature and multiple discrete septa.

image
(Right) Coronal T2WI MR of the same patient shows mural nodularity image within 1 of the larger cystic spaces, strongly suggesting a neoplastic etiology. This proved to be a biliary cystadenoma with foci of cellular atypia in the mucosal lining.
image
(Left) Axial CECT in a middle-aged woman with microcystic cystadenocarcinoma shows innumerable tiny cystic spaces with a “honeycomb” or sponge-like appearance reminiscent of serous microcystic adenoma of the pancreas.

image
(Right) Gross pathology shows the cut surface of a resected mass, revealing innumerable small cystic spaces with a “honeycomb” or sponge-like appearance. Diagnosis was microcystic variant of biliary cystadenocarcinoma.
image
(Left) Axial CECT in a 47-year-old woman shows a complex cystic mass image with foci of coarse calcification image.

image
(Right) CECT section shows more of the complex cystic mass image, which was clearly separate from the gallbladder. This was interpreted as an “indeterminate mass” with a recommendation for follow-up, but no differential diagnosis nor recommended interval for follow-up was provided.
image
(Left) The same patient returned 5 years later with acute abdominal pain. Axial CECT shows a large, complex, multiseptate cystic mass image with mural nodularity and solid components image as well as focal calcifications image. The presence of higher-than-water-density fluid in the peritoneal cavity suggests that the mass has ruptured.

image
(Right) Axial CT section shows more of the complex cystic mass, including solid mural nodularity image, calcification, and exudative or hemorrhagic ascites.
image
(Left) Coronal-reformatted CT of the same patient shows the large mass image with its mural nodularity and coarse calcification, along with complex ascites due to spontaneous rupture of the cystic tumor.

image
(Right) Coronal CT section shows more of the mass image and the focal calcification image. At surgery (right trisegmentectomy), a ruptured biliary cystadenocarcinoma was confirmed. This lesion could have been resected 5 years earlier with much less morbidity and with a better prognosis.
image
(Left) The initial CECT scan of this 56-year-old man shows a large cystic mass with septa image. Note the proximity to, and mass effect on, the IVC image and hepatic veins.

image
(Right) CECT section in this patient shows an enhancing septum image. At surgery, the mass was resected, but a portion of the posterior wall could not be resected completely due to the adjacent large veins.
image
(Left) Repeat CECT scan 1 year later in the same patient shows recurrence of a septate cystic mass. Portions of the radiopaque staple line image are visible.

image
(Right) CECT section in the same study shows the surgical staple line image and a multiseptate cystic mass very similar in size and appearance to the tumor seen before surgery. Incompletely resected biliary cystadenomas and cystadenocarcinomas often recur.
image
(Left) CECT section from the same study shows dilation of intrahepatic bile ducts image, which can be considered a bad prognostic sign of invasion, and a malignant character of cystic hepatic tumors.

image
(Right) The classic spindled and cellular ovarian-type stroma is seen underneath the cyst lining of a biliary cystadenoma. (Courtesy M. Yeh, MD, PhD.)
image
Axial CECT in a young female with prior left hepatectomy shows a recurrent multiloculated mass with large cystic spaces, visible wall, and septa. Diagnosis was biliary cystadenocarcinoma.

image
Transverse sonogram shows anechoic cystic spaces separated by thin and thick septa.

SELECTED REFERENCES

1. Arnaoutakis, DJ, et al. Management of biliary cystic Tumors: a multi-institutional analysis of a rare liver tumor. Ann Surg. 2014. [ePub].

2. Chen, YW, et al. Surgical management of biliary cystadenoma and cystadenocarcinoma of the liver. Genet Mol Res. 2014; 13(3):6383–6390.

3. Cogley, JR, et al. MR imaging of benign focal liver lesions. Radiol Clin North Am. 2014; 52(4):657–682.

4. Doepker, M, et al. Biliary cystadenoma: case series and review of the literature. Am Surg. 2011; 77(4):505–506.

5. Sang, X, et al. Hepatobiliary cystadenomas and cystadenocarcinomas: a report of 33 cases. Liver Int. 2011; 31(9):1337–1344.

 

Romagnoli, R, et al. Liver transplantation for symptomatic centrohepatic biliary cystadenoma. Clin Res Hepatol Gastroenterol. 2011; 35(5):408–413.

Bogert, JN, et al. Education and imaging. Hepatobiliary and pancreatic: Mucinous cystadenoma of the biliary tree. J Gastroenterol Hepatol. 2010; 25(7):1332.

Choi, HK, et al. Differential diagnosis for intrahepatic biliary cystadenoma and hepatic simple cyst: significance of cystic fluid analysis and radiologic findings. J Clin Gastroenterol. 2010; 44(4):289–293.

Seo, JK, et al. Appropriate diagnosis of biliary cystic tumors: comparison with atypical hepatic simple cysts. Eur J Gastroenterol Hepatol. 2010; 22(8):989–996.

Yu, J, et al. Hepatobiliary mucinous cystadenoma and cystadenocarcinoma: report of six cases and review of the literature. Hepatogastroenterology. 2010; 57(99–100):451–455.

Limongelli, P, et al. Cystic tumors of the biliary tract: a complete excision is crucial. Int Surg. 2009; 94(2):136–140.

Del Poggio, P, et al. Cystic tumors of the liver: a practical approach. World J Gastroenterol. 2008; 14(23):3616–3620.

Delis, SG, et al. Intrahepatic biliary cystadenoma: a need for radical resection. Eur J Gastroenterol Hepatol. 2008; 20(1):10–14.

Mourra, N, et al. Clinical challenges and images in GI. Extrahepatic biliary cystadenoma with mesenchymal stroma. Gastroenterology. 2008; 134(5):1295. [1637].

Levy, AD, et al. From the archives of the AFIP. Benign tumors and tumorlike lesions of the gallbladder and extrahepatic bile ducts: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 2002; 22(2):387–413.

Mortelé, KJ, et al. Cystic focal liver lesions in the adult: differential CT and MR imaging features. Radiographics. 2001; 21(4):895–910.

Gabata, T, et al. Biliary cystadenoma with mesenchymal stroma of the liver: correlation between unusual MR appearance and pathologic findings. J Magn Reson Imaging. 1998; 8(2):503–504.

Singh, Y, et al. Multiloculated cystic liver lesions: radiologic-pathologic differential diagnosis. Radiographics. 1997; 17(1):219–224.

Buetow, PC, et al. Biliary cystadenoma and cystadenocarcinoma: clinical-imaging-pathologic correlations with emphasis on the importance of ovarian stroma. Radiology. 1995; 196(3):805–810.

[/level-membership-for-radiology-category][not-level-membership-for-radiology-category] Tumor is encapsulated

image Recurs after incomplete excision
image Variable MR signal intensity locules depending on content of cystic fluid
• Malignant potential to develop into cystadenocarcinoma even after years of stability

TOP DIFFERENTIAL DIAGNOSES

• Hemorrhagic or infected hepatic cyst

image No enhancement of nodules or septa
• Hepatic pyogenic abscess

image Cluster sign: Small abscesses aggregate, sometimes coalesce into single large septate cavity
image Rim of abscess locules will enhance
• Cystic metastases

image Show debris and mural nodularity
• Hydatid (echinococcal) disease

CLINICAL ISSUES

• Usually occurs in middle-aged women

image > 90% in women

DIAGNOSTIC CHECKLIST

• Large, well-defined, homogeneous or heterogeneous, complex cystic mass with septations and nodularity

image Almost always solitary
image Enhancing mural nodules suggest malignancy
• May mimic hemorrhagic or infected hepatic cyst
image
(Left) Graphic shows a lobulated complex cystic mass with a vascularized wall and septa.

image
(Right) Axial CECT in a middle-aged woman shows a complex cystic mass with lobulated margins and an enhancing wall and septa image. These findings in a patient with no other known tumor could be considered sufficiently diagnostic of a biliary cystadenoma to warrant resection without further evaluation.
image
(Left) Axial T1WI MR in a middle-aged woman shows a classic multiseptate, cystic, hepatic mass image with cyst contents having features characteristic of fairly simple fluid. There is slight heterogeneity of the signal that varies between some of the cyst compartments.

image
(Right) Axial T2WI MR in the same patient shows the bright signal of fluid content within the large septate mass.

TERMINOLOGY

Synonyms

• Bile duct cystadenocarcinoma, hepatobiliary cystadenoma

Definitions

• Rare, malignant or premalignant, unilocular or multilocular, cystic tumor

image May arise from intrahepatic bile ducts (IHBDs) within liver (common site)
image Very rarely from extrahepatic biliary tree or gallbladder

IMAGING

General Features

• Best diagnostic clue

image Complex, multiloculated, cystic mass in liver, often with septations and mural calcifications
• Location

image Right lobe (55%), left lobe (29%), both lobes (16%)
image Arising from

– Intrahepatic biliary ducts (83%)
– Extrahepatic bile ducts (13%)
– Gallbladder (0.02%)
• Size

image 1.5-25 cm in diameter

– Usually large at time of diagnosis if symptomatic
• Key concepts

image Biliary cystadenocarcinoma

– Malignant transformation of benign biliary cystadenoma
– Typically solitary tumor; usually multilocular, but sometimes unilocular
– Tumor is encapsulated
– Usually seen in middle-aged women
– Recurs after incomplete excision
image Benign biliary cystadenoma

– Probably congenital in origin due to presence of aberrant bile ducts
– Recurs after incomplete excision
– Malignant potential to develop into cystadenocarcinoma even after years of stability
image Benign and malignant lesions together account for only 5% of all intrahepatic lesions of bile duct origin
image Microcystic cystadenoma variant

– Composed of multiple small cysts
– Glycogen-rich cystadenoma
– Typical papillary and mesenchymal stromal features are not seen
– Lined by single layer of cuboidal epithelial cells
– Resembles serous microcystic adenoma of pancreas in pathology and on imaging

CT Findings

• NECT

image Large, well-defined, homogeneous, hypodense, water-density mass

– Some are heterogeneous (cystic and hemorrhagic areas)
image Cystadenocarcinoma: Septations and nodularity
image Cystadenoma: Septations without nodularity
image Mural or septal calcifications are common
image Biliary dilatation (due to pressure effect) of IHBDs

– Biliary obstruction considered to favor malignant tumor
• CECT

image Multilocular tumor

– Nonenhancing cystic spaces
– Enhancement of internal septa, capsule, and nodules
– Enhancement of papillary excrescences
– Mural or septal calcifications

image Less commonly, “honeycomb” or “sponge” appearance (microcystic variant)
– Uncommonly has metastases or adenopathy at initial diagnosis
image Unilocular tumor

– Large or small nonenhancing cystic space
– Enhancement of outer capsule and papillary excrescences
– Fine mural calcifications

MR Findings

• T1WI

image Variable signal intensity locules depending on content of cystic fluid
image High signal intensity (mucoid or hemorrhagic fluid)
image Low signal intensity (serous fluid)
image Septal or mural calcifications: Hypointense
• T2WI

image High signal intensity (serous fluid)
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