Biliary Cystadenocarcinoma

Published on 19/07/2015 by admin

Filed under Radiology

Last modified 22/04/2025

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Tumor is encapsulated

Recurs after incomplete excision

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

No enhancement of nodules or septa

• Hepatic pyogenic abscess

Cluster sign: Small abscesses aggregate, sometimes coalesce into single large septate cavity

Rim of abscess locules will enhance

• Cystic metastases

Show debris and mural nodularity

• Hydatid (echinococcal) disease

CLINICAL ISSUES

• Usually occurs in middle-aged women

> 90% in women

DIAGNOSTIC CHECKLIST

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

Almost always solitary

Enhancing mural nodules suggest malignancy

• May mimic hemorrhagic or infected hepatic cyst

(Left) Graphic shows a lobulated complex cystic mass with a vascularized wall and septa.

(Right) Axial CECT in a middle-aged woman shows a complex cystic mass with lobulated margins and an enhancing wall and septa . 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.

(Right) Axial CECT in a middle-aged woman shows a complex cystic mass with lobulated margins and an enhancing wall and septa . 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.

(Left) Axial T1WI MR in a middle-aged woman shows a classic multiseptate, cystic, hepatic mass 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.

(Left) Axial T1WI MR in a middle-aged woman shows a classic multiseptate, cystic, hepatic mass 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.

(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

May arise from intrahepatic bile ducts (IHBDs) within liver (common site)

Very rarely from extrahepatic biliary tree or gallbladder

IMAGING

General Features

• Best diagnostic clue

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

• Location

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

Arising from

– Intrahepatic biliary ducts (83%)

– Extrahepatic bile ducts (13%)

– Gallbladder (0.02%)

• Size

1.5-25 cm in diameter

– Usually large at time of diagnosis if symptomatic

• Key concepts

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

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

Benign and malignant lesions together account for only 5% of all intrahepatic lesions of bile duct origin

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

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

– Some are heterogeneous (cystic and hemorrhagic areas)

Cystadenocarcinoma: Septations and nodularity

Cystadenoma: Septations without nodularity

Mural or septal calcifications are common

Biliary dilatation (due to pressure effect) of IHBDs

– Biliary obstruction considered to favor malignant tumor

• CECT

Multilocular tumor

– Nonenhancing cystic spaces

– Enhancement of internal septa, capsule, and nodules

– Enhancement of papillary excrescences

– Mural or septal calcifications

Less commonly, “honeycomb” or “sponge” appearance (microcystic variant)

– Uncommonly has metastases or adenopathy at initial diagnosis

Unilocular tumor

– Large or small nonenhancing cystic space

– Enhancement of outer capsule and papillary excrescences

– Fine mural calcifications

MR Findings

• T1WI

Variable signal intensity locules depending on content of cystic fluid

High signal intensity (mucoid or hemorrhagic fluid)

Low signal intensity (serous fluid)

Septal or mural calcifications: Hypointense

• T2WI

High signal intensity (serous fluid)

Low signal intensity (mucoid fluid)

Septa are well delineated

Septal or mural calcifications: Hypointense

• T1WI C+

Enhancement of capsule and septa

Ultrasonographic Findings

• Grayscale ultrasound

Large, well-defined, multiloculated, anechoic mass

Highly echogenic septa

Tumor nodules or papillary growths

Mural or septal calcifications or fluid levels

Complex fluid: Areas of anechoic and internal echoes (cystic and hemorrhagic)

Angiographic Findings

• Conventional

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

Stretching and displacement of vessels

Imaging Recommendations

• Best imaging tool

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

Well-defined, round, hypodense mass (0-45 HU)

• Cluster sign: Small abscesses aggregate, sometimes coalesce into single large septate cavity

Rim of abscess locules will enhance

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

Due to pressure effect or rupture into ducts

• May have extrahepatic foci of disease

PATHOLOGY

General Features

• Etiology

Malignant transformation of benign biliary cystadenoma by invasion of capsule

Biliary cystadenoma

– Probably derived from ectopic nests of primitive biliary tissue

Gross Pathologic & Surgical Features

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

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

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

Abdominal pain, obstructive jaundice, nausea, vomiting

Abdominal swelling with palpable mass (90%)

• Diagnosis: Fine-needle aspiration may yield fluid with ↑ CA19-9

Results are not definitive, and cytology is inconclusive

Diagnosis is based mostly on imaging and resection with final pathology

Demographics

• Age

Peak incidence in 5th decade

• Gender

Usually occurs in middle-aged women

Rare in men (< 10%)

• Ethnicity

Predominantly seen in Caucasians

• Epidemiology

Incidence: 5% of all intrahepatic cystic masses

Natural History & Prognosis

• Complications

Rupture into peritoneum or retroperitoneum

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

Tumors with ovarian stroma found in women have indolent course and good prognosis

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

Enhancing mural nodules suggest malignancy

• May mimic hemorrhagic or infected hepatic cyst

(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 within 1 of the larger cystic spaces strongly suggests a neoplastic etiology.

(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 within 1 of the larger cystic spaces strongly suggests a neoplastic etiology.

(Right) Sagittal US in the same patient shows coarse septa within the cystic mass.

(Right) Sagittal US in the same patient shows coarse septa within the cystic mass.

(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.

(Right) Coronal T2WI MR of the same patient shows mural nodularity 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.

(Right) Coronal T2WI MR of the same patient shows mural nodularity 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.

(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.

(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.

(Left) Axial CECT in a 47-year-old woman shows a complex cystic mass with foci of coarse calcification .

(Left) Axial CECT in a 47-year-old woman shows a complex cystic mass with foci of coarse calcification .

(Right) CECT section shows more of the complex cystic mass , 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.

(Right) CECT section shows more of the complex cystic mass , 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.

(Left) The same patient returned 5 years later with acute abdominal pain. Axial CECT shows a large, complex, multiseptate cystic mass with mural nodularity and solid components as well as focal calcifications . The presence of higher-than-water-density fluid in the peritoneal cavity suggests that the mass has ruptured.

(Left) The same patient returned 5 years later with acute abdominal pain. Axial CECT shows a large, complex, multiseptate cystic mass with mural nodularity and solid components as well as focal calcifications . The presence of higher-than-water-density fluid in the peritoneal cavity suggests that the mass has ruptured.

(Right) Axial CT section shows more of the complex cystic mass, including solid mural nodularity , calcification, and exudative or hemorrhagic ascites.

(Right) Axial CT section shows more of the complex cystic mass, including solid mural nodularity , calcification, and exudative or hemorrhagic ascites.

(Left) Coronal-reformatted CT of the same patient shows the large mass with its mural nodularity and coarse calcification, along with complex ascites due to spontaneous rupture of the cystic tumor.

(Left) Coronal-reformatted CT of the same patient shows the large mass with its mural nodularity and coarse calcification, along with complex ascites due to spontaneous rupture of the cystic tumor.

(Right) Coronal CT section shows more of the mass and the focal calcification . 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.

(Right) Coronal CT section shows more of the mass and the focal calcification . 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.

(Left) The initial CECT scan of this 56-year-old man shows a large cystic mass with septa . Note the proximity to, and mass effect on, the IVC and hepatic veins.

(Left) The initial CECT scan of this 56-year-old man shows a large cystic mass with septa . Note the proximity to, and mass effect on, the IVC and hepatic veins.

(Right) CECT section in this patient shows an enhancing septum . At surgery, the mass was resected, but a portion of the posterior wall could not be resected completely due to the adjacent large veins.

(Right) CECT section in this patient shows an enhancing septum . At surgery, the mass was resected, but a portion of the posterior wall could not be resected completely due to the adjacent large veins.

(Left) Repeat CECT scan 1 year later in the same patient shows recurrence of a septate cystic mass. Portions of the radiopaque staple line are visible.

(Left) Repeat CECT scan 1 year later in the same patient shows recurrence of a septate cystic mass. Portions of the radiopaque staple line are visible.

(Right) CECT section in the same study shows the surgical staple line 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.

(Right) CECT section in the same study shows the surgical staple line 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.

(Left) CECT section from the same study shows dilation of intrahepatic bile ducts , which can be considered a bad prognostic sign of invasion, and a malignant character of cystic hepatic tumors.

(Left) CECT section from the same study shows dilation of intrahepatic bile ducts , which can be considered a bad prognostic sign of invasion, and a malignant character of cystic hepatic tumors.

(Right) The classic spindled and cellular ovarian-type stroma is seen underneath the cyst lining of a biliary cystadenoma. (Courtesy M. Yeh, MD, PhD.)

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.

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

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