Biliary cystadenoma and cystadenocarcinoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Biliary cystadenoma is an uncommon unilocular or multilocular cystic neoplasm that usually arises in the liver or occasionally in the extrahepatic biliary tree and gallbladder. Biliary cystadenomas constitute less than 5% of cystic lesions of the liver. On gross pathology, biliary cystadenoma is unilocular or multilocular cystic lesion filled with fluid or blood. Cystic fluid may be clear and mucinous. On microscopic histopathological analysis, presence of biliary-type epithelium wall supported by ovarian-like stroma is characteristic finding of biliary cystadenoma. The cause of biliary cystadenoma has not been identified. However, it is thought to occur either as a result of the development of ectopic foci of primitive foregut sequestered within the liver or due to the obstruction of the congenitally aberrant bile duct. Neoplastic transformation to cystadenocarcinoma is the most common complication of biliary cystadenoma. Biliary cystadenoma with mesenchymal stroma is associated with most favorable prognosis. Symptoms of biliary cystadenoma include abdominal pain in right upper quadrant, abdominal distension, abdominal mass, nausea, and vomiting. Common physical examination findings of biliary cystadenoma are a palpable, tender mass in the right upper quadrant of abdomen or epigastrium, jaundice, and ascites. On ultrasound, biliary cystadenoma is characterized by fluid filled multiloculated cyst with enhanced transmission. The cyst fluid may contain low level echoes from blood products, mucin, or proteinaceous fluid. Contrast-enhanced ultrasound demonstrates minimal enhancement since the tumors are largely avascular. Surgical resection of tumor is the mainstay of treatment for biliary cystadenoma. Complete enucleation of the cyst is a safe and effective treatment for biliary cystadenoma and hepatic resection is a suitable treatment option for biliary cystadenocarcinoma.

There is no classification system established for biliary cystadenoma and biliary cystadenocarcinoma.

On gross pathology, biliary cystadenoma is unilocular or multilocular cystic lesion filled with fluid. Cystic fluid may be clear and mucinous. On microscopic histopathological analysis, presence of biliary-type epithelium wall, occasional papillary projections, and ovarian-like stroma are characteristic findings of biliary cystadenoma.

The cause of biliary cystadenoma has not been identified. However, it is thought to occur either as a result of the development of ectopic foci of primitive foregut sequestered within the liver or due to the obstruction of the congenitally aberrant bile duct.

Biliary cystadenoma and cystadenocarcinoma must be differentiated from simple liver cysts, liver hematoma, hepatic echinococcal cyst, hepatic abscess, post-traumatic cysts, and bilomas.

Biliary cystadenoma is a rare disease. Females are more commonly affected with biliary cystadenoma than males. The median age at diagnosis of biliary cystadenoma is 45 years.

There are no established risk factors for biliary cystadenoma.

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for biliary cystadenoma.

Common complication of biliary cystadenoma include neoplastic transformation to biliary cystadenocarcinoma. Biliary cystadenoma with mesenchymal stroma is associated with most favorable prognosis.

There is no established system for the staging of biliary cystadenoma and biliary cystadenocarcinoma.

Symptoms of biliary cystadenoma include abdominal pain in right upper quadrant, abdominal distension, abdominal mass, nausea, and vomiting.

Common physical examination findings of biliary cystadenoma are a palpable, tender mass in the right upper quadrant of abdomen or epigastrium, jaundice, and ascites.

Laboratory findings consistent with the diagnosis of biliary cystadenoma include elevation of serum alkaline phosphatase, serum bilirubin, and CA 19-9 in cystic fluid. Some patients with biliary cystadenoma may have leukocytosis with left shift, which is usually suggestive of superinfection of the tumor.

On CT scan, biliary cystadenoma is characterized by fluid filled multiloculated cyst with calcification of septa and cyst wall. The appearance of the cyst fluid on CT is variable depending on its composition.

Abdominal MRI may be helpful in the diagnosis of biliary cystadenoma, which demonstrates variability on T1-and T2-weighted images.

On ultrasound, biliary cystadenoma is characterized by fluid filled multiloculated cyst with enhanced transmission. The cyst fluid may contain low level echoes from blood products, mucin, or proteinaceous fluid. Contrast-enhanced ultrasound demonstrates minimal enhancement since the tumors are largely avascular.

There is no medical therapy for biliary cystadenoma and cystadenocarcinoma. The mainstay of therapy is surgical resection.

Surgical resection of tumor is the mainstay of treatment for biliary cystadenoma. Complete enucleation of the cyst is a safe and effective treatment for biliary cystadenoma and hepatic resection is a suitable treatment option for biliary cystadenocarcinoma.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

In 1777 Maxmillan de Stol described the gallbladder cancer and since studies have established in the identification of the disease and ineffective treatment of this disease.Gallbladder cancer(GBC) are often clinically asymptomatic and an surprising finding at incision, most commonly detected incidentally on histological examination.GBC is characterised by local invasion, intensive regional lymphoid tissue metastasis and distant metastases. In general, GBC is that the most aggressive of the biliary cancers with the shortest median survival period.

• In between 1922 –1956, 4,271 cholecystectomy samples were collected and adenocarcinomas were noted.^[1]
• In between 1962–1999, Stephen et al was to discover the association between calcium deposits and the development of gallbladder cancer is 7%.

• The association between calcium deposits in the gall bladder and the gallbladder cancer was made during 1797.^[2][3][4]
• Current literature evaluation shows that the general occurrence of Porcelain gallbladder cancer, and gallbladder cancer most cancers is 0.2% and 0.8%, respectively—with a 15% concomitant occurrence.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

There is no classification system established for biliary cystadenoma and biliary cystadenocarcinoma.

There is no classification system established for biliary cystadenoma and biliary cystadenocarcinoma.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

On gross pathology, biliary cystadenoma is unilocular or multilocular cystic lesion filled with fluid or blood. Cystic fluid may be clear and mucinous. On microscopic histopathological analysis, presence of biliary-type epithelium wall supported by ovarian-like stroma is the characteristic finding of biliary cystadenoma.

The vast majority of biliary cystadenomas are intrahepatic (97%) with a small proportion extrahepatic (3%). Only rarely are they found in the extrahepatic biliary tree and gallbladder.

• Biliary cystadenomas are cystic neoplasms that may be either unilocular or multilocular.
• The septa within the cyst may rarely show calcification.^[1]
• The majority of biliary cystadenomas do not communicate with the bile ducts, but luminal communication may be occasionally observed.
• The cystic fluid may be clear and mucinous.
• Blood stained fluid within the cyst indicates a malignant component (cystadenocarcinoma).
• Rarely, the fluid may be bile stained, purulent, proteinaceous, or gelatinous.

Biliary cystadenoma is usually a multiloculated cyst covered by a biliary-type epithelium wall and surrounded by an ovarian-like stroma, containing smooth muscle cells. Microscopic evaluation can easily distinguish cystadenoma from cystadenocarcinoma, based on nuclear pleomorphism and loss of nuclear stratification in the latter.^[2]

• Biliary cystadenomas are composed of multiple cysts lined by single layer of cuboidal or nonciliated columnar epithelium that resembles normal biliary epithelium resting on a basement membrane.
• At places the epithelium forms multiple polypoidal or papillary projections.^[1]
• In some of the cases, dysplastic mucinous epithelium itself may proliferate within the bile ducts causing obstruction. This variant is considered an intraductal papillary neoplasm with prominent cystic dilatation of the duct rather than a true biliary cystic neoplasm.^[1]
• The ovarian-like stroma is thick consisting of compact spindle-shaped cells and supports the epithelium and is often observed exclusively in women.
• A histological variant of biliary cystadenomas has been described, namely cystadenomas with mesenchymal stroma. This variant, which is more common in females, is characterized by the presence of spindle cells in the mesenchymal stroma. These cells are capable of differentiating into different cell types, with a high premalignant potential.
• The tumor may express receptors for progesterone while histological characteristics include positivity for vimentin and cytokeratin.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

The cause of biliary cystadenoma has not been identified. However, it is thought to occur either as a result of the development of ectopic foci of primitive foregut sequestered within the liver or due to the obstruction of the congenitally aberrant bile duct.

The cause of biliary cystadenoma has not been identified. However, it is thought to occur either as a result of the development of ectopic foci of primitive foregut sequestered within the liver or due to the obstruction of the congenitally aberrant bile duct.^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Biliary cystadenoma and cystadenocarcinoma must be differentiated from simple liver cysts, liver hematoma, hepatic echinococcal cyst, hepatic abscess, post-traumatic cysts, and bilomas.

Biliary cystadenoma and cystadenocarcinoma must be differentiated from: ^[1][2][3]

• Simple liver cysts
• Hepatic echinococcal cyst
• Hepatic abscess
• Hematoma
• Post-traumatic cysts
• Metastatic ovarian or pancreatic adenocarcinoma
• Bilomas
• Cystic hamartoma
• Embryonal sarcoma
• Polycystic liver disease
• Caroli disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Biliary cystadenoma is a rare disease. The median age at diagnosis of biliary cystadenoma is 45 years. Females are more commonly affected with biliary cystadenoma than males.

• Biliary cystadenomas constitute less than 5% of cystic lesions of the liver.^[1] Biliary cystadenoma presents as an intrahepatic lesion in 90% of cases, while in the remaining cases it involves the extrahepatic biliary tree.^[2]

• The majority of patients are middle-aged women with an average age of 45 at time of diagnosis.^[3]
• The peak frequency has been reported between the fourth and the sixth decades.^[2]
• However, in a few cases, cystadenomas have been observed in the second decade of life.

• The majority of biliary cystadenomas occur in women (80-85%), and this suggested a role for hormonal influence.^[2]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

There are no established risk factors for biliary cystadenoma.

There are no established risk factors for biliary cystadenoma.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for biliary cystadenoma.^[1]

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for biliary cystadenoma.^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Common complication of biliary cystadenoma include neoplastic transformation to biliary cystadenocarcinoma. Biliary cystadenoma with mesenchymal stroma is associated with most favorable prognosis.^[1]

• In fact, even if the cystadenoma is a benign neoplasm, it has a high rate of recurrence and a potential for neoplastic transformation.
• Malignant transformation rate can be as high as 30%.^[2]

Although biliary cystadenomas are benign tumors, they may recur after excision and have potential to develop into biliary cystadenocarcinomas.^[3]

• The prognosis of completely removed billiary cystadenomas is excellent, recurrence is rare.^[4]
• Cystadenomas with mesenchymal stroma are considered premalignant with a good prognosis while those without are known to transform to malignancy more often with a poor prognosis.^[1]

• When treated with radical excision biliary cystadenocarcinomas have good prognosis, particularly those with mesenchymal stroma, unless the tumor invades the adjacent liver tissue or neighboring organs, or metastases are present.^[4]
• Biliary cystadenocarcinomas in men which are not associated with mesenchymal stroma have worse prognosis, even after complete excision.^[4]

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