A simple liver cyst or a biliary A simple liver cyst or a biliary cystadenoma?cystadenoma?

The diagnostic challengeThe diagnostic challenge

A 54 years old gentleman presented with epigastric distension and discomfort for 2 months

Ultrasound abdomen performed in private showed a huge liver cystic mass measuring 17cm arising from left lobe of liver

Physical examination:- No pallor or jaundice- No palpable neck lymph nodes- Abdomen: gross hepatomegaly, smooth

surface

Blood tests- Bilirubin 16, ALP 133- CEA 0.5, AFP 3

HBsAg and anti- HCV : Negative CT abdomen with contrast was

performed

19cm huge thick walled cystic lesion arising from left liver19cm huge thick walled cystic lesion arising from left liver

Would it be a biliary cystadenoma?

What should be done next?

Keep observe?

Laparoscopy findings:− No obvious peritoneal nodule- 21 cm thick wall cystic lesion arising from

left lobe of liver

Laparotomy and left hepatectomy of segment 2/3 and part of segment 4 was performed

Thickness of wall around 1 cm, unilocular cyst2.4 L turbid yellowish fluid inside cystic lesion

Benign inflammed liver cyst- No evidence of malignancy- Mixed inflammatory infiltrate- No ovarian type of stroma

Cystic lesions of the liver consist of a heterogenous group of disorders that present diagnostic and therapeutic challenge

In patients presenting with large solitary liver cystic lesion, it is important to distinguish biliary cystadenoma and cystadenocarcinoma from the benign condition of a simple liver cyst

Inappropriate management may lead to recurrence or even malignant change

Benign developmental lesion Lined by simple cuboidal epithelieum Surrounding mesenchyme is

hypocellular and fibrous Present in ~ 2.5%- 5% of the

population

Malignant counterpart of cystadenoma is biliary cystadenocarcinoma

Biliary cystadenoma and cystadenocarcinoma accounts for 5% of all solitary cystic lesions of the liver

Lined by mucus secreting cuboidal or columnar epithelium with densely cellular “ovarian-like” stroma

Cystic content: mucinous (predominant) or serous type

FemaleFemale predominant Median age of 50 years old Malignant transformation Malignant transformation of

cystadenoma ~ 25-30%25-30% Evidenced by histopathology finding

that areas of pre-existing benign cystadenoma were found in cystadenocarcinoma

Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and

immunohistochemical study of 70 patients. The American Journal of surgical pathology 18(11): 1078-1091, 1994

Similar clinical presentations in liver cyst as well as cystadenoma

Most are asymptomatic asymptomatic › Symptoms:- Abdominal pain ( most common)- Abdominal distension- Palpable mass- Jaundice

USG- anechoic with posterior

acoustic enhancement

CT- Appeared homogenous on non-

enhanced CT- No enhancement of its wall or

content after contrast injection

MRI- Homogenous low signal

intensity in T1- Very high signal density on T2

USGUSG- septated, thick-walled, mural

nodules

Contrast enhanced USGContrast enhanced USG- hyper-enhancement of the

cystic wall in the arterial phase and washed out progressively in portal and late phase may indicate the possibility of underlying malignant nature

Diagnosis of biliary cystadenocarcinoma. World J Gastroenterol 2010 Jan 7 ; 16(1): 131-135

CTCT- Thick fibrous wall, mural

nodules, internal septa, capsular calcifications, papillary projections, contrast enhancement of cystic wall

MRIMRI- Homogenous low signal

intensity on T1- Signal intensity on T2

depends on cyst content, mostly have high signal

Not all cystadenomas or cystadenocarcinoma showed the above radiological features

Diagnostic accuracy varies from 30%-95%

Inflammation or hemorrhage into simple hepatic cyst may have misleading radiological features mimicking biliary cystadenoma

Hemorrhagic hepatic cysts mimicking biliary cystadenoma. World J Gastroenterol 2009

Sept 28; 15 (36): 4601-4603

Liver function tests Liver function tests - usually normal

Serum tumor markersSerum tumor markers- CEA and CA 19-9 are usually within normal range- Not diagnostic

- Differential Diagnosis for intrahepatic biliary cystadenoma and hepatic simple cyst. Significance of cystic fluid analysis and radiological findings. J Clini Gastroenterol 2010; 44 :289-293

- Intrahepatic biliary cystadenoma: role of cyst fluid analysis and surgical management in the laparoscopic era. Surgery 2004; 136:926-936

Cystic fluid analysis for tumor Cystic fluid analysis for tumor markersmarkers

- CEA and CA 19-9- Koffron et al reported all 22 patients with biliary

cystadenoma exhibited elevation in cystic fluid CA 19-9

- Few subsequent studies showed no significant difference between two groups of patients in both CEA and CA 19-9

- Not diagnostic

Fluid cytologyFluid cytology

- Presence of atypical cells may suggest malignant lesion of cystadenocarcinoma

- Majority of the cytology results are negative

- Possibility of disease dissemination by fine needle aspiration

Intra-operative frozen sectionIntra-operative frozen section- Not reliable

Definite diagnosis can only be Definite diagnosis can only be made upon histopathology after made upon histopathology after

excisionexcision

-Management and long-term follow up of hepatic cysts. Am J Surg 2001;181: 404410-Cystadenoma and laparoscopic surgery for hepatic cyst disease: a need for

laparotomy? Surg Endosc 2005; 19:1077-1081-Intrahepatic biliary cystadenoma: a need for radical resection. Eur J of Gastroen &

Hepatology 2008, 20:10-14

Complete excision with enucleation, wedge resection, or hepatectomy should be offered if there is any suspicion of biliary cystadenoma or cystadenocarcinoma

If biliary cystadenoma is misdiagnosed and is treated as an simple hepatic cyst, it is associated with risks of malignant transformation and high local recurrence rate with some literature

even reported 100% recurrence

Management and long-term follow-up of hepatic cysts. The American Journal of Surgery 181 (2001) 404-410

Biliary cystadenoma and cystadenocarcinoma is rare

However, no reliable laboratory or radiologic methods can accurately diagnosis it from simple hepatic cyst

Complete excision for any suspicious lesion remains the best method of diagnosis and treatment

Depends on pathology Cystadenoma: good, low recurrence Cystadenocarcinoma- Those arising from pre-existing

cystadenoma with mesenchymal stroma carried a better prognosis after complete excision

Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. The American Journal of surgical pathology 18(11): 1078-1091, 1994

-Differential Diagnosis for intrahepatic biliary cystadenoma and hepatic simple cyst. Significance of cystic fluid analysis and radiological findings. J Clini Gastroenterol 2010; 44 :289-293