a simple liver cyst or a biliary cystadenoma? the diagnostic challenge
TRANSCRIPT
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A simple liver cyst or a biliary A simple liver cyst or a biliary cystadenoma?cystadenoma?
The diagnostic challengeThe diagnostic challenge
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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
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Blood tests- Bilirubin 16, ALP 133- CEA 0.5, AFP 3
HBsAg and anti- HCV : Negative CT abdomen with contrast was
performed
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19cm huge thick walled cystic lesion arising from left liver19cm huge thick walled cystic lesion arising from left liver
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Would it be a biliary cystadenoma?
What should be done next?
Keep observe?
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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
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Thickness of wall around 1 cm, unilocular cyst2.4 L turbid yellowish fluid inside cystic lesion
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Benign inflammed liver cyst- No evidence of malignancy- Mixed inflammatory infiltrate- No ovarian type of stroma
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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
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Benign developmental lesion Lined by simple cuboidal epithelieum Surrounding mesenchyme is
hypocellular and fibrous Present in ~ 2.5%- 5% of the
population
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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
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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
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Similar clinical presentations in liver cyst as well as cystadenoma
Most are asymptomatic asymptomatic › Symptoms:- Abdominal pain ( most common)- Abdominal distension- Palpable mass- Jaundice
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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-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