1. Joint Hospital Surgical Grand Round
A simple liver cyst or a biliary cystadenoma?
The diagnostic challenge
Joint Hospital Surgical Grand Round
Dr Violet Yee-Kei Tsoi
Department of Surgery
Prince of Wales Hospital

2. Case illustration
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

3. Blood tests
Bilirubin 16, ALP 133
CEA 0.5, AFP 3
HBsAg and anti- HCV : Negative
CT abdomen with contrast was performed

4. 19cm huge thick walled cystic lesion arising from left liver

6. Would it be a biliary cystadenoma?
What should be done next?
Keep observe?

8. Progress 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

10. Thickness of wall around 1 cm, unilocular cyst
2.4 L turbid yellowish fluid inside cystic lesion

12. Final Pathology Benign inflammed liver cyst No evidence of malignancy
Mixed inflammatory infiltrate
No ovarian type of stroma

13. Introduction
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

14. Simple liver cyst Benign developmental lesion
Lined by simple cuboidal epithelieum
Surrounding mesenchyme is hypocellular and fibrous
Present in ~ 2.5%- 5% of the population

15. Biliary cystadenoma/cystadenocarcinoma
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

16. Biliary cystadenoma/cystadenocarcinoma
Female predominant
Median age of 50 years old
Malignant transformation of cystadenoma ~ 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): , 1994

17. Clinical Presentation
Similar clinical presentations in liver cyst as well as cystadenoma
Most are asymptomatic
Symptoms:
Abdominal pain ( most common)
Abdominal distension
Palpable mass
Jaundice

18. Imaging – Simple liver cyst
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

19. Imaging – biliary cystadenoma/cystadenocarcinoma
USG
septated, thick-walled, mural nodules
Contrast 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):

20. Imaging – biliary cystadenoma/cystadenocarcinomaCT
Thick fibrous wall, mural nodules, internal septa, capsular calcifications, papillary projections, contrast enhancement of cystic wall
MRI
Homogenous low signal intensity on T1
Signal intensity on T2 depends on cyst content, mostly have high signal

21. Pitfalls
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):

22. Biochemical investigations
Liver function tests
usually normal
Serum 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 :
Intrahepatic biliary cystadenoma: role of cyst fluid analysis and surgical management in the laparoscopic era. Surgery 2004; 136:

23. Cystic fluid analysis Cystic fluid analysis for tumor markers
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

24. Cystic fluid analysis Fluid 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

25. Definite diagnosis can only be made upon histopathology after excision
Intra-operative frozen section
Not reliable
Definite diagnosis can only be made upon histopathology after excision
-Management and long-term follow up of hepatic cysts. Am J Surg 2001;181:
-Cystadenoma and laparoscopic surgery for hepatic cyst disease: a need for laparotomy? Surg Endosc 2005; 19:
-Intrahepatic biliary cystadenoma: a need for radical resection. Eur J of Gastroen & Hepatology 2008, 20:10-14

26. What should we do?

27. Management
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)

28. Conclusion 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

29. The End
Thank you!

30. Prognosis 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): , 1994

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