1. Biliary Cystadenoma and other complicated cystic lesions of the liver: Diagnostic and therapeutic challenges Teoh AYB Division of HBP Surgery Department of Surgery Prince of Wales Hospital CUHK

2. Biliary Cystadenoma Rare cystic neoplasm arising from biliary epithelium Less than 5% of all cystic liver lesions Less than 200 cases described First described by Keen in 1892

3. Histology Defined by Edmund 1958 “Multiloculated cystic lesion lined by mucus secreting cuboidal or columnar epithelium with an accompanying densely cellular “ovarian-like” stroma

4. Biliary Cystadenoma Middle-aged female 85% intrahepatic, but also in extrahepatic biliary tree and gallbladder Potential for malignant transformation up to 20-30% => Biliary cystadenocarcinoma

5. Radiological features USG and CT are complementary* –Internal septations –Thickened and irregular wall –Mural nodules and papillary projections –Calcifications –Wall enhancement * Korobkin et al AJR Am J Roentgenol Sept;153(3):507-11, 1989

6. Radiological features UltrasonographyCT scan

7. Diagnostic challenge Pre-operative diagnosis often difficult Other pathological lesions can mimic clinical and radiological characteristics * : –Simple cyst –Hydatid cyst –Liver abscess –Cystic degeneration of liver neoplasm –Caroli’s disease –Polycystic liver disease *Lewis et al Arch Surg. 123, 563-8, 1988.

8. Treatment Enucleation is the preferred choice of surgery Any intervention short of complete excision will invariably lead to local recurrence

9. Biliary Cystadenoma and other complicated cystic lesions of the liver: Diagnostic and therapeutic challenges Teoh AYB, Ng SSM, Lai PBS Department of Surgery Prince of Wales Hospital CUHK

10. Objective To report on our experience in the diagnosis and management of complex cystic liver lesions at the Prince of Wales Hospital

11. Patients and methods Between January 1995 to Jan 2005 Retrospective review of all case records of patients with complicated lesions of the liver 19 patients underwent a total of 21 operations 3 male and 16 female

12. Data retrieved include: –Clinical presentation –Radiological and pathological characteristics –Types of intervention –Outcome

13. Clinical Presentation Abdominal pain9 Abdominal mass2 Asymptomatic 4 Abdominal distension2 Deranged LFT1 Ankle oedema 1 Usually long with mean duration 505 days (4-3650 days)

14. Radiological characteristics Right sided 10 Left sided 10 Bilobed 1 Solitary 12 Multiloculated8 Mean size11.4 +/- 5.59 cm

15. Sonographic and CT features Internal septations14 Mural nodules and papillary projections 7 Internal echoes4 Calcifications 4 Wall enhancement 3 “Daughter cysts”1

16. Hepatic Angiography Hypovascular lesions 13 out of 17

17. FNAC USG guided FNAC performed in 5 patients with cystadenoma Fluids: dark brown or straw coloured All were negative for malignant or atypical cells 1 cystadenoma had communication with biliary tract => yield is usually low except in cystadenocarcinoma

18. Diagnosis and intervention 15 patients with pre-op Dx of cystadenoma on imaging Enucleation 11 patients Formal Hepatectomy 4 patients –Extended Left Hepatectomy 1 –Left hepatectomy1 –Left lateral sectionectomy1 –Extended Right Hepatectomy1

19. Diagnosis and intervention Final pathology of 15 patients with pre-op Dx of Biliary Cystadenoma –Biliary cystadenomcarcinoma1 –Biliary cystadenoma6 –HCC1 –Liver cyst5 –TB liver abscess1 –Resolving liver abscess1

20. Biliary cystadenoma

21. TB liver abscess

22. Resolving abscess

23. Biliary cystadenoma

24. HCC

25. Biliary cystadenoma

26. Haemorrhagic cyst

27. Diagnosis and intervention Diagnosis of biliary cystadenoma was not suspected in 4 patients 2 patients: symptomatic liver cysts 1 patient: hydatid cyst 1 patient: ruptured infected cyst

28. ? Simple cyst 2 patients with initial Dx of symptomatic cyst underwent Laparoscopic fenestration Early recurrences within 6 months noted (17 and 14 cm) Complete excision: Biliary cystadenoma

29. ? Hydatid Cyst 1 patient with history of traveling had presence of ‘daughter cyst’ within a complex cystic liver lesion Echinococcal indirect haemagglutination test -ve Enucleation: biliary cystadenoma

30. ? Ruptured infected cyst 1 patient presented with acute peritonism EOT found a 18cm ruptured complicated liver cyst Marsupialisation and drainage Histology: Biliary cystadenoma

31. Conclusion Overall diagnostic accuracy 31% (6/19) Clinical and radiological features are not useful in diagnosing biliary cystadenoma FNAC not useful in our series

32. Conclusion New aids for diagnosis Serum CA 19-9 may be raised in some cases of biliary cystadenoma* Cystic fluid analysis for CA 19-9 raised in all 22 cystadenoma † *Lee JH J Gastroenterol Oct;31(5):732-6, 1996. † Koffron A Surgery Oct;136(4):926-36, 2004.

33. How to manage? Rule out differential diagnosis Serial imaging to monitor lesion Refer to HBP specialist

34. How to manage? All suspicious cases should have the cystic lesions completely excised and sent for histological confirmation Any therapy short of complete excision may result in early recurrence