Biliary Cystadenoma and other complicated cystic lesions of the liver: Diagnostic and therapeutic challenges

Teoh AYBDivision of HBP Surgery Department of Surgery

Prince of Wales Hospital CUHK

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

Histology

• Defined by Edmund 1958

“Multiloculated cystic lesion lined by mucus secreting cuboidal or columnar epithelium with an accompanying densely cellular “ovarian-like” stroma

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

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

Radiological features

Ultrasonography CT scan

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.

Treatment

• Enucleation is the preferred choice of surgery

• Any intervention short of complete excision will invariably lead to local recurrence

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

Objective

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

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

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

Clinical Presentation

• Abdominal pain 9• Abdominal mass 2• Asymptomatic 4• Abdominal distension 2• Deranged LFT 1• Ankle oedema 1

• Usually long with mean duration 505 days (4-3650 days)

Radiological characteristics

• Right sided 10 • Left sided 10• Bilobed 1• Solitary 12• Multiloculated 8

• Mean size 11.4 +/- 5.59 cm

Sonographic and CT features

• Internal septations 14• Mural nodules and

papillary projections 7• Internal echoes 4• Calcifications 4• Wall enhancement 3

• “Daughter cysts” 1

Hepatic Angiography

• Hypovascular lesions 13 out of 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

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 hepatectomy 1– Left lateral sectionectomy 1– Extended Right Hepatectomy 1

Diagnosis and intervention

• Final pathology of 15 patients with pre-op Dx of Biliary Cystadenoma

– Biliary cystadenomcarcinoma 1– Biliary cystadenoma 6– HCC 1– Liver cyst 5– TB liver abscess 1– Resolving liver abscess 1

Biliary cystadenoma

TB liver abscess

Resolving abscess

Biliary cystadenoma

HCC

Biliary cystadenoma

Haemorrhagic cyst

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

? 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

? 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

? Ruptured infected cyst

• 1 patient presented with acute peritonism

• EOT found a 18cm ruptured complicated liver cyst

• Marsupialisation and drainage

• Histology: Biliary cystadenoma

Conclusion

• Overall diagnostic accuracy 31% (6/19)

• Clinical and radiological features are not useful in diagnosing biliary cystadenoma

• FNAC not useful in our series

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.

How to manage?

• Rule out differential diagnosis

• Serial imaging to monitor lesion

• Refer to HBP specialist

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