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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