Biliary Cystadenoma and other complicated cystic lesions of
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
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 Abdominal mass Asymptomatic Abdominal distension Deranged LFT Ankle oedema 9 2 4 2 1 1 • Usually long with mean duration 505 days (4 -3650 days)
Radiological characteristics • • • Right sided Left sided Bilobed Solitary Multiloculated • Mean size 10 10 1 12 8 11. 4 +/- 5. 59 cm
Sonographic and CT features • Internal septations • Mural nodules and papillary projections • Internal echoes • Calcifications • Wall enhancement 14 • “Daughter cysts” 1 7 4 4 3
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 Left hepatectomy Left lateral sectionectomy Extended Right Hepatectomy 1 1
Diagnosis and intervention • Final pathology of 15 patients with pre-op Dx of Biliary Cystadenoma – – – Biliary cystadenomcarcinoma Biliary cystadenoma HCC Liver cyst TB liver abscess Resolving liver abscess 1 6 1 5 1 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 18 cm 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
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