OBJECTIVETo report the experience in surveillance and early detection of cholangiocarcinoma (CC) and in using en bloc total hepatectomy-pancreaticoduodenectomy-orthotopic liver transplantation (OLT-Whipple) to achieve complete eradication of early-stage CC complicating primary sclerosing cholangitis (PSC).

METHODSAsymptomatic PSC patients underwent surveillance using endoscopic ultrasound and endoscopic retrograde cholangiopancreatography (ERCP) with multilevel brushings for cytological evaluation. Patients diagnosed with CC were treated with combined extra-beam radiotherapy, lesion-focused brachytherapy, and OLT-Whipple.

RESULTSBetween January 1988 and February 2001, 42 of 119 PSC patients were followed according to the surveillance protocol. CC was detected in 8 patients, 6 of whom underwent OLT-Whipple. Of those 6 patients, 4 had stage I CC, and 2 had stage II CC. All 6 OLT-Whipple patients received combined external-beam and brachytherapy radiotherapy. The median time from diagnosis to OLT-Whipple was 144 days. One patient died 55 months post-transplant of an unrelated cause, without tumor recurrence. The other 5 were well without recurrence at 79, 82, 108, 128, 129 and 145 months.

CONCLUSIONSFor patients with PSC, ERCP surveillance cytology and intralumenal endoscopic ultrasound examination allow for early detection of CC. Broad and lesion-focused radiotherapy combined with OLT-Whipple to remove the biliary epithelium en bloc offers promising long-term, tumor-free survival. All patients tolerated this extensive surgery well with good quality of life following surgery and recovery. These findings support consideration of the complete excision of an intact biliary tree via OLT-Whipple in patients with early-stage hilar CC complicating PSC.

Adolescent ; Adult ; Bile Duct Neoplasms ; diagnosis ; radiotherapy ; surgery ; Bile Ducts, Intrahepatic ; Cholangiocarcinoma ; diagnosis ; radiotherapy ; surgery ; Disease-Free Survival ; Early Diagnosis ; Female ; Follow-Up Studies ; Hepatectomy ; Humans ; Liver Transplantation ; Male ; Mass Screening ; Middle Aged ; Pancreaticoduodenectomy ; Retrospective Studies

Background/Aims: Endoscopic ultrasound (EUS)-guided fine-needle aspiration is very effective for providing specimens for cytological evaluation. However, the ability to provide sufficient tissue for histological evaluation has been challenging due to the technical limitations of dedicated core biopsy needles. Recently, a modified EUS needle has been introduced to obtain tissue core samples for histological analysis. We aimed to determine (1) its ability to obtain specimens for histological assessment and (2) the diagnostic accuracy of EUS-guided fine-needle biopsy (EUS-FNB) using this needle. Methods: We retrospectively analyzed consecutive cases of FNB using modified EUS needles for 342 lesions in 303 patients. The cytology and histological specimens were analyzed. Diagnostic accuracy was calculated. Results: Adequate cytological and histological assessment was possible in 293/342 (86%) and 264/342 (77%) lesions, respectively. Diagnostic accuracy of the cytological specimen was 294/342 (86%) versus 254/342 (74%) for the histological specimen (p<0.01). Diagnostic accuracy of the combined cytological and histological assessment was 323/342 (94.4%), which was significantly higher than that of both histology alone (p<0.001) and cytology alone (p=0.001). Conclusions EUS-FNB with the modified EUS needle provided histologic tissue cores in the majority of cases and achieved excellent diagnostic accuracy with few needle passes.