Long-term, tumor-free survival after radiotherapy combining hepatectomy-Whipple en bloc and orthotopic liver transplantation for early-stage hilar cholangiocarcinoma

Abstract

This retrospective study reviews our 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). Asymptomatic 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. Between 1988 and 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 are well without recurrence at 5.7, 7.0, 8.7, 8.8, and 10.1 years. In conclusion, for 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. Liver Transpl 14: [279][280][281][282][283][284][285][286] 2008 Hilar cholangiocarcinoma (CC) is a deadly malignant tumor that arises from the bile duct epithelium. 1 CC is often diagnosed at an untreatable advanced stage. National tumor registry data show that approximately two-thirds of patients with CC have, at minimum, regional lymph node or adjacent organ involvement at presentation. Such advanced disease is uniformly fatal. 2 Although surgical strategies afford patients the best chance for short-term survival, with resection, 5-year survival is less than 50%, despite a notably high rate (nearly 90%) of complete excision. [3][4][5][6][7][8][9][10][11] Orthotopic liver transplantation (OLT) for otherwise unresectable CC has also been associated with limited success, with 5-year survival reported to range from 0% to 55% (transplant registry data: 23%). 4,[12][13][14][15][16][17][18][19][20] Because of this poor survival after OLT, CC has become recognized as a contraindication to OLT (except in experimental protocols). However, a recent report describing chemoirradiation followed by OLT for early-stage CC located above the cystic duct demonstrated encouraging 5-year Abbreviations: CC, cholangiocarcinoma; ERCP, endoscopic retrograde cholangiopancreatography; EUS, endoscopic ultrasound ex...

Liver transplantation and whipple surgery combined with chemoradiotherapy for treatment of hilar cholangiocarcinoma in patients with primary sclerosing cholangitis

“…Another series of 4 patients with extrahepatic CC in the context of PSC showed tumor-free survival of 39.5 months after OLT-Whipple surgery. 10 Wu et al 4 described 6 patients with stage I and II CC who were managed with OLT-Whipple surgery combined with external-beam and brachytherapy radiotherapy. Five of 6 patients were alive without tumor recurrence 5 years after surgery.…”

“…3 OLT-Whipple surgery (en bloc total hepatectomypancreaticoduodenectomy-OLT) combined with radiotherapy has been reported as an approach to improve the survival rates of PSC patients with hilar CC. 4 Herein, we describe 4 PSC patients affected with CC who were treated with liver transplantation and Whipple surgery combined with perioperative chemoradiotherapy. This study was approved by our local institutional review board.…”

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“…[27][28][29] If extended extrahepatic bile duct resection, including pancreatic head resection in combination with LT, leads to improved survival in extrahepatic CCA patients; however, partly because of increased perioperative mortality, most centers do not have improved results with this patient collective. [14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] For patients with anatomically unresectable Klatskin tumors or considerable parenchymal damage, extended liver resection is not a choice. For these patients, LT is the only chance for a potential cure.…”

Pancreatic Diseases: The Need to Assess the Quality of Life Raffaele Pezzilli

“…In addition, in patients operated on for malignant disease, the QoL may also be impaired by recurrence of the disease and the need for adjuvant chemotherapy. The data on clinical outcomes and PROs in patients who underwent PHR for various reasons are based either on a large number of patients enrolled over a long period [45,46] or on a small number of cases enrolled over a short period [28,47,48]. The results of these studies are not conclusive; in patients enrolled over a long period, the survival rate evaluated retrospectively suffers from changes in their care whereas studies carried out over a short period do not have definitive conclusions.…”