- Case Report
- Open Access
A long survivor with local relapse of hilar cholangiocarcinoma after R1 surgery treated with chemoradiotherapy: a case report and literature review
- Hirohisa Okabe1, 3,
- Akira Chikamoto1,
- Masataka Maruno1,
- Daisuke Hashimoto1,
- Katsunori Imai1,
- Katsunobu Taki1,
- Kota Arima1,
- Takatoshi Ishiko1,
- Hideaki Uchiyama3,
- Toru Ikegami3,
- Norifumi Harimoto3,
- Shinji Itoh3,
- Tomoharu Yoshizumi3,
- Toru Beppu2,
- Hideo Baba1Email authorView ORCID ID profile and
- Yoshihiko Maehara3
© The Author(s). 2016
Received: 8 January 2016
Accepted: 22 June 2016
Published: 4 July 2016
The treatment outcome of extrahepatic cholangiocarcinoma remains insufficient because it is difficult to obtain accurate diagnosis of tumor spreading and effective treatment agent is quite limited in spite of substantial current efforts, all of which have been unsuccessful except for gemcitabine plus cisplatin. The patient was a 60-year-old female who had developed hilar cholangiocarcinoma and underwent extrahepatic bile duct resection. Although it was conceivable that it would be the R1 resection, the patient wanted to receive limited resection to avoid postoperative complication mainly because she was depressed. In histology, interstitial spreading of tumor was appreciated at the surgical margin of bile duct. The patient did not accept to receive the additional treatment after the surgery and hardly visited the hospital to take the periodical test for monitoring the residual cancer cells. As expected, the local relapse of tumor was appreciated 1 year after the R1 surgery. She chose radiotherapy and agreed with subsequent S-1 treatment for 26 months. Consequently, elevated CA19-9 was decreased, and local relapse has been successfully controlled for more than 7 years after the relapse of tumor. Here, we report quite a rare case in terms of long survivor after chemoradiotherapy on locally relapsed unresectable hilar cholangiocarcinoma.
Curative resection of extrahepatic cholangiocarcinoma remains challenging mainly due to the limitation of diagnosis regarding vertical and horizontal tumor spreading. Although prognosis of R1 surgery on extrahepatic cholangiocarcinoma is reported to be better than that of best supportive care, the optimal additional treatment for R1 surgery is controversial and has not been reviewed so far. Here, we aim to report a case who has successfully survived for long time after R1 surgery on hilar cholangiocarcinoma and review the treatment on extrahepatic cholangiocarcinoma patients with R1 surgery.
Surgical resection or liver transplantation remains the only curative treatment that can offer long-term survival for hilar cholangiocarcinoma (Hl-CC) [1, 2]. Despite the preoperative and surgical advances for Hl-CC, some patients still have microscopic positive resection (R1) and recurrence occurs in many of them even after curative resection (R0). For patients who unfortunately have unresectable Hl-CC or the unresectable recurrence after curative resection, gemcitabine plus cisplatin is the only effective therapeutic agent that has been globally appreciated based on the proper clinical study to date . Here, we report the case representing the local relapse of Hl-CC after R1 surgery and long-term survival with the treatment of radiation and subsequent chemotherapy and discuss the previous outcome of concomitant treatment plus surgery in extrahepatic cholangiocarcinoma (ECC) patients with R1 surgery.
Outcome of concomitant treatment plus R1 surgery in patients with ECC
Patient and treatment for residual cancer cells
Number of R1 patients
Locoregional failure (%)
Distant failure (%)
Surgery + RT (45–59 Gy) + CTx (5FUa or GEM)
Surgery + RT (45–50Gy) + CTx (GEM)
Surgery → local relapse → EBRT (48Gy) + CTx (CDDP or 5FUa)
Surgery + CTx (5FUa + CDDP or GEM)
Surgery + RT (40–50 Gy)
Surgery + CRT (5FUa or GEM + RT)
To date, only gemcitabine plus cisplatin has been accepted for first-line therapy for unresectable biliary tract cancer , and the subsequent challenges fail to establish the next promising therapeutic agent [11, 12]. S1 treatment might be effective for some patients if we can select the good responder to S1 treatment or arrange the optimal dose of drug . In this case, S1 treatment plus radiotherapy was eventually effective. However, it was difficult to determine when adjuvant S1 treatment was finished. The patient hoped to keep the S1 treatment, and we continued it for more than 2 years. Translational research is required for finding the biomarker selecting those patients responsive to 5-FU based treatment or the new strategy of how to use 5-FU based chemotherapeutic agent and how long to continue the treatment. Since the efficacy of radiotherapy alone is limited in terms of the locoregional therapy, the combination of chemotherapy and radiotherapy seems to be best additional option to control residual cancer cells at the locoregional area as well as latent cells in distant organ. Furthermore, postoperative complications postpone the chance of adjuvant chemotherapy resulting in the propagation of residual cancer cells. In this sense, neoadjuvant supportive treatment might be the best way to control microscopic cancer cells both around the main tumor and in distant organ, which are hard to eradicate by surgery alone. Neoadjuvant chemoradiation plus liver transplantation provided promising outcome for patients with perihilar cholangiocarcinoma .
R0 resection is the most effective treatment for hilar cholangiocarcinoma. However, we encounter the patients who resulted in receiving R1 surgery in spite of extended surgical procedure due to the limitation of accuracy in diagnosis. Concomitant treatment for ECC patients with R1 surgery has not been established. Local relapse after R1 surgery was controlled by chemoradiation for long term in the current case. Since survival rate of R1 surgery is worse than R0 surgery in ECC , patients with borderline resectable ECC, who are potentially candidates for R1 surgery, need optimal multimodal treatment in addition to the planned surgery.
Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images.
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