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Objectives: Systematic regional lymph node dissection (LND) combined with radical nephroureterectomy (RNU) has been reported to improve the outcomes of upper tract urothelial carcinoma (UTUC) patients. The objective of the present study was to assess the prognostic outcomes of UTUC patients undergoing systematic regional LND combined with RNU. Patients and methods: We conducted a retrospective evaluation of prognostic outcomes of 68 consecutive UTUC patients treated with RUN and systematic regional LND. Results: The median durations of recurrence-free survival (RFS) and overall survival (OS) were 45 and 166 months, respectively. Univariate analyses of several parameters showed that despite the lack of a significant predictor for RFS, the presence of lymph node metastasis correlated with poor OS. Conclusion: These results suggest that comparatively favorable outcomes are achievable in UTUC patients by RNU and systematic regional LND; however, special attention is needed for patients with nodal involvement even after systematic regional LND due to the adverse impact of this factor on OS.

BackgroundThe safety and benefit of sentinel lymph node biopsy (SLNB) compared with regional lymph node dissection (RLND) and no lymph nodes removed (NA) in patients with vulvar squamous cell cancer (VSCC) was not well studied.MethodsA retrospective analysis on VSCC patients without distant metastasis and adjacent organ invasion from the Surveillance, Epidemiology, and End Results Program database between 2004 and 2016 was carried out. Within subgroups stratified by negative (LN−) or positive (LN+) regional lymph node findings, inverse probability weighting (IPW) adjusted multivariate Fine-Gray compete risk (CR) model and accelerated failure time (AFT) model was used to investigate the factors associated with and cancer-specific survival (CSS) and overall survival (OS).ResultsOf the 3,161 VSCC patients treated with surgery, 287 (9.1%) underwent SLNB, 1,716 (54.3%) underwent RLND, and 1,158 (36.6%) had no regional lymph nodes removed. As illustrated by IPW adjusted multivariate regressions, SLNB was significantly associated with prolonged CSS (LN−, adjusted sub-proportional hazard ratio [sHR] = 0.42; 95% confidence interval [CI], 0.19–0.93; P=0.032; LN+, adjusted sHR = 0.29; 95% CI, 0.16–0.54, P<0.001) and OS (LN−, adjusted time ratio [TR] = 1.38; 95% CI, 0.82–2.32; P=0.226; LN+, adjusted TR = 2.68; 95% CI, 1.73–4.14; P<0.001), although the effect of SLNB on OS was not significant within the LN− cohort. Moreover, SLNB led to improved CSS (adjusted sHR = 0.40; 95% CI, 0.23–0.70; P = 0.001) and OS (adjusted TR=1.15, 95% CI 0.76-1.73, P=0.279) compared with NA. Age was a significant prognostic factor of CSS and OS, whereas tumor size, surgery type, and invasion depth were not.ConclusionsSLNB leads to significantly prolonged CSS and OS in VSCC surgery patients without distant metastasis and adjacent organ invasion than RLND, except for the similar OS in the LN− cohort. SLNB could be carried out preferentially for VSCC surgery patients without distant metastasis and adjacent organ invasion, irrespective of tumor size, surgery type, invasion depth, and regional lymph nodes metastasis. Further prospective clinical trials are warranted to confirm the findings of this study.

Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor associated with aggressive clinical behavior, metastasis and low survival. To date, only 67 cases have been described in the English language literature, and an understanding of the behavior of CCOC has been based on limited case reports. The aim of the research was to further reveal the features of CCOC. We report 5 new cases of CCOC, with a mean age of 52.4 years. The clinical and histopathologic data of the disease obtained from earlier literature (95 cases) and the 5 new cases were analyzed. Data were extracted, including demographics, histopathologic findings, clinical presentation, primary treatment and outcomes. Immunohistochemical results revealed that the cancer is positive for AE1/AE3, EMA and CK19, negative for smooth muscle actin SMA, Vim and S-100. EWSR1 translocation was also observed in the new cases, which may help in the diagnosis of CCOC. Metastases of CCOC were rare, but the local recurrence rate of CCOC rose to 42%. The best treatment for patients with CCOC is wide local excision combined with regional lymph node dissection.

Organ-preserving surgery (OPS) has been accepted for pancreatic neuroendocrine neoplasms, particularly for the management of small tumors. This study aimed to analyze the surgical outcome following this treatment at our institute, focusing on the perioperative factors and postoperative locoregional recurrence.We analyzed 71 consecutive patients with no synchronous liver metastasis. These patients were classified into 1 of 2 groups: the standard operation group (SOG, 41 patients) with prophylactic regional lymph node dissection and the organ-preserving surgery group (OPG, 30 patients). We performed OPS based on size criteria (tumor size1.5 cm in nonfunctional pancreatic neuroendocrine neoplasms and2 cm in insulinoma with no evident bulky lymph node swelling on preoperative imaging).The median follow-up periods were 37 months. The OPG included enucleation, partial resection, proximal parenchymal pancreatectomy, central pancreatectomy, spleen-preserving distal pancreatectomy, and Warshaw operation. The SOG included pancreatoduodenectomy and distal pancreatectomy, showing no statistically significant differences between the 2 groups in terms of operation time, hospitalization duration, and postoperative complications. Ten patients showed lymph node metastasis (25%) only in the SOG. There were no locoregional recurrent cases in the OPG.In selected patients, OPS may be effective based on the appropriate tumor size criteria.

This study aimed to explore the association of tumor sidedness with the prognosis of patients with colon signet ring cell carcinoma (SRCC). Eligible patients were retrieved from the Surveillance, Epidemiology, and End Results database between 2004 and 2015. Cancer-specific survival (CSS) and overall survival (OS) were compared between patients with left-sided colon SRCC and those with right-sided lesions. A total of 2660 patients were included, among them, 1983 (74.5%) had right-sided colon SRCC. Compared to patients with left-sided colon SRCC, those who had the right-sided colon SRCC showed higher proportion of white race, female, aged ≥ 65 years, receiving total colectomy and ≥ 4 regional lymph node dissection; while had lower proportion of advanced AJCC stage. Besides, right-sided patients exhibited superior 5-year CSS (32.74% vs. 25.89%, P = 0.001) and OS (27.38% vs. 23.02%, P = 0.024) rates compared with left-sided ones. Multivariate analysis revealed that tumor sidedness was an independent prognostic factor. To be specific, patients with right-sided colon SRCC showed better CSS (HR: 0.873; 95% CI 0.777–0.981; P = 0.023) and OS (HR: 0.838; 95% CI 0.753–0.965; P = 0.002). Moreover, subgroup analysis demonstrated superior CSS and OS for right-sided patients in most subgroups. Tumor sidedness was an independent prognostic indicator for colon SRCC. Besides, patients with right-sided colon SRCC have superior prognosis than those with left-sided lesions.

Colorectal cancer metastasis to the retroperitoneum, especially solitary metastasis allowing curative resection, is rare. We report a case of complete resection of retroperitoneal metachronous solitary metastasis from caecal cancer without distant metastasis. An 80-year-old woman with caecal cancer underwent laparoscopic ileocaecal resection with regional lymph node dissection. According to the eighth edition of the TNM classification, the pathological diagnosis was stage IIA (T3N0M0). Six months following the surgery, computed tomography revealed a solitary mass of 2cm diameter, dorsal to the right kidney. A second procedure for the removal of the tumour was performed. The lesion was pathologically diagnosed as a metachronous solitary retroperitoneal metastasis from caecal cancer. The patient is surviving and free from recurrence 17 months following the second procedure.

Gastrectomy, which means removal of the stomach, has currently been performed mostly for curative treatment of stomach cancer in combination with regional lymph node dissection. After the removal, digestive tract is reconnected using esophagojejunostomy, gastroduodenostomy, gastrojejunostomy, or esophagogastrostomy. Some complications take place early or late after gastrectomy in association with the extent of stomach and lymph node dissection. Minimally invasive approach improves the patient's recovery and quality of life.

Curative-intent resection is the only treatment of perihilar cholangiocarcinoma (pCCA) offering patients the chance for long-term survival. Surgery generally requires an (extended) hemi-hepatectomy including the caudate lobe with resection of the extrahepatic bile duct and a regional lymph node dissection. pCCA is considered resectable when negative surgical margins can be obtained while safeguarding adequate vascular inflow to the future liver remnant.

Radical cystectomy and regional lymph node dissection is the standard treatment for localized muscle-invasive and for high-risk non-muscle-invasive bladder cancer, and represents one of the main surgical urologic procedures. The open surgical approach is still widely adopted, even if in the last two decades efforts have been made in order to evaluate if minimally invasive procedures, either laparoscopic or robot-assisted, might show a benefit compared to the standard technique. Open radical cystectomy is associated with a high complication rate, but data from the laparoscopic and robotic surgical series failed to demonstrate a clear reduction in post-operative complication rates compared to the open surgical series. Laparoscopic and robotic radical cystectomy show a reduction in blood loss, in-hospital stay and transfusion rates but a longer operative time, while open radical cystectomy is typically associated with a shorter operative time but with a longer in-hospital admission and possibly a higher rate of high grade complications.

Rectal neuroendocrine tumor (NET) is a relatively rare lesion of the gastrointestinal tract, but the prospective examination with colonofiberoscopy or endoscopic ultrasound has increased the frequency of its detection. It is often difficult to determine the optimal treatment for NETs sized 1 cm carry a risk for metastasis equivalent to that of adenocarcinomas. Therefore, it is mandatory to histologically examine lymphovascular invasion in specimens retrieved via endoscopic resection to determine the necessity for further radical surgery with regional lymph node dissection. The treatment of NETs sized

Introduction/background Regional lymph node dissection (LND) may provide oncologic benefit among patients with clinical lymphadenopathy in renal cell carcinoma (RCC) at the of time radical nephrectomy (RN). Yet, little is known about contemporary use of LND for patients with RCC and regional lymphadenopathy. Thus, we assessed national trends in RN and LND among RCC patients with renal masses and clinical lymphadenopathy. Materials and methods From the National Cancer Data Base (NCDB), we identified patients with RCC and clinical lymphadenopathy without distant metastasis (clinical T1-4 N1 + M0) who underwent RN from 2001 to 2011. The primary outcome was concomitant LND at the time of RN. Multivariable logistic regression analysis was used to identify patient and hospital characteristics associated with the primary outcome. Results Among 1840 patients with clinical N1+ treated surgically, 78% received LND (n=1444). The proportion of patients who underwent LND increased from 77% in 2001 to 82% in 2011 (p Conclusion Among patients with RCC and clinical lymphadenopathy, ~20% are not receiving LND at the time of RN in the U.S. Academic hospitals and open surgery was associated with receipt of LND. Centralization to tertiary academic hospitals may facilitate greater use of LND.

Objective Unlike for bladder cancer, the impact of regional lymph node dissection for upper tract urothelial carcinoma is unclear. We explored whether patient survival was influenced by systematic regional lymph node dissection, using resection templates according to the main tumor location, during radical nephroureterectomy for upper tract urothelial carcinoma. Methods The systematic regional lymph node dissection group was defined as cases in which the dissection of nodes and surrounding tissues followed the established template, and the non-systematic regional lymph node dissection group as cases undergoing limited or no lymph node dissection. We performed radical nephroureterectomy on 98 consecutive patients with various stages of upper tract urothelial carcinoma from May 1994 to September 2014 at our institution. Of these, 77 patients with cTanyN0M0 of upper tract urothelial carcinoma undergoing radical nephroureterectomy were grouped into systematic regional lymph node dissection or non-systematic regional lymph node dissection cohorts according to the extent of dissection, and their outcomes compared. Results Forty-four patients were categorized as systematic regional lymph node dissection and 33 as non-systematic regional lymph node dissection, including 17 with more limited nodal dissection and 16 with no nodal dissection. Five-year recurrence-free survival and cancer-specific survival were significantly higher in the systematic regional lymph node dissection (93% and 94%, respectively) than in the non-systematic regional lymph node dissection group (75% and 77% recurrence-free survival and cancer-specific survival, respectively). Further, 5-year recurrence-free survival and cancer-specific survival of muscle-invasive upper tract urothelial carcinoma (pT2-4) were significantly higher in the systematic regional lymph node dissection (87% and 91%, respectively) than in the non-systematic regional lymph node dissection group (59% and 62%, respectively) (P = 0.0237 and P = 0.0224). Neither recurrence-free survival nor cancer-specific survival was significantly prolonged by systematic dissection in patients with pTis-1 histology. Conclusions Systematic regional lymph node dissection during radical nephroureterectomy for cTanyN0M0 upper tract urothelial carcinoma patients has a significantly beneficial impact on survival compared with patients undergoing more limited dissection, especially in the cases involving muscle invasion.

Objective: Nodal involvement is important for the prognosis in skin cancers. In the absence of palpable lymph nodes or when the nodal involvement is suspicious, sentinel lymph node biopsy (SLNB) is the preferred method instead of agrressive regional lymph node dissection. In our study we aimed to share our experience regarding SLNB in malignant skin tumors. Material and Method: From August 2009 to August 2015, 30 patients (17 male, 13 female) aged between 21-81, who underwent SLNB, were analysed retrospectively. Results: Thirty-three SLNBs were performed in 30 patients. In all procedures sentinel lymph node was identified and excised successfully. Eight positive sentinel lymph nodes were obtained. Four patients, who had negative SLNB previously, developed symptoms on behalf of metastasis on follow-up, and underwent therapeutic lymph node dissection. Conclusion: Patients, who have no clinical evidence of nodal involvement but suspicion of nodal status, should undergo SLNB procedure, which has a low morbidity and complication rate.

In 1960, Cahan reported the first patients to successfully undergo lobectomy with regional lymph node dissection, which was called radical lobectomy (1). Since then, this procedure has been broadly accepted and has remained as a standard surgery for non-small cell lung cancer (NSCLC). Systematic nodal dissection (SND) has been reported to be important for survival as well as accurate disease staging (2,3). However, the effect of SND on the prognosis remains controversial.

Laparoscopic gastrectomy is a treatment for gastric cancer, and isoperistaltic side-to-side reconstruction is called “overlap anastomosis.” The physiological advantages of preserving the autonomic nerves in the jejunal limb for digestive reconstruction are well known. Here, we focused on overlap anastomosis with autonomic nerve-preserved mesojejunum of the lifted jejunal limb for laparoscopic distal gastrectomy with intentional lymph node dissection. Our surgical techniques and technical pitfalls were described in detail. The jejunum was partially sacrificed to preserve the autonomic nerves in the lifted jejunal limb. The length of the staple line was 35 – 40 mm. The endostapler entry was carefully closed to avoid even subtle stenosis. Twelve patients were retrospectively evaluated with a follow-up of 5.0 ± 0.6 years. Histological findings according to the Japanese classification were stage IA or IB. Dietary intake and postoperative ambulation occurred at 3.3 ± 1.0 and 1.3 ± 0.5 days after surgery, respectively. Postoperative complications according to Clavien–Dindo classification were one each of grade I and grade II. Postoperative hospital stay was 6.7 ± 1.6 days. Five patients were medication-free at final follow-up, with no recurrence in any patient. Overlap anastomosis with autonomic nerve-preserved jejunal limb was safe and feasible for laparoscopic distal gastrectomy with lymph node dissection.

Enhanced recovery after surgery (ERAS) protocols aim to improve patient care, reduce complication rates and shorten hospital stay following surgery. The key elements of these protocols include preoperative counselling, standardized analgesia and early mobilization. We present our experience in applying ERAS pathway for skin cancer patients requiring regional lymph node dissection (LND). Our protocol streamlines from the initial clinic consultation and perioperative management to safe discharge and regular clinic follow-up. Prospective data collection of 100 consecutive patients who underwent LND, performed by the senior author, was done. Data included patient demographics, initial diagnosis, initial clinic appointment, surgery date, discharge date, number of follow-up clinics and complications. Our results were compared to the results of an audit of 50 consecutive patients managed in our department prior to the introduction of the presented protocol. Our ERAS pathway resulted in a shorter hospital stay and fewer complication rates. Our patients were discharged, with drains in situ. Our mean length of stay (LOS) for axillary, inguinal and neck LND was 1.18, 2 and 4.1 days, respectively. The overall LOS was 2.4 versus 9.5 days in the audited previous practice. Our complication rate was 50 % less (P = 0.002). This also resulted in substantial financial savings. ERAS protocol for skin cancer patients requiring LND is based on multi-disciplinary team (MDT) approach. It has proven to be an efficient and safe streamlined pathway from consultation to discharge, with shorter hospital stay and satisfactory clinical outcomes as well as substantial financial savings that could be recruited in other services. Level of Evidence: Level III, risk/prognostic study.

Surgery remains the curative treatment of choice for colorectal cancer (CRC). However, to our knowledge, no report has addressed the usefulness of additional regional lymph node dissection for primary CRC that has invaded another colon region. We reviewed the clinicopathological characteristics and outcomes of eight patients who underwent surgery between March, 2005 and August, 2014, for CRC that invaded another region of the colon. Five patients underwent additional regional lymph node dissection in the area of the invaded colon and one patient had lymph node metastasis in the region. Two of three patients who did not undergo additional regional lymph node dissection were found to have regional lymph node recurrences in the area during the follow-up period. Although there was no statistical correlation between extra-regional lymph node metastasis and clinicopathological or operative factors, the patients with extra-regional lymph node metastasis or recurrence had primary regional lymph node metastasis. For curative intent, surgeons may need to perform additional regional lymph node dissection for primary CRC invading another colon region.

BACKGROUND: Surgery (R0 resection) is the mainstay of treatment of gallbladder cancer (GBC) as GBC is relatively resistant to currently known chemotherapy and radiotherapy regimens. AIM: to assess if wedge resection of the gallbladder bed achieves an adequate oncological clearance in GBC (namely T1 and T2) and some T3 GBC with minimal liver infiltration. PATIENTS AND METHODS: Patients with GBC who underwent radical cholecystectomy (en bloc cholecystectomy, wedge resection of the gallbladder fossa with a ≥2 cm rim of nonneoplastic liver tissue, and regional lymph node dissection) between October 2012 and June 2015 after obtaining informed consent. RESULTS: Of thirty patients, mean age of 52 years, 5 had T1b, 13 T2, and 12 T3 GBC. R0 resection was achieved in all thirty GBC patients. Hepatic invasion was found in seven patients. The depth of hepatic invasion ranged from 0 to 9 mm. Follow-up ranged from a minimum of 12 to 43 months. Nineteen (63%) patients had N0 and 11 (37%) had N1 GBC. Total lymph node (TLND) count ranged from 1 to 12/patient with a median of 3. There was no local recurrence or systemic relapse of the disease. CONCLUSION: Wedge resection of the gallbladder bed achieves an adequate oncological clearance in early GBC. TLND counts remain poor even after a thorough standard lymph node dissection for resectable GBC.

We herein present a case of a 59-year-old man who had undergone pylorus preserving pancreaticoduodenectomy with regional lymph node dissection prior to episodes of melena. Series of conventional endoscopic investigations failed to identify the bleeding source. Enhanced computed tomography scan revealed complete obstruction of the main portal vein with numerous collateral veins running towards the hepatic hilus. Comprehensively, hemorrhage from the jejunal varices caused by postoperative portal hypertension was highly suspected. As the jejunal loop was out of reach, adult variable-stiffness colonoscope (AVSC) was utilized to solve the Roux-en-Y anatomy. Numerous telangiectasis and small varices at hepaticojejunostomy were observed and in the mean time, bleeding was noticed and endoclips were placed without any delay. Ectopic variceal bleeding in jejunal loop after pancreaticoduodenectomy is difficult to manage. We believe that AVSC is an alternative device when specialized jejunal endoscopy is not available.

512 Background: Advances in prognostic allocation of pts with PSCC receiving regional LND are needed, along with an assessment of the role of perioperative treatments. Methods: An international, multicenter, retrospective database was queried for all pts with PSCC who had received inguinal ± pelvic LND from 1980 to 2017. We classified or re-classified pts according to the 2009 TNM classification system. The primary endpoint was OS. Survival analysis methods were adopted for the purpose of detecting and modeling putative prognostic factors, and develop a prognostic stratification tool. The analysis was performed in two steps: first, we modeled outcome data and covariates by resorting to a random forest (RF) method (an “ensemble” machine-learning approach, with an ad hoc approach incorporating missing data imputation). Second, data was modeled using Cox proportional hazard regression. In addition, a Cox model was fit including pre-specified variables that were deemed to be clinically relevant. Results: There were 743 pts who received LND at seven referral centers from Europe, the USA, Brazil, and China. Of these pts, 689 were analyzed: 86 (12.5%) received neoadjuvant chemotherapy (NAC); 171 (24.8%) received adjuvant chemotherapy (AC), and 74 (10.7%) received adjuvant radiotherapy. The variables significantly associated with OS were age (p < 0.001), the pathologically involved/total removed LN ratio (p < 0.001), pN stage (overall p < 0.001) and NAC (HR: 1.58, 95%CI: 1.10-2.26, p = 0.013). NAC and AC were ineffective or detrimental in N1-2 patients, whereas they provided OS improvements in N3 patients. OS improvement reached statistical significance for AC in pelvic LN-positive pts (p = 0.046). Finally, we developed a nomogram predicting 12- and 24-month OS based on pre-specified variables (c-index: 0.75). The study is limited by its retrospective nature. Conclusions: We propose a post-treatment prognostic tool that can be offered as an aid to physicians to enhance decision-making, clinical research, and patient counseling after LND in PSCC. Administration of NAC and AC should be restricted to N3 patients.

The standardization of colon cancer surgery has been an area of intense interest. The recent establishment of the complete mesocolic excision (CME) technique has defined the operative approach for colon cancer surgeries and enabled the collection of high-quality oncological specimens for histopathological evaluation. Standard for the Diagnosis and Treatment of Colorectal Cancer (2010), issued by the Ministry of Health of China, has provided legal bases for the treatment of colorectal cancers. However, certain confusions remain due to lack of detailed guidelines for operations. This raised the key question: “What is the standardized colon cancer surgery?” The present study re-examined the core ideas of General Rules for Clinical and Pathological Studies on Cancer of the Colon, Rectum and Anus (seventh edition) published by the Japanese Society for Cancer of the Colon and Rectum. CME-related studies published in English academic journals between April 2009 and July 2012 were surveyed and analysed. Several technical issues related to the requirement of R0 resection were analysed, including the theoretical basis for the safety range of bowel resection and the rational determination of the range of regional lymph node dissection.

We report herein a case of adenocarcinoma arising from short-segment Barrett's esophagus (SSBE) in a 36-year-old man. An elevated tumor was found at the esophagogastric junction, and a histological evaluation of the biopsy specimen led to a diagnosis of adenocarcinoma. The tumor was found to be confined to the mucosa surrounding the SSBE, and endoscopic submucosal dissection was done without complications. Histological examination of the resected specimen showed that the adenocarcinoma had also invaded the muscularis mucosae and provided evidence of lymphovascular invasion. Additional surgical resection and regional lymph node dissection were therefore carried out; however, no lymph node metastasis was found. Adenocarcinoma arising from Barrett's mucosa is rare in young patients, especially in Japan, and this case is therefore particularly noteworthy.

Aim: Laparoscopy-assisted distal gastrectomy (LADG) with regional lymph node dissection is a treatment option for patient with early gastric cancer. However, LADG is a technically complex and advanced procedure, which is challenging for inexperienced surgeons. In this report, we retrospectively evaluated the learning curve for LADG of a single surgeon with no previous experience in LADG and the usefulness of direct instruction by a surgeon experienced in LADG in shortening the learning curve. Patients and Methods: This study was analyzed 80 consecutive patients, who underwent LADG by a single surgeon (first assistant in 10 cases and operator in 70 cases) between January 2008 and December 2012. Patients were divided into 3 sequential groups of 10 (training period), 30 (learning period), and 40 (operating period) cases in each group. Median operation time and estimated blood loss for these 3 groups were determined. Other learning indicators, including transfusion requirement, postoperative complications, number of lymph node harvested, and rate of conversion open gastrectomy, were also evaluated. Results: During the training period, median operation time and estimated blood loss were 219.5 min and 83.0 ml, respectively. During the learning period, the operation time was significantly longer than that of training period. In the operating period, the operation time was significantly lesser than that during the learning period. However, the operation time was not different from that during the training period and reached a plateau. The estimated blood loss during the operating period was significantly lesser than that during the learning period. The difference in the number of lymph nodes retrieved between each group was not significant. Conclusions: Direct instructions by an experienced surgeon can decrease the number of cases required for learning. Because LADG is technically more complex than other laparoscopic procedures, standardization of LADG and an effective training system for performing it should be established.

Background The most common cause of perihilar obstruction is cholangiocarcinoma, especially in Thailand. Benign perihilar stricture represents less than 20% of cases. IgG4-related disease and IgG4-related sclerosing cholangitis, however, have been receiving increased recognition. Isolated IgG4-related sclerosing cholangitis is less common. The preoperative diagnosis of IgG4-related sclerosing cholangitis without pancreatic involvement is very difficult because the clinical presentation and preoperative evaluation are extremely difficult to distinguish from perihilar cholangiocarcinoma. Case presentation We report the case of a 56-year-old man who presented with obstructive jaundice with preoperative imaging showing proximal common bile duct obstruction. He underwent right lobe liver hepatectomy with extrahepatic bile duct resection and regional lymph node dissection due to high suspicion of malignancy. The pathological report showed severe acute and chronic inflammation of the bile duct with morphology and immunohistochemistry suggestive of IgG4-related sclerosing cholangitis. Conclusions IgG4-related sclerosing cholangitis with perihilar obstruction should be considered even in areas where cholangiocarcinoma is endemic.