Published online Oct 27, 2026. doi: 10.4240/wjgs.123695
Revised: July 15, 2026
Accepted: September 4, 2026
Published online: October 27, 2026
Processing time: 146 Days and 4.8 Hours
Intraductal papillary neoplasia of the bile (IPNB) duct is a rare precancerous lesion characterized by intraductal papillary proliferation and mucin secretion. Radical resection–the standard treatment-is precluded by inadequate residual liver volume. Non-radically resectable IPNB typically presents with more severe clinical symptoms and a worse prognosis. The application of debulking surgery-involving hepatic lobectomy-in these patients remains relatively unexplored.
We present three IPNB cases with both left and right intrahepatic bile duct involvement. Herein, radical resection was predicted to spare inadequate residual liver volume; thus, debulking surgery with 24-French T-tube drainage was per
Debulking surgery with T-tube drainage should be considered for non-radically resectable IPNB. In patients with residual tumors, maintaining biliary drainage patency and continued hepatoprotective therapy and antimicrobial therapy are essential to prolong survival and improve quality of life.
Core Tip: Patients with non-radically resectable intraductal papillary neoplasm of the bile (IPNB) duct usually have more severe clinical symptoms (abdominal pain, jaundice, and recurrent cholangitis), a higher risk of malignant transformation, and a worse prognosis. Therefore, proactive interventions are necessary to relieve clinical symptoms and prolong survival. Herein, we describe three patients with type I IPNB who underwent debulking surgery (left lateral segment resection, two patients; left hepatectomy, one patient). The surgery reduced tumor burden and alleviated symptoms in all patients. The findings emphasize the importance of comprehensive management of patients with IPNB for raising clinicians’ awareness and improving patient outcomes.
- Citation: Yang YL, Zhang TQ, Le WJ, Zhou L, Cheng H, Li YJ, Zhang KM, Yang ZC, Peng T, Lu HF. Debulking surgery for non-radically resectable intraductal papillary neoplasm of the bile duct: Three case reports and literature review. World J Gastrointest Surg 2026; 18(10): 123695
- URL: https://www.wjgnet.com/1948-9366/full/v18/i10/123695.htm
- DOI: https://dx.doi.org/10.4240/wjgs.123695
Intraductal papillary neoplasm of the bile (IPNB) duct is a rare bile duct tumor characterized by intraductal papillary proliferation and mucin secretion. First reported in 2001, IPNB was included in the World Health Organization (WHO) tumor classification in 2010 and defined as a precancerous lesion in the 2019 WHO classification[1-3]. The pathogenesis of IPNB remains unclear. Major risk factors for IPNB include bile duct stones, hepatic schistosomiasis infection, and primary sclerosing cholangitis (all prevalent in East Asia)[1-3]. The median age at onset in patients with IPNB is 60-66 years, and males are predominantly affected[4,5]. IPNB accounts for approximately 5%-15% of all bile duct tumors. Radical resection is the standard treatment for IPNB and offers a better prognosis than it does for cholangiocarcinoma[6]. IPNB progression is characterized by unique biological behavior-its insidious onset, multifocal distribution, and superficial spread along the bile duct walls[6-8]. Patients are asymptomatic or present with abdominal pain (35%-88.5%), recurrent acute cholangitis (5%-59%), and obstructive jaundice (20%-36%)[3]. These clinical symptoms are nonspecific, often leading to missed or delayed diagnosis of IPNB[9]. Consequently, in some patients, the tumors have already extensively involved the intra
To our knowledge, a systematic description of debulking surgery for IPNB, including patient selection criteria, volumetric considerations, and long-term management protocols, has not been previously reported in the literature. We systematically searched PubMed, EMBASE, Web of Science, and Cochrane Library up to April 1, 2026, using the keywords “IPNB” AND “debulking surgery”. Our search identified no prior reports of debulking hepatectomy for unresectable IPNB. The present case series represents the first dedicated report to systematically describe and analyze debulking surgery as a surgical strategy for unresectable IPNB.
Case 1: A 64-year-old man was admitted in February 2017 with a chief complaint of upper abdominal pain accompanied by jaundice of the skin and scleral icterus lasting for 10 days.
Case 2: A 69-year-old woman was admitted in July 2023 with a chief complaint of scleral icterus lasting for 12 days.
Case 3: A 76-year-old woman was admitted in September 2025 for dilated intrahepatic and extrahepatic bile ducts since 1 year and scleral icterus since one week (Table 1).
| Variable | Case 1 | Case 2 | Case 3 |
| Age/sex | 64/male | 69/female | 76/female |
| Chief complaint | Abdominal pain, jaundice | Jaundice | Incidental biliary dilatation |
| Relevant history | Schistosomiasis, ERCP | Schistosomiasis, cholecystectomy, CBDE | Cholecystectomy, CBDE |
| Pre-op TBIL (μmol/L) | 207.1 | 41.8 (peaked 167.3) | 41.1 |
| CA19-9 (U/mL) | 126.5 | Normal | > 1000 |
| Pre-op biliary drainage | ENBD, PTCD (left) | PTCD (bilateral) | None |
| Dominant lobe involved | Left lateral, right posterior | Left lateral, right posterior | Left lobe |
| Debulking procedure | Open left lateral sectionectomy | Lap. left lateral sectionectomy | Lap left hepatectomy |
| SRLVR of curative plan | N/A | 29.5% | 33.4% |
| SRLVR of post-debulking | N/A | 72.5% | 90.5% |
| Pathologic diagnosis | Type 1 IPNB, margin+ | Type 1 IPNB with HGD, margin+ | IPNB with invasive carcinoma |
| Follow-up duration | 7 years | 2 years | 7 months (alive) |
| Outcome | Died from liver failure | Died from biliary hemorrhage | Alive with residual tumor |
Case 1: He had upper abdominal pain since 10 days, accompanied by jaundice of the skin and scleral icterus, without fever.
Case 2: She developed scleral icterus 12 days ago, without fever or abdominal pain.
Case 3: She had a 1-year history of dilation of intrahepatic and extrahepatic bile ducts without abdominal pain or fever. She had scleral icterus since 1 week.
Case 1: He had a history of schistosomiasis. He underwent ERCP for common bile duct stones 5 years ago.
Case 2: She had a history of schistosomiasis and hypertension. She underwent an open cholecystectomy 15 years ago and an open common bile duct exploration with lithotripsy 10 years ago.
Case 3: She received a laparoscopic cholecystectomy and common bile duct exploration 10 years ago.
Case 1: He had a 40-year history of smoking and alcohol consumption.
Case 2 and case 3: No significant personal or family history was reported.
Case 1: His vital signs were as follows: Consciousness, clear; blood pressure 130/82 mmHg; heart rate 81 beats/minute; body temperature 36.4 °C; and respiratory rate 20 breaths/minute. He had jaundice of the skin, scleral icterus, upper abdominal tenderness, and no rebound tenderness.
Case 2: She was 145 cm tall and weighed 53.5 kg, with a body mass index (BMI) of 25.4 kg/m2 and no recent significant weight loss. Her vital signs were as follows: Consciousness, clear; blood pressure 155/89 mmHg; heart rate 60 beats/minute; body temperature 36.5 °C; and respiratory rate 19 breaths/minute. She had scleral icterus but no abdominal tenderness or rebound tenderness.
Case 3: She was 147 cm tall and weighed 41.7 kg, with a BMI of 19.3 kg/m2 and no recent weight loss. Her vital signs were as follows: Consciousness, clear; blood pressure 131/71 mmHg; heart rate 88 beats/minute; body temperature
Case 1: His total bilirubin (TBIL) level was elevated to 207.1 μmol/L (reference range: 0-21 μmol/L), and the direct bilirubin (DBIL) level was elevated to 172.5 μmol/L (reference range: 0-4 μmol/L). Alanine transaminase (ALT) level was elevated to 160 U/L (reference range: 7-40 U/L). Aspartate transaminase (AST) level was elevated to 81 U/L (reference range: 13-35 U/L), γ-glutamyl transferase (GGT) was elevated to 1250 U/L (reference range: 7-45 U/L) and carbohydrate antigen (CA19-9) level was elevated to 126.50 U/mL (reference range: 0-28 U/mL).
Case 2: Her TBIL level was elevated to 41.8 μmol/L. DBIL level was elevated to 22.9 μmol/L. AST level was elevated to 48 U/L, and GGT was elevated to 447 U/L. Hemoglobin was decreased to 108.00 g/L (reference range: 115-150 g/L), and CA19-9 was normal.
Case 3: Her hemoglobin level was 116.00 g/L. TBIL level was elevated to 41.1 μmol/L. DBIL level was elevated to 19.7 μmol/L, and indirect bilirubin level was elevated to 21.4 μmol/L. ALT level was elevated to 31 U/L. AST level was elevated to 42 U/L. GGT was elevated to 1173 U/L. Carcinoembryonic antigen (CEA) level was elevated to 14.91 ng/mL (reference range: 0–5.09 U/mL). CA19-9 level exceeded 1000 U/mL.
Case 1: Abdominal contrast-enhanced computed tomography (CT) (Figure 1) showed schistosomiasis-related cirrhosis, dilation of intrahepatic and extrahepatic bile ducts, and atrophy of the left and right liver lobes. Papillary lesions of varying sizes were found within the dilated bile ducts of the left and right posterior liver lobes. The papillary lesions showed mild enhancement in the arterial phase (Figure 1A) and slightly lower density in the venous (Figure 1B) and delayed phases (Figure 1C).
Case 2: Contrast-enhanced CT revealed schistosomiasis-related cirrhosis, biliary dilatation, and papillary lesions in the left and right posterior lobes. The lesions showed mild arterial phase enhancement (Figure 1E) and slightly low density (Figure 1F and G). Contrast-enhanced magnetic resonance imaging (MRI) confirmed the presence of papillary lesions in the left and right posterior hepatic bile ducts (Figure 1D and H-K). The lesions were hypointense on T1-weighted images (Figure 1H) and mildly hyperintense on T2-weighted images, with a signal intensity higher than that of the hepatic parenchyma (Figure 1D). They showed mild arterial phase enhancement (Figure 1H), low signal intensity in the venous phase (Figure 1I), and mild hyperintensity on diffusion-weighted images (Figure 1J). Preoperative 3D reconstruction showed a total liver volume of 1632.1 mL, with segmental volumes as follows: S1: 30.2 mL, S2: 462.1 mL, S3: 430.9 mL, S4: 292.6 mL, S5: 162.5 mL, S6: 94.9 mL, S7: 50.8 mL, and S8: 108.1 mL (Figure 1K).
Case 3: Imaging revealed left lobe atrophy and papillary masses in the left hepatic bile ducts (Figure 1M–T). The papillary masses showed mild arterial phase enhancement (Figure 1M) and were hypodense in the venous (Figure 1N) and delayed phases (Figure 1P). Magnetic resonance cholangiopancreatography (MRCP) confirmed the dilatation of the intrahepatic and extrahepatic bile ducts (Figure 1T). T2-weighted imaging revealed multiple papillary masses within the dilated bile ducts of the left lobe (Figure 1Q). The filling mass within the bile ducts showed mild enhancement (Figure 1S) and hypodensity in the venous phase (Figure 1T). Preoperative 3D reconstruction showed a total liver volume of 1088.8 mL, with segmental volumes as follows: S1: 14.6 mL, S2: 6.9 mL, S3: 11.9 mL, S4: 218.1 mL, S5: 272.1 mL, S6: 104.7 mL, S7: 204.3 mL, and S8: 256.2 mL (Figure 2E).
ERCP and ENBD were performed on hospital day 5. Ultrasound-guided PTCD of the right lobe was performed in the third week because of progressive jaundice (TBIL: 470.9 μmol/L; DBIL: 355.8 μmol/L). The catheter was flushed daily, and yellow gelatinous mucus was drained. The preoperative diagnosis was IPNB.
Jaundice was progressive on day 7 of hospitalization (TBIL: 167.3 μmol/L; DBIL: 102.1 μmol/L). Bilateral ultrasound-guided PTCD was performed. The catheters were flushed daily, and yellow gelatinous mucus was collected. The preoperative diagnosis was IPNB.
According to the findings of contrast-enhanced CT and MRI, the preoperative diagnosis was IPNB of the left lobe. The malignant transformation was suspected because of the abnormally high CA19-9 levels.
Pathology confirmed type I IPNB (Figure 2). The tumor showed a papillary arrangement with a fibrovascular core (Figure 2B, hematoxylin and eosin × 100). The liver showed cirrhotic changes (Figure 2C). Tumor tissue was present at the resection margin (Figure 2D), along with chronic cholecystitis.
Pathology confirmed type I IPNB with high-grade dysplasia (Figure 2F). Microscopic examination revealed focal high-grade intraepithelial neoplasia at the bile duct resection margin (Figure 2G). Schistosomiasis-related cirrhotic changes were also noted (Figure 2H). Immunohistochemistry was as follows: EMA (partially positive), Muc-2 (partially positive), CDX-2 (focally positive), CK7 (positive), CK20 (partially positive), MUC-5AC (positive), P53 (scattered positive, wild-type), Ki67 approximately 40%, CD31 and ERG (vascular +), and P53 (scattered +, wild-type).
Postoperative pathology of the left hepatic lobe showed an intraductal papillary mucinous neoplasm, with a portion of the mass measuring 3.0 cm × 2.5 cm × 2.5 cm, accompanied by moderately to poorly differentiated adenocarcinoma with necrosis (Figure 2J). Nerve plexus invasion was observed; however, no evident intravascular tumor emboli were detected (Figure 2K). Immunohistochemistry was as follows: PCK (+), CK19 (+), hepatocyte (focal+), Glypican-3 (-), CK7 (+), MUC-5AC (+), EMA (+), CD56 (focal+), MUC-6 (focal+), MLH1 (+), MSH2 (+), MSH6 (+), PMS2 (partially weak+), Her-2 (1+), claudin 18.2 (approximately 30% moderate-to-strong+), P53 (heterogeneous+, suggesting wild-type), and Ki67 (approximately 30% in hotspots).
The liver showed schistosomiasis-related cirrhosis. Intraoperative cholangioscopy revealed large amounts of yellow mucin occupying the bile ducts and diffuse villous lesions lining the ductal walls in the left and right posterior lobes. The patient underwent debulking surgery (open resection of the left lateral lobe of the liver), and a 24F T-tube was placed in the common bile duct. Gross examination showed a white papillary mass in markedly dilated ducts filled with mucin (Figure 2A).
Laparoscopy revealed right lobe atrophy with compensatory enlargement of the left lateral lobe. Cholangioscopy showed that the lesions mainly involved the left lateral and right posterior lobes. The patient’s standard liver volume (SLV) was 1019.3 mL. Volumetric analysis calculated a standardized residual liver volume ratio (SRLVR) of 29.5% for radical left hemihepatectomy, which was considered to lead to liver failure. Thus, the patient underwent laparoscopic left lateral sectionectomy as a debulking procedure, with the placement of a 24F T-tube, achieving an SRLVR of 72.5%.
Laparoscopy revealed left lobe atrophy and evident dilatation of the common bile duct and left hepatic duct. Volumetric analysis predicted an SRLVR of 33.4% after radical resection (left hemihepatectomy and right anterior lobectomy) (Table 1), which was deemed insufficient. The patient underwent laparoscopic left hepatectomy as a debulking procedure with the placement of a 24F T-tube, preserving an SRLVR of 90.5%. Papillary lesions and mucinous material were observed at the bile duct stump (Figure 2I).
The T-tube was flushed every week postoperatively. Interval cholangioscopy revealed residual right-sided lesions, which were treated with cholangioscopic ablation therapy. Abdominal MRI and MRCP were performed in June 2019, revealing dilation of the intrahepatic and extrahepatic bile ducts on MRCP (Figure 3A). T2-weighted imaging revealed the T-tube located within the common bile duct (Figure 3B). Numerous papillary lesions were visible within the dilated bile ducts of the atrophic right posterior lobe (Figure 3C). The patient died in February 2024 due to liver failure and a biliary tract infection.
The patient was discharged on postoperative day 20 and was followed up regularly. By 1 year postoperatively, she had developed common bile duct stones. The stones were removed by choledochoscopy through the T-tube tract. Follow-up MRI on August 3, 2024, showed mild enhancement in the arterial phase (Figure 3D) with isointensity in the venous phase (Figure 3E). The patient refused to undergo cholangioscopic ablation. She later developed massive biliary hemorrhage due to residual papillary lesions (Figure 3F). Two years postoperatively, she died from massive hemorrhagic biliary hemorrhage and shock.
The patient took ursodeoxycholic acid capsules as directed in the package insert. The T-tube was flushed every week postoperatively. She underwent cholangioscopic ablation therapy 3 months postoperatively and remained alive at 7 months postoperatively.
IPNB is frequently diagnosed at an invasive stage with poor preoperative recognition[13]. Although the preoperative diagnosis of IPNB is challenging, imaging features, PTCD, cholangioscopy, and ERCP can make it feasible. The clinical symptoms of the three patients were nonspecific and had limited diagnostic value. According to existing research, contrast-enhanced abdominal CT and MRI show dilation of the intra- and extrahepatic bile ducts, with papillary lesions visible within the bile ducts. Although these lesions appear isointense or hyperintense in the late arterial phase, they do not appear hyperintense in the portal venous or delayed phases. The IPNB shown on contrast-enhanced MRI is similar to that seen on contrast-enhanced CT. The pattern of tumor enhancement, known as the “wash-in/wash-out” pattern, plays an important role in diagnosis[14,15]. The lesions show hypointense or isointense signals on T1-weighted MRI and hyperintense signals on T2-weighted MRI, with signal intensities higher than those of the liver parenchyma[16,17]. MRCP clearly visualizes biliary tract dilation. The advantages of ERCP include the ability to perform tumor biopsies and preoperative biliary decompression. The characteristic yellow mucus observed during ERCP or PTCD is crucial for the diagnosis of IPNB[18,19]. Percutaneous transesophageal cholangioscopy (POSC) permits direct visualization and targeted biopsies[6,20]. The use of POSC with narrow-band imaging (NBI) has further improved diagnostic accuracy to 93% and can change the preoperatively defined disease extent in 37% of patients[21,22]. POSC with NBI can also detect radiologically occult invasive carcinoma[23]. Pathologically, IPNB features papillary or villous arrangements with a fibro
The indications and principles for debulking surgery should be carefully evaluated. The indications for IPNB are as follows: (1) Patients unsuitable for radical resection: Patients with insufficient residual liver volume who refuse liver transplantation, patients with extrahepatic metastases or vascular invasion that preclude radical resection, and patients in poor general condition who cannot tolerate radical resection and (2) Symptom control: PTCD and ERCP are ineffective for obstructive jaundice and cholangitis, with the patient exhibiting worsening symptoms. The principles of debulking surgery were as follows: (1) The volume of each hepatic segment should be estimated by preoperative 3D reconstruction; ideally, the effective liver volume is calculated, excluding the dilated bile ducts[30,31]. In the present study, patient 1 refused 3D reconstruction, making preoperative evaluation difficult; (2) Liver function should be evaluated using the Child-Pugh classification or indocyanine green clearance test[32]. Patients with Child-Pugh class C liver function should be given liver-protective therapy. In this study, marked obstructive jaundice was preoperatively noted in both cases 1 and 2, classifying liver function as Grade C, and the patients underwent treatment to alleviate jaundice[33]. The liver function of three patients eventually reached Child–Pugh class B; (3) The extent of tumor involvement should be confirmed using preoperative MRI and MRCP and intraoperative cholangiography; the residual liver volume and residual liver ratio should be calculated after radical resection; and it should be confirmed that IPNB is not amenable to radical resection[34]; (4) Maximal surgical debulking should be performed, particularly in regions suspected of malignancy[35]. In this study, IPNB in all three patients involved both the left and right intrahepatic bile ducts, with the main invasion in the left hepatic lobe. Among these patients, two underwent palliative left lobectomy, and one underwent left hepatectomy because of suspected malignancy; (5) An adequate residual liver volume should be preserved. Patients having IPNB present with severely dilated bile ducts, cirrhosis, and hepatic insufficiency, indicating that the actual functional volume of the residual liver is often overestimated. During surgery, maximal liver volume should be preserved (SRLVR: ≥ 25%-30% for Child–Pugh class A and ≥ 40%-50% for Child–Pugh class B)[34,36]. The following formulas were used in this study: Body surface area (BSA) was calculated using the DuBois formula: BSA (m2) = 0.007184 × weight (kg)0.425 × height (cm)0.725. The SLV was calculated using the Urata formula: SLV (mL) = 706.2 × BSA (m2) + 2.4 (mL). Case 2 had a BSA of 1.44 m2, SLV of 1019.328 mL, and a residual liver volume ratio of 72.5%. Case 3 had a BSA of 1.31 m2, SLV of 925 mL, and a residual liver volume ratio of 90.5%. The Vauthey formula, used in Europe and the United States to calculate SLV, is as follows: SLV (mL) = 1267.28 × BSA (m2) - 794.41 mL[36]; and (6) The patency of the biliary tract should be maintained. Surgical measures to ensure this include placing a 24F tube in the common bile duct or performing a choledochoenterostomy during surgery. In this study, T-tube placement was recommended in all three cases. Traditional palliative treatments include radiofrequency ablation, biliary stenting, ERCP, and PTCD. Compared with conservative measures like PTCD or ERCP drainage alone, debulking surgery directly reduces tumor burden, creates a T-tube tract that facilitates surveillance and subsequent intervention, and may confer a survival advantage. However, clinicians must balance these potential benefits against operative risks and the ongoing care demands associated with a T-tube.
Comprehensive management of patients with IPNB is crucial for prolonging survival and improving quality of life (QoL). Since debulking surgery is a palliative approach, patients with residual tumors still have a risk of malignancy and recurrent clinical symptoms (abdominal pain, jaundice, and recurrent cholangitis). First, relieving pressure from the biliary tract and maintaining long-term biliary drainage are essential. External drainage via 24F T-tube or PTCD is cost-effective and permits cholangioscopic surveillance. Our three patients underwent T-tube placement with regular flushing and choledochoscopic treatment. Internal drainage (Roux-en-Y choledochojejunostomy or biliary stent placement) eliminates the need for external tubes but complicates lavage and endoscopic access[37]. Second, liver function should be regularly monitored every 1-2 weeks. If any anomalies are noted in the results, hepatoprotective therapy may be indicated[38]. Our patient 1 died from liver failure, primarily due to extensive involvement of the tumor in the biliary system. Third, control of the biliary tract infection is crucial. The risk of infection is elevated by factors like intestinal bacterial invasion, mucus secretion leading to increased intraductal pressure, and the placement of foreign matter (such as drainage tubes and biliary stents)[39]. Severe infections of the biliary tract can lead to septic shock and hemorrhage. Patient 2 showed bacterial colonization of the biliary tract; bile culture should be performed to guide antibiotic selection for infection management. Besides, in patient 2, biliary bleeding was influenced not only by infection but also by the tumor’s vascular structure. IPNB tumors exhibit a vascular core with a villous architecture. Fourth, consider palliative tumor control via cholangioscopic ablation, argon plasma coagulation, photodynamic therapy, or radiofrequency ablation[40]. Patients 1 and 3 received ablation, whereas patient 2 declined. Five, therapy targeting mutations, such as KRAS, STK11, and KMT2C, may improve outcomes[27]. With adequate financial resources, more frequent cholangioscopic surveillance (every 3 months), adjuvant argon plasma coagulation, and molecularly guided therapy may offer improved outcomes.
Commonalities and differences among the three cases are as follows: In the three patients, preoperative IPNB diagnosis relied on aneurysmal bile duct dilation and intraductal papillary hyperplasia on CT/MRI, plus characteristic yellow mucus on PTCD or ERCP. CA19-9 exceeded 1000 IU/L in patient 3, indicating high malignancy risk, subsequently confirmed as invasive carcinoma on pathology. The three cases shared several common features: (1) All patients presented with obstructive jaundice as the primary clinical manifestation; (2) All patients had type I IPNB involving both the left and right intrahepatic bile ducts, precluding radical resection because of insufficient residual liver volume; (3) All patients underwent debulking surgery with 24-French T-tube drainage; and (4) All patients required long-term main
Comparative analysis reveals that IPNB has a more favorable prognosis than conventional cholangiocarcinoma, supporting consideration of aggressive surgical intervention even for palliative intent. The 1-, 2-, and 3-year survival rates for malignant IPNB are 71.9%, 58.9%, and 48.5%, respectively (median: 33 months)[13], whereas the 5-year survival rate for conventional intrahepatic cholangiocarcinoma is < 20%. Unresected malignant IPNB has a median survival of 6-8 months[25]. In our case series, survival ranges from 6 to 57 months. A meta-analysis identifies several adverse prognostic factors: R1 resection, extrahepatic location, type 2 subclassification, and elevated CA19-9 levels[41]. Postoperative recurrence and abdominal pain independently predict poorer outcomes[43]. Conversely, radical surgery independently reduces mortality risk[13]. To optimize outcomes, clinicians should develop stratified treatment algorithms that account for the histologic subtype, anatomic location, and tumor grade. Gastric-type IPNB warrants particular attention, given its association with higher rates of lymph node metastasis[27].
This study was limited by its retrospective, single-center design and small sample size. The absence of a control group precluded formal statistical comparisons. The QoL implications of debulking surgery with long-term T-tube drainage require careful consideration. The T-tube itself represents a QoL burden. However, when compared with progressive biliary obstruction with recurrent cholangitis and jaundice, debulking surgery with maintained biliary drainage provides meaningful palliation[13,25]. These observations are consistent with the principle that maintaining biliary drainage patency is the cornerstone for preserving QoL in patients with unresectable IPNB. Standardized QoL assessments (e.g., European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 or The MOS 36-Item Short-Form Health Survey) were not performed prospectively. Our QoL observations are based on clinical follow-up records. Future multi-institutional studies are needed to validate our proposed selection criteria and compare debulking surgery with conventional palliative strategies in terms of symptom control, QoL, and overall survival.
For patients with unresectable type I IPNB, volumetric-guided debulking surgery with T-tube placement represents a novel palliative strategy that reduces tumor burden, alleviates obstructive symptoms, and establishes a conduit for long-term surveillance and intervention. Long-term management should prioritize biliary drainage patency, hepatoprotective therapy, and antimicrobial treatment to prolong survival and preserve QoL.
The authors would like to thank all the patients and their families for their cooperation and consent to publish these case reports. We also thank the staff of the Department of Hepatobiliary Surgery, the Department of Radiology, and the Department of Pathology at the First Affiliated Hospital of Yangtze University for their assistance in clinical data collection and image preparation.
| 1. | Chen TC, Nakanuma Y, Zen Y, Chen MF, Jan YY, Yeh TS, Chiu CT, Kuo TT, Kamiya J, Oda K, Hamaguchi M, Ohno Y, Hsieh LL, Nimura Y. Intraductal papillary neoplasia of the liver associated with hepatolithiasis. Hepatology. 2001;34:651-658. [RCA] [PubMed] [DOI] [Full Text] [Cited by in Crossref: 232] [Cited by in RCA: 190] [Article Influence: 7.6] [Reference Citation Analysis (3)] |
| 2. | Wu X, Li B, Zheng C. Clinicopathologic characteristics and long-term prognosis of intraductal papillary neoplasm of the bile duct: a retrospective study. Eur J Med Res. 2023;28:132. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in Crossref: 11] [Cited by in RCA: 10] [Article Influence: 3.3] [Reference Citation Analysis (1)] |
| 3. | Nagtegaal ID, Odze RD, Klimstra D, Paradis V, Rugge M, Schirmacher P, Washington KM, Carneiro F, Cree IA; WHO Classification of Tumours Editorial Board. The 2019 WHO classification of tumours of the digestive system. Histopathology. 2020;76:182-188. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in Crossref: 3203] [Cited by in RCA: 3084] [Article Influence: 514.0] [Reference Citation Analysis (21)] |
| 4. | Zen Y, Pedica F, Patcha VR, Capelli P, Zamboni G, Casaril A, Quaglia A, Nakanuma Y, Heaton N, Portmann B. Mucinous cystic neoplasms of the liver: a clinicopathological study and comparison with intraductal papillary neoplasms of the bile duct. Mod Pathol. 2011;24:1079-1089. [RCA] [PubMed] [DOI] [Full Text] [Cited by in Crossref: 151] [Cited by in RCA: 125] [Article Influence: 8.3] [Reference Citation Analysis (2)] |
| 5. | Chalamalasetti K, R P, P SK, Chidambaranathan S. Intraductal Papillary Neoplasm of the Bile Duct Posing Diagnostic and Therapeutic Challenges. Cureus. 2024;16:e73501. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 1] [Reference Citation Analysis (1)] |
| 6. | Xiao SY, Shi YT, Xu JX, Sun JH, Yu RS. To develop a classification system which helps differentiate cystic intraductal papillary neoplasm of the bile duct from mucinous cystic neoplasm of the liver. Eur J Radiol. 2025;182:111822. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 3] [Reference Citation Analysis (0)] |
| 7. | Jeon SK, Lee JM, Yoo J, Park S, Joo I, Yoon JH, Lee KB. Intraductal papillary neoplasm of the bile duct: diagnostic value of MRI features in differentiating pathologic subclassifications-type 1 versus type 2. Eur Radiol. 2024;34:4674-4685. [RCA] [PubMed] [DOI] [Full Text] [Cited by in Crossref: 5] [Cited by in RCA: 6] [Article Influence: 3.0] [Reference Citation Analysis (1)] |
| 8. | Luvira V. Tri-modal death distribution towards personalized management of intraductal papillary neoplasm of the bile duct patients: Every story has its own conclusion. Hepatobiliary Pancreat Dis Int. 2024;23:313-316. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 2] [Reference Citation Analysis (1)] |
| 9. | Ramzi J, Feretis M, Hickman KE, Martin JL, Godfrey E, Liau SS. Preoperative diagnosis of intraductal papillary neoplasm of the bile duct with endoscopic ultrasound. Ann R Coll Surg Engl. 2021;103:e65-e68. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in Crossref: 1] [Cited by in RCA: 3] [Article Influence: 0.6] [Reference Citation Analysis (2)] |
| 10. | Wu RS, Liao WJ, Ma JS, Wang JK, Wu LQ, Hou P. Epidemiology and outcome of individuals with intraductal papillary neoplasms of the bile duct. World J Gastrointest Oncol. 2023;15:843-858. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in CrossRef: 12] [Cited by in RCA: 11] [Article Influence: 3.7] [Reference Citation Analysis (2)] |
| 11. | Lee SS, Kim MH, Lee SK, Jang SJ, Song MH, Kim KP, Kim HJ, Seo DW, Song DE, Yu E, Lee SG, Min YI. Clinicopathologic review of 58 patients with biliary papillomatosis. Cancer. 2004;100:783-793. [RCA] [PubMed] [DOI] [Full Text] [Cited by in Crossref: 204] [Cited by in RCA: 164] [Article Influence: 7.5] [Reference Citation Analysis (3)] |
| 12. | Ren X, Qu YP, Zhu CL, Xu XH, Jiang H, Lu YX, Xue HP. Percutaneous transhepatic cholangioscopy-assisted biliary polypectomy for local palliative treatment of intraductal papillary neoplasm of the bile duct. World J Gastrointest Oncol. 2024;16:1821-1832. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in RCA: 2] [Reference Citation Analysis (1)] |
| 13. | Zhao ZH, Zhang R, Zhang A, Yao XX, Ma L, Shi J, Bi J, Zhang HT, Zhang R, Feng Q, Lv G, Wang M. Clinicopathologic characteristics, progression, and prognostic analysis of intraductal papillary neoplasm of the bile duct: a retrospective multicenter cohort study. Int J Surg. 2026;112:4210-4218. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 1] [Reference Citation Analysis (0)] |
| 14. | Omoto S, Takenaka M, Fukunaga T, Okamoto A, Komeda Y, Jeong S, Kudo M. Diagnosis of an intraductal papillary neoplasm of the bile duct with fibrovascular stalks using detective flow imaging. Endoscopy. 2023;55:E1012-E1014. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in RCA: 3] [Reference Citation Analysis (0)] |
| 15. | Fernández Bezanilla R, Casado Morentin I, Ibarra Ponce de León C, Durá Gil M, Gendive Martín N, Martínez Moneo E. Intraductal papillary neoplasm of the bile duct: diagnostic challenges in a tertiary center. Rev Esp Enferm Dig. 2026. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 1] [Reference Citation Analysis (0)] |
| 16. | Samar MR, Abbasi Z, Masood B, Zehra NE, Zaki A. Trick or Treat: An Intrahepatic Intraductal Papillary Neoplasm of the Bile Duct. Cureus. 2023;15:e43494. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 1] [Reference Citation Analysis (0)] |
| 17. | Liu LC, Chen YF, Shan YQ, Chen LL, Zeng MS, Wang ML. [Magnetic resonance imaging features and classification of intraductal papillary neoplasm of the bile duct]. Zhonghua Gan Zang Bing Za Zhi. 2024;32:461-468. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in RCA: 1] [Reference Citation Analysis (1)] |
| 18. | Koiwai A, Hirota M, Murakami K, Katayama T, Kin R, Endo K, Kogure T, Takasu A, Sakurai H, Kondo N, Takami K, Yamamoto K, Katayose Y, Satoh K. Direct peroral cholangioscopy with red dichromatic imaging 3 detected the perihilar margin of superficial papillary extension in a patient with intraductal papillary neoplasm of the bile duct. DEN Open. 2023;3:e228. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in Crossref: 5] [Cited by in RCA: 6] [Article Influence: 2.0] [Reference Citation Analysis (1)] |
| 19. | Nakahata A, Yamashita Y, Yamazaki H, Tsuda I, Kawaji Y, Ashida R, Kitano M. Intraductal Papillary Neoplasm of the Bile Duct Treated with Argon Plasma Coagulation via Endoscopic Ultrasound-guided Choledochoduodenostomy. Intern Med. 2024;63:957-962. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 1] [Reference Citation Analysis (0)] |
| 20. | Zen Y, Fujii T, Itatsu K, Nakamura K, Konishi F, Masuda S, Mitsui T, Asada Y, Miura S, Miyayama S, Uehara T, Katsuyama T, Ohta T, Minato H, Nakanuma Y. Biliary cystic tumors with bile duct communication: a cystic variant of intraductal papillary neoplasm of the bile duct. Mod Pathol. 2006;19:1243-1254. [RCA] [PubMed] [DOI] [Full Text] [Cited by in Crossref: 142] [Cited by in RCA: 108] [Article Influence: 5.4] [Reference Citation Analysis (3)] |
| 21. | Shin IS, Moon JH, Lee YN, Park JW, Chung JC, Kim HK, Lee TH, Yang JK, Cho YD, Park SH. Utility of Peroral Cholangioscopy in the Diagnosis and Management of Intraductal Papillary Neoplasms of the Bile Duct (With Videos). J Gastroenterol Hepatol. 2026;41:803-812. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 4] [Reference Citation Analysis (3)] |
| 22. | Sung MJ, Shin SP, Kwon CI, Kang I, Lee SH, Yang SJ, Kang B, Chon HJ, Kim G, An C, Ko KH. Diagnostic cholangioscopy for surgical planning of extrahepatic cholangiocarcinoma. Sci Rep. 2025;15:3654. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 2] [Reference Citation Analysis (0)] |
| 23. | Shin IS, Moon JH, Lee YN. Intraductal Papillary Neoplasm of the Bile Duct With Associated Invasive Carcinoma Diagnosed by Peroral Cholangioscopy With Narrow-band Imaging. Clin Gastroenterol Hepatol. 2026;S1542-3565(26)00051. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 2] [Reference Citation Analysis (2)] |
| 24. | Mocchegiani F, Vincenzi P, Conte G, Nicolini D, Rossi R, Cacciaguerra AB, Vivarelli M. Intraductal papillary neoplasm of the bile duct: The new frontier of biliary pathology. World J Gastroenterol. 2023;29:5361-5373. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in CrossRef: 28] [Cited by in RCA: 16] [Article Influence: 5.3] [Reference Citation Analysis (1)] |
| 25. | Han SY, Kim DU, Nam HS, Kang DH, Jang SI, Lee DK, Shin DW, Cho KB, Yang MJ, Hwang JC, Kim JH, So H, Bang SJ, Sung MJ, Kwon CI, Lee DW, Cho CM, Cho JH. Comparison of the Malignant Predictors in Intrahepatic and Extrahepatic Intraductal Papillary Neoplasm of the Bile Duct. J Clin Med. 2022;11:1985. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in Crossref: 12] [Cited by in RCA: 13] [Article Influence: 3.3] [Reference Citation Analysis (1)] |
| 26. | Kim JR, Lee KB, Kwon W, Kim E, Kim SW, Jang JY. Comparison of the Clinicopathologic Characteristics of Intraductal Papillary Neoplasm of the Bile Duct according to Morphological and Anatomical Classifications. J Korean Med Sci. 2018;33:e266. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in Crossref: 32] [Cited by in RCA: 32] [Article Influence: 4.0] [Reference Citation Analysis (4)] |
| 27. | Shimada Y, Yamamoto T, Shindo K, Nakanishi Y, Matsumoto T, Noguchi S, Aishima S, Nakamura M, Oda Y. Clinicopathologic and Genomic Features of Gastric-Type Intraductal Papillary Neoplasm of the Bile Duct: Potential Role of STK11 in Malignant Progression. Am J Surg Pathol. 2025;49:1004-1014. [RCA] [PubMed] [DOI] [Full Text] [Cited by in Crossref: 2] [Cited by in RCA: 1] [Article Influence: 1.0] [Reference Citation Analysis (1)] |
| 28. | Nakanuma Y, Kakuda Y, Matsubayashi H, Sugino T. Intraductal Polypoid Neoplasm in the Intrahepatic Large Bile Ducts of Small Duct-type Intrahepatic Cholangiocarcinoma May Result From Cancerization of Ducts. Am J Surg Pathol. 2025;49:284-293. [RCA] [PubMed] [DOI] [Full Text] [Cited by in Crossref: 5] [Cited by in RCA: 4] [Article Influence: 4.0] [Reference Citation Analysis (1)] |
| 29. | Zheng W, Xue Y. Epithelial precursor lesions of the bile duct. Semin Diagn Pathol. 2025;42:150961. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 2] [Reference Citation Analysis (0)] |
| 30. | Tan Y, Zhu JY, Li J, Wu LM, Ouyang Z, Liu WY, Song H, Huang CY. Application of 3-dimensional visualization and image fusion technology in liver cancer with portal vein tumor thrombus surgery. Medicine (Baltimore). 2024;103:e38980. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 1] [Reference Citation Analysis (0)] |
| 31. | Wan H, Lan T, Duan T, Xie K, Huang J, Yang J, Zeng Y, Jiang L, Wu H. New classification-oriented laparoscopic anatomical hepatectomy strategy for hepatocellular carcinoma invading two or more (sub)segments in the left lobe. Chin Med J (Engl). 2022;135:2599-2601. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in RCA: 2] [Reference Citation Analysis (0)] |
| 32. | Melandro F, Centonze L, Celsa C, Famularo S, Ghinolfi D, Nardelli S, Pallozzi M, Abenavoli L, Romano F, Ponziani FR, Russo FP, Lai Q. Role of Liver Function in the Multiparametric Assessment of Hepatocellular Carcinoma. Medicina (Kaunas). 2026;62:138. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in RCA: 1] [Reference Citation Analysis (2)] |
| 33. | Meng Q, Yang Z, Zhu Z, Li J, Bi X, Chen X, Hao C, Huang Z, Li F, Li X, Li G, Yang Y, Zhang Y, Zhao H, Zhao H, Zhu X, Zhu J, Cai J; Liver Tumor Branch of the China International Exchange and Promotive Association for Medical and Healthcare (CPAM); Gastrointestinal Cancer Multidisciplinary Cooperation Group of Cancer Hospital; Chinese Academy of Medical Sciences & Peking Union Medical College; Liver Cancer Professional Committee of Chinese Medical Doctor Association. Chinese expert consensus on the overall management of liver function in conversion therapy for liver cancer (2022 edition). Chin Med J (Engl). 2023;136:2909-2911. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in Crossref: 1] [Cited by in RCA: 2] [Article Influence: 0.7] [Reference Citation Analysis (0)] |
| 34. | Ito T, Hisa T, Ito Y, Kudo A, Yamada T, Osera S, Tomori A, Fukushima H, Aoyagi D, Shiozawa S. Intraductal papillary neoplasm of the bile duct with metachronous development in the downstream bile duct after radical resection. Clin J Gastroenterol. 2024;17:155-163. [RCA] [PubMed] [DOI] [Full Text] [Cited by in Crossref: 1] [Cited by in RCA: 2] [Article Influence: 1.0] [Reference Citation Analysis (0)] |
| 35. | Zhao Y, Bie YK, Zhang GY, Feng YB, Wang F. Rare and lacking typical clinical symptoms of liver tumors: Four case reports. World J Gastrointest Oncol. 2024;16:4264-4273. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in RCA: 1] [Reference Citation Analysis (0)] |
| 36. | Urata K, Kawasaki S, Matsunami H, Hashikura Y, Ikegami T, Ishizone S, Momose Y, Komiyama A, Makuuchi M. Calculation of child and adult standard liver volume for liver transplantation. Hepatology. 1995;21:1317-1321. [PubMed] |
| 37. | Patel A, Sonnenday CJ, Schulman AR. Recurrent extrahepatic cholangiocarcinoma after bile duct resection for intraductal papillary mucinous neoplasm of the bile duct. VideoGIE. 2019;4:519-521. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in Crossref: 3] [Cited by in RCA: 4] [Article Influence: 0.6] [Reference Citation Analysis (0)] |
| 38. | Marín-Serrano E, Barbado Cano A. Intraductal papillary neoplasm of the bile duct: a recurring disease. Rev Esp Enferm Dig. 2019;111:890-891. [RCA] [PubMed] [DOI] [Full Text] [Cited by in Crossref: 1] [Cited by in RCA: 3] [Article Influence: 0.5] [Reference Citation Analysis (0)] |
| 39. | Aparicio Serrano A, Gómez Pérez A, Zamora Olaya JM, Rodríguez Perálvarez ML. Malignancy of intraductal papillary neoplasm of the bile duct. Rev Esp Enferm Dig. 2022;114:170-171. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 2] [Reference Citation Analysis (0)] |
| 40. | Tang W, Qiu JG, Wei XF, Xiao H, Deng X, Wang SD, Du CY, Wu Q. Endoscopic Endoluminal Radiofrequency Ablation and Single-Operator Peroral Cholangioscopy System (SpyGlass) in the Diagnosis and Treatment of Intraductal Papillary Neoplasm of the Bile Duct: A Case Report and Literature Review. Front Med (Lausanne). 2021;8:675720. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in Crossref: 1] [Cited by in RCA: 11] [Article Influence: 2.2] [Reference Citation Analysis (0)] |
| 41. | Zeng D, Li B, Cheng N. Prognostic factors for intraductal papillary neoplasm of the bile duct following surgical resection: a systematic review and meta-analysis. Surg Today. 2025;55:131-143. [RCA] [PubMed] [DOI] [Full Text] [Cited by in RCA: 2] [Reference Citation Analysis (0)] |
| 42. | Khot R, Ganeshan D, Sundaram KM, Depetris JN, Ludwig DR. Cystic lesions and their mimics involving the intrahepatic bile ducts and peribiliary space: diagnosis, complications, and management. Abdom Radiol (NY). 2025;50:2969-2988. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in RCA: 2] [Reference Citation Analysis (1)] |
| 43. | Fang LY, Tang WW, Bai J, Zheng P, Huang D, Cao L, Shu J, Gan L. Intraductal papillary neoplasm of the bile duct: a clinical analysis of 62 cases and evaluation of prognostic factors. BMC Surg. 2026;26:238. [RCA] [PubMed] [DOI] [Full Text] [Full Text (PDF)] [Cited by in RCA: 1] [Reference Citation Analysis (0)] |