Overview of ectopic pancreas

This editorial discusses the article written by Zheng et al that was published in the latest edition of the World Journal of Gastrointestinal Surgery.Our primary focus is on the causes,location,diagnosis,histological classification,and therapy of ectopic pancreas.Ectopic pancreas refers to the presence of pancreatic tissue that is situated in a location outside its usual anatomical placement,and is not connected to the normal pancreas in terms of blood supply or anatomical struc-ture.Currently,the embryological origin of ectopic pancreas remains uncertain.The most prevalent form of ectopic pancreatic is gastric ectopic pancreas.Endoscopic ultrasonography examination can visualize the morphological charac-teristics of the ectopic pancreatic lesion and pinpoint its anatomical location.The histological categorization of ectopic pancreas evolves.Endoscopic treatment has been widely advocated in ectopic pancreas.

Plea to radiologists:Please consider Mahvash disease when encountering an enlarged pancreas

Radiologists play a key role in establishing an early and accurate diagnosis,especially for rare diseases.Mahvash disease(OMIM 619290)is an autosomal recessive hereditary disease caused by inactivating mutations of the glucagon receptor and its main clinical consequences are pancreatic neuroendocrine tumors and in some cases,porto-sinusoidal vascular disease and portal hypertension.Untreated Mahvash disease can be lethal.The diagnosis of Mahvash disease has almost always been delayed in the past due to radiologists’unawareness of or unfamiliarity with the unique imaging features of Mahvash disease which are moderately to enormously enlarge pancreas with preserved pancreas contour and parenchyma without vascular involvement or lymphadenopathy.These features help differentiate Mahvash disease from other etiologies of diffusely enlarged pancreas such as diffuse pancreatic ductal carcinoma,diffuse pancreatic lymphoma,and autoimmune pancreatitis.Invoking Mahvash disease in the differential diagnosis of an enlarged pancreas has recently been shown to facilitate early diagnosis.To prevent missing the diagnosis of this significant disease,I sincerely ask radiologists to consider Mahvash disease in their differential diagnoses of diffusely enlarged pancreas.

Metastatic clear cell sarcoma of the pancreas:A rare case report

BACKGROUND Clear cell sarcoma(CCS)is a rare soft-tissue sarcoma.The most common metastatic sites for CCS are the lungs,bones and brain.CCS is highly invasive and mainly metastasizes to the lung,followed by the bone and brain;however,pancreatic metastasis is relatively rare.CASE SUMMARY We report on a rare case of CCS with pancreatic metastasis in a 47-year-old man.The patient had a relevant medical history 3 years ago,with abdominal pain as the main clinical manifestation.No abnormalities were observed on physical examination and the tumor was found on abdominal computed tomography.Based on the medical history and postoperative pathology,the patient was diagnosed with CCS with pancreatic metastasis.The patient was successfully treated with surgical interventions,including distal pancreatectomy and sple-nectomy.CONCLUSION This report summarizes the available treatment modalities for CCS and the importance of regular postoperative follow-up for patients with CCS.

Unraveling the enigma:A comprehensive review of solid pseudopapillary tumor of the pancreas

Solid pseudopapillary tumor of the pancreas(SPTP)is a rare neoplasm predom-inantly observed in young females.Pathologically,CTNNB1 mutations,β-catenin nuclear accumulation,and subsequent Wnt-signaling pathway activation are the leading molecular features.Accurate preoperative diagnosis often relies on imaging techniques and endoscopic biopsies.Surgical resection remains the mainstay treatment.Risk models,such as the Fudan Prognostic Index,show promise as predictive tools for assessing the prognosis of SPTP.Establishing three types of metachronous liver metastasis can be beneficial in tailoring individu-alized treatment and follow-up strategies.Despite advancements,challenges persist in understanding its etiology,establishing standardized treatments for unresectable or metastatic diseases,and developing a widely recognized grading system.This comprehensive review aims to elucidate the enigma by consolidating current knowledge on the epidemiology,clinical presentation,pathology,molecular characteristics,diagnostic methods,treatment options,and prognostic factors.

Imaging, pathology, and diagnosis of solitary fibrous tumor of the pancreas: A case report and review of literature

BACKGROUND A solitary fibrous tumor(SFT)is often located in the pleura,while SFT of the pancreas is extremely rare.Here,we report a case of SFT of the pancreas and discuss imaging,histopathology,and immunohistochemistry for accurate diagnosis and treatment.CASE SUMMARY A 54-year-old man presented to our hospital with pancreatic occupancy for over a month.There were no previous complaints of discomfort.His blood pressure was normal.Blood glucose,tumor markers,and enhanced computed tomography(CT)suggested a malignant tumor.Because the CT appearance of pancreatic cancer varies,we could not confirm the diagnosis;therefore,we performed endoscopic ultrasound-guided fine-needle biopsy(EUS-FNB).Pathology and immunohistochemistry were consistent with SFT of the pancreas.The posto-perative pathology and immunohistochemistry were consistent with the puncture results.The patient presented for a follow-up examination one month after discharge with no adverse effects.CONCLUSION Other diseases must be excluded in patients with a pancreatic mass that cannot be diagnosed.CT and pathological histology have diagnostic value for pancreatic tumors.Endoscopic puncture biopsy under ultrasound can help diagnose pancreatic masses that cannot be diagnosed preoperatively.Surgery is an effective treatment for SFT of the pancreas;however,long-term follow-up is strongly recommended because of the possibility of malignant transformation of the tumor.

Heterotopic pancreas in the gallbladder:A case report

BACKGROUND Heterotopic pancreas(HP)refers to pancreatic tissue located in areas with no vascular or anatomical connection to the pancreas.HP occurs mostly in the stomach,duodenum,and colon,and rarely in the gallbladder.CASE SUMMARY A 57-year-old woman was referred to our hospital complaining of right upper quadrant discomfort for 3 years.An abdominal computed tomography scan revealed adenomyomatosis with a thickened fundus of the gallbladder.The patient underwent a laparoscopic cholecystectomy,and pathological examination unexpectedly showed heterotopic pancreatic tissue in the gallbladder.The patient had a favorable recovery and was discharged on postoperative day 3.She did not report any symptoms or complications at the 6-mo postoperative follow-up.Pathologists should pay close attention to such pancreatic tissue and carefully examine it for dysplasia or malignancy.CONCLUSION This case provides more information about HP in the gallbladder,a rare occurrence.

Metastatic clear cell sarcoma of the pancreas:A sporadic cancer

Primary or secondary clear cell sarcoma of the pancreas is an exceedingly rare and aggressive disease.In addition to pathology,molecular analysis is pivotal in differential diagnosis,especially with malignant melanoma.A key aspect in identifying clear cell sarcoma is specific genetic alterations,notably the translocation of t(12;22)(q13;q13),a diagnostic hallmark of this sarcoma subtype,which is absent in malignant melanoma.Treatment of primary clear cell sarcoma of the pancreas is the same as that for adenocarcinoma.

Solid pseudopapillary tumor of the pancreas:A systematic review of clinical,surgical and oncological characteristics of 1384 patients underwent pancreatic surgery

Background:Pancreatic solid pseudopapillary tumors(SPTs)are rare clinical entity,with low malignancy and still unclear pathogenesis.They account for less than 2%of exocrine pancreatic neoplasms.This study aimed to perform a systematic review of the main clinical,surgical and oncological characteristics of pancreatic SPTs.Data sources:MEDLINE/PubMed,Web of Science and Scopus databases were systematically searched for the main clinical,surgical and oncological characteristics of pancreatic SPTs up to April 2021,in accordance with the preferred reporting items for systematic reviews and meta-analyses(PRISMA)standards.Primary endpoints were to analyze treatments and oncological outcomes.Results:A total of 823 studies were recorded,86 studies underwent full-text reviews and 28 met inclusion criteria.Overall,1384 patients underwent pancreatic surgery.Mean age was 30 years and 1181 patients(85.3%)were female.The most common clinical presentation was non-specific abdominal pain(52.6%of cases).Mean overall survival was 98.1%.Mean recurrence rate was 2.8%.Mean follow-up was 4.2 years.Conclusions:Pancreatic SPTs are rare,and predominantly affect young women with unclear pathogenesis.Radical resection is the gold standard of treatment achieving good oncological impact and a favorable prognosis in a yearly life-long follow-up.

Metastatic clear cell sarcoma of the pancreas:An overview

Clear cell sarcoma(CCS)is a rare soft-tissue sarcoma that accounts for less than 1%of all cases and was originally reported in 1965.The incidence of CCS is estimated to be approximately 0.014/100000 depending on the surveillance,epidemiology and end results databases.CCS is a highly invasive type that mainly metastasizes to the lungs,followed by the bones and brain;however,pancreatic metastasis is relatively rare.It has a high probability of recurrence or metastasis and has a poor prognosis with a high mortality rate.Finally,even after recovery,it is fundamental to keep regular postoperative follow-up for CCS patients.

Long-term effects of gestational diabetes mellitus on the pancreas of female mouse offspring

BACKGROUND Prolonged fetal exposure to hyperglycemia may increase the risk of developing abnormal glucose metabolism and type-2 diabetes during childhood,adolescence,and adulthood;however,the mechanisms by which gestational diabetes mellitus(GDM)predisposes offspring to metabolic disorders remain unknown.AIM To quantify the nerve axons,macrophages,and vasculature in the pancreas from adult offspring born from mouse dams with GDM.METHODS GDM was induced by i.p.administration of streptozotocin(STZ)in ICR mouse dams.At 12 wk old,fasting blood glucose levels were determined in offspring.At 15 wk old,female offspring born from dams with and without GDM were sacrificed and pancreata were processed for immunohistochemistry.We quantified the density of sensory[calcitonin gene-related peptide(CGRP)]and tyrosine hydroxylase(TH)axons,blood vessels(endomucin),and macro-phages(CD68)in the splenic pancreas using confocal microscopy.RESULTS Offspring mice born from STZ-treated dams had similar body weight and blood glucose values compared to offspring born from vehicle-treated dams.However,the density of CGRP+and TH+axons,endomucin+blood vessels,and CD68+macrophages in the exocrine pancreas was significantly greater in offspring from mothers with GDM vs control offspring.Likewise,the microvasculature in the islets was significantly greater,but not the number of macrophages within the islets of offspring born from dams with GDM compared to control mice.CONCLUSION GDM induces neuronal,vascular,and inflammatory changes in the pancreas of adult progeny,which may partially explain the higher propensity for offspring of mothers with GDM to develop metabolic diseases.

Advances in the diagnosis and treatment of heterotopic pancreas

Heterotopic pancreas,a rare congenital malformation,manifests outside the normal pancreas.Research suggests that abnormal embryonic development is linked to the presence of heterotopic pancreas.Three prevailing theories explain its mechanism:Dislocation theory,metaplasia theory,and totipotent stem cell theory.Clinical presentations of heterotopic pancreas are often nonspecific,with most patients being asymptomatic and incidentally discovered during unrelated surgeries or examinations.Endoscopic ultrasound,computed tomography,and magnetic resonance imaging are commonly employed diagnostic tools for heterotopic pancreas.However,the accuracy of diagnosis based on these methods is not consistently high,necessitating histopathological confirmation in many cases.Treatment options for heterotopic pancreas typically involve endoscopic resection,surgical resection,or observation through follow-up.

Meningeal cryptococcosis in a pancreas transplant recipient requiring grafectomy: A case report

BACKGROUND Through continuous improvement in transplantation medicine,a wider range of solid organ transplant(SOT)recipients is considered suitable for complex procedures.Despite advances in modern transplantation practice,transpiring invasive fungal infections pose a substantial threat for SOT recipients.To our knowledge,cryptococcal infection confined amidst sole pancreas SOT recipients has not been described to date.Enforcement of a multidisciplinary transplant team approach in the management of pancreas SOT recipients presenting with complex cryptococcal complications is fundamental in improving patient outcomes.CASE SUMMARY We present the case of a female pancreas transplant recipient,with confirmed meningeal cryptococcosis,referred to our institution for further evaluation and treatment from the Regional Center for Infectious Diseases.On admission,the patient was weaned from the protocolized immunosuppression therapy for two consecutive weeks,in addition to tapering systemic corticosteroid remedial treatment.Our novel multidisciplinary transplant team approach embodied exhaustive discussions of possible complex and diverse multiple organ system physiologic and pathologic challenges associated with distinct management strategies in pancreas transplant recipients.Owing to the potentially devastating impact of invasive cryptococcosis in terms of morbidity and mortality,a definitive surgical intervention of pancreas transplant grafectomy was reinforced,as a pathway towards secure access to early meaningful expertise care.The patient was discharged to the Regional Center for Infectious Diseases 2 mo after the admittance further advancing to a clinical improvement.CONCLUSION The precision transplantation approach by tailoring complex medical interventions to individual needs proved indispensable in improving our patient’s outcomes.

Reproducibility study of intravoxel incoherent motion and apparent diffusion coefficient parameters in normal pancreas

BACKGROUND The consistency of pancreatic apparent diffusion coefficient(ADC)values and intravoxel incoherent motion(IVIM)parameter values across different magnetic resonance imaging(MRI)devices significantly impacts the patient’s diagnosis and treatment.AIM To explore consistency in image quality,ADC values,and IVIM parameter values among different MRI devices in pancreatic examinations.METHODS This retrospective study was approved by the local ethics committee,and informed consent was obtained from all participants.In total,22 healthy volunteers(10 males and 12 females)aged 24-61 years(mean,28.9±2.3 years)underwent pancreatic diffusion-weighted imaging using 3.0T MRI equipment from three vendors.Two independent observers subjectively scored image quality and measured the pancreas’s overall ADC values and signal-to-noise ratios(SNRs).Subsequently,regions of interest(ROIs)were delineated for the IVIM parameters(true diffusion coefficient,pseudo-diffusion coefficient,and perfusion fraction)using post-processing software.These ROIs were on the head,body,and tail of the pancrease.The subjective image ratings were assessed using the kappa consistency test.Intraclass correlation coefficients(ICCs)and mixed linear models were used to evaluate each device’s quantitative parameter values.Finally,a pairwise analysis of IVIM parameter values across each device was performed using Bland-Altman plots.RESULTS The Kappa value for the subjective ratings of the different observers was 0.776(P<0.05).The ICC values for interobserver and intra-observer agreements for the quantitative parameters were 0.803[95% confidence interval(CI):0.684-0.880]and 0.883(95%CI:0.760-0.945),respectively(P<0.05).The ICCs for the SNR between different devices was comparable(P>0.05),and the ICCs for the ADC values from different devices were 0.870,0.707,and 0.808,respectively(P<0.05).Notably,only a few statistically significant inter-device agreements were observed for different IVIM parameters,and among those,the ICC values were generally low.The mixed linear model results indicated differences(P<0.05)in the f-value for the pancreas head,D-value for the pancreas body,and D-value for the pancreas tail obtained using different MRI machines.The Bland-Altman plots showed significant variability at some data points.CONCLUSION ADC values are consistent among different devices,but the IVIM parameters’repeatability is moderate.Therefore,the variability in the IVIM parameter values may be associated with using different MRI machines.Thus,caution should be exercised when using IVIM parameter values to assess the pancreas.

Sclerosing epithelioid fibrosarcoma of the pancreas:A case report

BACKGROUND A sclerosing epithelioid fibrosarcoma(SEF)is a rare malignant fibroblastic soft tissue tumor that rarely occurs in intra-abdominal organs.A case of a SEF in the pancreatic head is reported herein,including its clinical manifestations,preoperative imaging features,gross specimen and pathological findings.CASE SUMMARY A 33-year-old male patient was admitted to Peking Union Medical College Hospital in December 2023 due to a one-year history of intermittent upper abdominal pain and the discovery of a pancreatic mass.The patient underwent an enhanced computed tomography scan of the abdomen,which revealed a welldefined,round mass with clear borders and calcifications in the pancreatic head.The mass exhibited progressive,uneven mild enhancement,measuring approximately 6.6 cm×6.3 cm.The patient underwent laparoscopic pylorus-preserving pancreaticoduodenectomy.Postoperative pathological examination revealed that the lesion was consistent with a SEF.At the 3-month postoperative follow-up,the patient did not report any short-term complications,and there were no signs of tumor recurrence.CONCLUSION SEFs are rare malignant fibrous soft tissue tumors.SEFs rarely develop in the pancreas,and its preoperative diagnosis depends on imaging findings,with confirmation depending on pathological examination and immunohistochemistry.Currently,only four cases of pancreatic SEF have been reported in studies written in English.This case is the first reported case of a pancreatic SEF by a clinical physician.

Comparison between solid pseudopapillary neoplasms of the pancreas and pancreatic ductal adenocarcinoma with cystic changes using computed tomography

BACKGROUND Solid pseudopapillary neoplasms of the pancreas(SPN)share similar imaging findings with pancreatic ductal adenocarcinoma with cystic changes(PDAC with cystic changes),which may result in unnecessary surgery.AIM To investigate the value of computed tomography(CT)in differentiation of SPN from PDAC with cystic changes.METHODS This study retrospectively analyzed the clinical and imaging findings of 32 patients diagnosed with SPN and 14 patients diagnosed with PDAC exhibiting cystic changes,confirmed through pathological diagnosis.Quantitative and qualitative analysis was performed,including assessment of age,sex,tumor size,shape,margin,density,enhancement pattern,CT values of tumors,CT contrast enhancement ratios,“floating cloud sign,”calcification,main pancreatic duct dilatation,pancreatic atrophy,and peripancreatic invasion or distal metastasis.Multivariate logistic regression analysis was used to identify relevant features to differentiate between SPN and PDAC with cystic changes,and receiver operating characteristic curves were obtained to evaluate the diagnostic performance of each variable and their combination.RESULTS When compared to PDAC with cystic changes,SPN had a lower age(32 years vs 64 years,P<0.05)and a slightly larger size(5.41 cm vs 3.90 cm,P<0.05).SPN had a higher frequency of“floating cloud sign”and peripancreatic invasion or distal metastasis than PDAC with cystic changes(both P<0.05).No significant difference was found with respect to sex,tumor location,shape,margin,density,main pancreatic duct dilatation,calcification,pancreatic atrophy,enhancement pattern,CT values of tumors,or CT contrast enhancement ratios between the two groups(all P>0.05).The area under the receiver operating characteristic curve of the combination was 0.833(95%confidence interval:0.708-0.957)with 78.6%sensitivity,81.3%specificity,and 80.4%accuracy in differentiation of SPN from PDAC with cystic changes.CONCLUSION A larger tumor size,“floating cloud sign,”and peripancreatic invasion or distal metastasis are useful CT imaging features that are more common in SPN and may help discriminate SPN from PDAC with cystic changes.

Mortality assessment for pancreas transplants in the United States over the decade 2008-2018

BACKGROUND Pancreas transplant is the only treatment that establishes normal glucose levels for patients diagnosed with diabetes.However,since 2005,no comprehensive analysis has compared survival outcomes of:(1)Simultaneous pancreas-kidney(SPK)transplant;(2)Pancreas after kidney(PAK)transplant;and(3)Pancreas transplant alone(PTA)to waitlist survival.AIM To explore the outcomes of pancreas transplants in the United States during the decade 2008-2018.METHODS Our study utilized the United Network for Organ Sharing Standard Transplant Analysis and Research file.Pre-and post-transplant recipient and waitlist characteristics and the most recent recipient transplant and mortality status were used.We included all patients with type I diabetes listed for pancreas or kidneypancreas transplant between May 31,2008 and May 31,2018.Patients were grouped into one of three transplant types:SPK,PAK,or PTA.RESULTS The adjusted Cox proportional hazards models comparing survival between transplanted and non-transplanted patients in each transplant type group showed that patients who underwent an SPK transplant exhibited a significantly reduced hazard of mortality[hazard ratio(HR)=0.21,95%confidence intervals(CI):0.19-0.25]compared to those not transplanted.Neither PAK transplanted patients(HR=1.68,95%CI:0.99-2.87)nor PTA patients(HR=1.01,95%CI:0.53-1.95)experienced significantly different hazards of mortality compared to patients who did not receive a transplant.CONCLUSION When assessing each of the three transplant types,only SPK transplant offered a survival advantage compared to patients on the waiting list.PKA and PTA transplanted patients demonstrated no significant differences compared to patients who did not receive a transplant.

Laparoscopic resection and endoscopic submucosal dissection for treating gastric ectopic pancreas

BACKGROUND Gastric ectopic pancreas(GEP)is a rare developmental abnormality that refers to the existence of pancreatic tissue in the stomach with no anatomical relationship with the main pancreas.It is usually difficult to diagnose through histological examination,and the choice of treatment method is crucial.AIM To describe the endoscopic ultrasound characteristics of GEP and evaluate the value of laparoscopic resection(LR)and endoscopic submucosal dissection(ESD).METHODS Forty-nine patients with GEP who underwent ESD and LR in the Second Affiliated Hospital of Fujian Medical University from May 2018 to July 2023 were retrospectively included.Data on clinical characteristics,endoscopic ultrasonography(EUS),ESD,and LR were collected and analyzed.The characteristics of EUS and the efficacy of the two treatments were analyzed.RESULTS The average age of the patients was 43.31±13.50 years,and the average maximum diameter of the lesions was 1.55±0.70 cm.The lesion originated from the mucosa in one patient(2.04%),from the submucosa in 42 patients(85.71%),and from the muscularis propria in 6 patients(12.25%).Twenty-nine patients(59.20%)with GEP showed umbilical depression on endoscopy.The most common initial symptom of GEP was abdominal pain(40.82%).Tumor markers,including carcinoembryonic antigen(CEA)and carbohydrate antigen 19-9(CA19-9),were generally within the normal range.One patient(2.04%)with GEP had increased CEA and CA-19-9 levels.However,no cancer tissue was found on postoperative pathological examination,and tumor markers returned to normal levels after resecting the lesion.There was no significant difference in surgery duration(72.42±23.84 vs 74.17±12.81 min)or hospital stay(3.70±0.91 vs 3.83±0.75 d)between the two methods.LR was more often used for patients with larger tumors and deeper origins.The amount of bleeding was significantly higher in LR than in ESD(11.28±16.87 vs 16.67±8.76 mL,P<0.05).Surgery was associated with complete resection of the lesion without any serious complications;there were no cases of recurrence during the follow-up period.CONCLUSION GEP has unique characteristics in EUS.LR and ESD seem to be good choices for treating GEP.LR is better for large GEP with a deep origin.However,due to the rarity of GEP,multicenter large-scale studies are needed to describe its characteristics and evaluate the safety of LR and ESD.

Intraductal papillary mucinous neoplasm originating from a jejunal heterotopic pancreas:A case report

BACKGROUND Intraductal papillary mucinous neoplasm(IPMN)is a rare pancreatic tumor and has the potential to become malignant.Surgery is the most effective treatment at present,but there is no consensus on the site of resection.Heterotopic pancreas occurs in the gastrointestinal tract,especially the stomach and duodenum but is asymptomatic and rare.We report a case of ectopic pancreas with IPMN located in the jejunum.CASE SUMMARY A 56-year-old male patient suffered from severe pain,nausea and vomiting due to a traffic accident and sought emergency treatment at our hospital.Contrast-enhanced computed tomography of the whole abdomen suggested splenic congestion,which was considered to be splenic rupture.Emergency laparotomy was performed,and the ruptured spleen was removed during the operation.Unexpectedly,a cauliflower-like mass of about 2.5 cm×2.5 cm in size was incidentally found about 80 cm from the ligament of Treitz during the operation.A partial small bowel resection was performed,and postoperative pathology confirmed the small bowel mass as heterotopic pancreas with low-grade IPMN.CONCLUSION Ectopic pancreas occurs in the jejunum and is pathologically confirmed as IPMN after surgical resection.

Gastric and intestinal ectopic pancreas: Two case reports

BACKGROUND Ectopic pancreas may be unfamiliar to many people because it is rare and difficult to diagnose.However,this disease is highly susceptible to misdiagnosis and missed diagnosis.In this article,we report two cases of pancreatic heterotopia in the gastric sinus and small intestine,respectively,both of which were confirmed by histopathological examination.CASE SUMMARY The first patient was a 43-year-old female which reported abdominal distension for 2 mo.The second was a 67-year-old female who experienced intermittent epigastric discomfort for 15 d.In both cases,there was no confirmed preoperative examination,and the postoperative pathology indicated the presence of ectopic pancreas.CONCLUSION The diagnosis of ectopic pancreas is difficult,and is often prone to misdiagnosis and the possibility of being overlooked.Various laboratory tests and imaging tests should be carefully evaluated before surgery to achieve early detection,early diagnosis and early treatment.

Reoperation for heterochronic intraductal papillary mucinous neoplasm of the pancreas after bile duct neoplasm resection:A case report

BACKGROUND Intraductal papillary neoplasm of the bile duct(IPNB)and intraductal papillary mucinous neoplasm(IPMN)of the pancreas have similar pathological manifestations.However,they often develop separately and it is rare for both to occur together.Patients presenting with heterochronic IPMN after IPNB are prone to be misdiagnosed with tumor recurrence.CASE SUMMARY A 67-year-old male patient was admitted 8.5 years after IPNB carcinoma and 4 years after the discovery of a pancreatic tumor.A left hepatic bile duct tumor with distal bile duct dilatation was found 8.5 years ago by the computed tomography;therefore,a left hepatectomy was performed.The postoperative pathological diagnosis was malignant IPNB with negative cutting edge and pathological stage T1N0M0.Magnetic resonance imaging 4 years ago showed cystic lesions in the pancreatic head with pancreatic duct dilatation,and carcinoembryonic antigen continued to increase.Positron emission tomography showed a maximum standard uptake value of 11.8 in the soft tissue mass in the pancreatic head,and a malignant tumor was considered.Radical pancreatoduodenectomy was performed.Postoperative pathological diagnosis was pancreatic head IPMN with negative cutting edge,pancreaticobiliary type,stage T3N0M0.He was discharged 15 d after the operation.Follow-up for 6 mo showed no tumor recurrence,and quality of life was good.CONCLUSION IPNB and IPMN are precancerous lesions with similar pathological characteristics and require active surgery and long-term follow-up.