Prevalence of malignant neoplasms in celiac disease patients-a nationwide United States population-based study

BACKGROUND Celiac disease(CeD)is an autoimmune disorder triggered by the immune response to gluten in genetically predisposed individuals.Recent research has unveiled a heightened risk of developing specific malignant neoplasms(MN)and various malignancies,including gastrointestinal,lymphomas,skin,and others,in individuals with CeD.AIM To investigate the prevalence of MN in hospitalized CeD patients in the United States.METHODS Using data from the National Inpatient Sample spanning two decades,from January 2000 to December 2019,we identified 529842 CeD patients,of which 78128(14.75%)had MN.Propensity score matching,based on age,sex,race,and calendar year,was employed to compare CeD patients with the general non-CeD population at a 1:1 ratio.RESULTS Positive associations were observed for several malignancies,including small intestine,lymphoma,nonmelanoma skin,liver,melanoma skin,pancreas myelodysplastic syndrome,biliary,stomach,and other neuroendocrine tumors(excluding small and large intestine malignant carcinoid),leukemia,uterus,and testis.Conversely,CeD patients exhibited a reduced risk of respiratory and secondary malignancies.Moreover,certain malignancies showed null associations with CeD,including head and neck,nervous system,esophagus,colorectal,anus,breast,malignant carcinoids,bone and connective tissues,myeloma,cervix,and ovary cancers.CONCLUSION Our study is unique in highlighting the detailed results of positive,negative,or null associations between different hematologic and solid malignancies and CeD.Furthermore,it offers insights into evolving trends in CeD hospital outcomes,shedding light on advancements in its management over the past two decades.These findings contribute valuable information to the understanding of CeD’s impact on health and healthcare utilization.

Clinicopathological characteristics and typing of multilocular cystic renal neoplasm of low malignant potential

BACKGROUND Up until now,no research has been reported on the association between the cli-nical growth rate of multilocular cystic renal neoplasm of low malignant potential(MCRNLMP)and computed tomography(CT)imaging characteristics.Our study sought to examine the correlation between them,with the objective of distin-guishing unique features of MCRNLMP from renal cysts and exploring effective management strategies.AIM To investigate optimal management strategies of MCRNLMP.METHODS We retrospectively collected and analyzed data from 1520 patients,comprising 1444 with renal cysts and 76 with MCRNLMP,who underwent renal cyst decom-pression,radical nephrectomy,or nephron-sparing surgery for renal cystic disease between January 2013 and December 2021 at our institution.Detection of MC-RNLMP utilized the Bosniak classification for imaging and the 2016 World Health Organization criteria for clinical pathology.RESULTS Our meticulous exploration has revealed compelling findings on the occurrence of MCRNLMP.Precisely,it comprises 1.48%of all cases involving simple renal cysts,5.26%of those with complex renal cysts,and a noteworthy 12.11%of renal tumors coexisting with renal cysts,indicating a statistically significant difference(P=0.001).Moreover,MCRNLMP constituted a significant 22.37%of the patient po-pulation whose cysts demonstrated a rapid growth rate of≥2.0 cm/year,whereas it only represented 0.66%among those with a growth rate below 2.0 cm/year.Of the 76 MCRNLMP cases studied,none of the nine patients who underwent subsequent nephron-sparing surgery or radical nephrectomy following renal cyst decompression experienced recurrence or metastasis.In the remaining 67 patients,who were actively monitored over a 3-year postoperative period,only one showed suspicious recurrence on CT scans.CONCLUSION MCRNLMP can be tentatively identified and categorized into three types based on CT scanning and growth rate indicators.In treating MCRNLMP,partial nephrectomy is preferred,while radical nephrectomy should be minimi-zed.After surgery,active monitoring is advisable to prevent unnecessary nephrectomy.

Malignant myopericytoma originating from the colon: A case report

BACKGROUND Myopericytoma is a benign tumor that typically occurs within subcutaneous tissue and most often involves the distal extremities,followed by the proximal extremities,neck,thoracic vertebrae and oral cavity.Complete resection is often curative.Malignant myopericytoma is extremely rare and has a poor prognosis.Here,we report for the first time a case of malignant myopericytoma originating from the colon.CASE SUMMARY A 69-year-old male was admitted to our hospital with right upper quadrant pain for five days.Imaging suggested a liver mass with hemorrhage.A malignant hepatic tumor was the initial diagnosis.Surgical resection was performed after a complete preoperative work up.Initial postoperative pathology suggested that the mass was a malignant myoblastoma unrelated to the liver.Four months after the first surgery,an enhanced computed tomography(CT)scan revealed a recurrence of the tumor.The diagnosis of malignant myopericytoma derived from the colon was confirmed on histopathological examination of the specimen from the second surgery.The patient did not return to the hospital regularly for surveillance.The first postoperative abdominal CT examination six months after the second surgery demonstrated multiple liver metastases.Survival time between the diagnosis of the tumor to death was approximately one year.CONCLUSION Malignant myopericytoma is a rare cancer.Preoperative diagnosis may be difficult.Due to a lack of treatment options,prognosis is poor.

Synchronous multiple primary malignant neoplasms in breast,kidney,and bilateral thyroid:A case report

BACKGROUND Multiple primary malignant neoplasms(MPMNs)are rare,while synchronous MPMNs(SMPMNs)are even less common.Owing to the progression of medical technology and the extension of life expectancy,its incidence is gradually increasing.CASE SUMMARY Although reports of breast and thyroid dual cancers are common,cases of an additional diagnosis of kidney primary cancer within the same individual are rare.CONCLUSION We present a case of simultaneous MPMN of three endocrine organs,reviewing the relevant literature to enhance our understanding of SMPMNs while emphasizing the increasingly important need for accurate diagnosis and multidisciplinary management whenever this challenging situation arises.

Malignancy risk factors and prognostic variables of pancreatic mucinous cystic neoplasms in Chinese patients

BACKGROUND Pancreatic mucinous cystic neoplasms(MCNs)represent one of the precursor lesions of pancreatic ductal adenocarcinoma,and their detection has been facilitated by advances in preoperative imaging.Due primarily to the rarity of MCNs,however,there is limited knowledge regarding the prognostic variables and high-risk factors for malignant transformation.A more comprehensive and nuanced approach is necessary to fill this gap and provide a basis for improved treatment decisions and patient outcomes.AIM To investigate the high-risk factors associated with malignant MCNs and to explore the prognostic factors of MCN with associated invasive carcinoma(MCNAIC).METHODS All cases of resected MCNs from a single high-volume institution between January 2012 and January 2022 were retrospectively reviewed.Only cases with ovarian-type stroma verified by progesterone receptor staining were included.Preoperative features,histological findings and postoperative course were documented.Multivariate logistic regression was employed to investigate variables related to malignancy.Survival analysis was performed using the Kaplan-Meier curve,and the prognostic factors were assessed to evaluate the postoperative course of patients with MCN-AIC.RESULTS Among the 48 patients,36 had benign MCNs,and 12 had malignant MCNs(1 high-grade atypical hyperplasia and 11 MCN-AIC).Age,tumour size,presence of solid components or mural nodules and pancreatic duct dilatation were identified as independent risk factors associated with malignancy.The follow-up period ranged from 12 mo to 120 mo,with a median overall survival of 58.2 mo.Only three patients with MCN-AIC died,and the 5-year survival rate was 70.1%.All 11 cases of MCN-AIC were stage I,and extracapsular invasion was identified as a prognostic factor for poorer outcomes.CONCLUSION The risk factors independently associated with malignant transformation of MCNs included age,tumour size,presence of solid components or mural nodules,and pancreatic duct dilatation.Our study also revealed that encapsulated invasion was a favourable prognostic factor in MCN-AIC patients.

Duodenum and ventral pancreas preserving subtotal pancreatectomy for low-grade malignant neoplasms of the pancreas: An alternative procedure to total pancreatectomy for low-grade pancreatic neoplasms

AIM To describe the indications, technique and outcomes of the novel surgical procedure of duodenum and ventral pancreas preserving subtotal pancreatectomy(DVPPSP).METHODS Data collected retrospectively from 43 patients who underwent DVPPSP and TP between 2009 and 2015 in our single centre were analysed. For enrolment, only patients with low-grade pancreatic neoplasms, such as pancreatic neuroendocrine tumors, intraductal papillary mucinous neoplasms(IPMNs), and solid pseudopapillary tumors, were included. Ten DVPPSP(group 1) and 13 TP(group 2) patients were selected in this study.RESULTS There were no significant differences in age, gender, comorbidities, preoperative symptoms, American Society of Anesthesiologists score or indications for surgery between the two groups. The most common indication was IPMN for DVPPSP and TP(60% vs 85%, P = 0.411). Compared with the TP group, the DVPPSP group had comparable postoperative morbidities(P = 0.405) and mortalities(both nil), but significantly shorter operative time(232 ± 19.6 min vs 335 ± 32.3 min, P < 0.001). DVPPSP preserved better long-term pancreatic function with less supplementary therapy(P < 0.001) and better quality of life(Qo L) after surgery, including better scores in social(P = 0.042) and global health(P = 0.047) on functional scales and less appetite loss(P = 0.049) on the symptom scale. CONCLUSION DVPPSP is a feasible and safe procedure that could be an alternative to TP for low-grade neoplasms arising from the body and tail region but across the neck region of the pancreas; DVPPSP had better metabolic function and Qo L after surgery.

A new combined criterion to better predict malignant lesions in patients with pancreatic cystic neoplasms

Objective:Cystic lesions of the pancreas have been increasingly recognized.Some lesions exhibit benign behavior,while others have unequivocal malignant potential.Thus,accurate identification of malignancy in patients diagnosed with pancreatic cystic neoplasms(PCNs)remains a major challenge.The aim of this study was to define a combined criterion to better predict malignant lesions in patients with PCNs.Methods:We retrospectively analyzed 165 patients who underwent resection of PCNs from October 2011 to May 2017.The relationship among malignancy and serum carbohydrate antigen 19-9(CA19-9),preoperative neutrophil-to-lymphocyte ratio(NLR),and the presence of enhanced solid component on imaging was analyzed.Results:NLR before surgery in patients with malignant PCNs(2.81±2.14)was significantly higher than that in patients diagnosed with pancreatic neuroendocrine tumor(1.90±0.69,P=0.013)or healthy volunteers(1.40±0.48;P<0.001).Serum CA19-9≥39U/m L,NLR>1.976 and presence of enhanced solid component were independent predictors of PCN malignancy.A combined criterion meeting any two or more of the three elements including CA19-9≥39 U/m L,NLR>1.976,and presence of enhanced solid component on computed tomography imaging is an indicator with a high positive predictive value of 80.5%and a high negative predictive value of 87.9%,and thus,represents a highly accurate test(86.1%).Conclusions:The new combined criterion is an effective predictor of tumor malignancy in patients with PCNs.

Preoperative ultrasound combined with routine blood tests in predicting the malignant risk of pancreatic cystic neoplasms

Objective:Accurate preoperative identification of benign or malignant pancreatic cystic neoplasms(PCN)may help clinicians make better intervention choices and will be essential for individualized treatment.Methods:Preoperative ultrasound and laboratory examination findings,and demographic characteristics were collected from patients.Multiple logistic regression was used to identify independent risk factors associated with malignant PCN,which were then included in the nomogram and validated with an external cohort.The Net Reclassification Index(NRI)and Integrated Discrimination Improvement(IDI)were calculated to evaluate the improvement in the predictive power of the new model with respect to that of a combined imaging and tumor marker prediction model.Results:Malignant PCN were found in 83(40.7%)and 33(38.7%)of the model and validation cohorts,respectively.Multivariate analysis identified age,tumor location,imaging of tumor boundary,blood type,mean hemoglobin concentration,neutrophil-tolymphocyte ratio,carbohydrate antigen 19-9,and carcinoembryonic antigen as independent risk factors for malignant PCN.The calibration curve indicated that the predictions based on the nomogram were in excellent agreement with the actual observations.A nomogram score cutoff of 192.5 classified patients as having low vs.high risk of malignant PCN.The model achieved good C-statistics of 0.929(95%CI 0.890–0.968,P<0.05)and 0.951(95%CI 0.903–0.998,P<0.05)in predicting malignancy in the development and validation cohorts,respectively.NRI=0.268;IDI=0.271(P<0.001 for improvement).The DCA curve indicated that our model yielded greater clinical benefits than the comparator model.Conclusions:The nomogram showed excellent performance in predicting malignant PCN and may help surgeons select patients for detailed examination and surgery.The nomogram is freely available at https://wangjunjinnomogram.shinyapps.io/DynNomapp/.

Spatial autocorrelation analysis of 13 leading malignant neoplasms in Taiwan: a comparison between the 1995-1998 and 2005-2008 periods

Spatial autocorrelation methodologies, including Global Moran’s I and Local Indicators of Spatial Association statistic (LISA), were used to describe and map spatial clusters of 13 leading malignant neoplasms in Taiwan. A logistic regression fit model was also used to identify similar characteristics over time. Two time periods (1995-1998 and 2005-2008) were compared in an attempt to formulate common spatio-temporal risks. Spatial cluster patterns were identified using local spatial autocorrelation analysis. We found a significant spatio-temporal variation between the leading malignant neoplasms and well-documented spatial risk factors. For instance, in Taiwan, cancer of the oral cavity in males was found to be clustered in locations in central Taiwan, with distinct differences between the two time periods. Stomach cancer morbidity clustered in aboriginal townships, where the prevalence of Helicobacter pylori is high and even quite marked differences between the two time periods were found. A method which combines LISA statistics and logistic regression is an effective tool for the detection of space-time patterns with discontinuous data. Spatio-temporal mapping comparison helps to clarify issues such as the spatial aspects of both two time periods for leading malignant neoplasms. This helps planners to assess spatio-temporal risk factors, and to ascertain what would be the most advantageous types of health care policies for the planning and implementation of health care services. These issues can greatly affect the performance and effectiveness of health care services and also provide a clear outline for helping us to better understand the results in depth.

A clinical trial of cytokine induced killer cells treating patients with malignant tumor after radiochemotherapy

Objective: Our study investigated the immunity changes and life quality changes after the treatment of cytokine induced killer (CIK) cells for patients with malignant tumor after radiochemotherapy, and explored the therapeutic effects of CIK cells on these patients. Methods: Thirty-one patients with malignant tumor after radiochemotherapy were treated with CIK cells. Before and after CIK cells being transfused back, the immunity indexes of the peripheral blood of these cases were detected and the changes of life quality of these cases were compared. Results: After radiochemotherapy, the percentage of CD3+, CD4+ cells declined, the percentage of CD8+ cells rose; the ratio of CD4+/CD8+ declined, and the percentage of CD16+, CD56+ cells declined. As all the above indexes compared with that of normal people, the difference was significant (P < 0.05). After CIK cells therapy, the above indexes improved (P < 0.05). Life quality improved significantly after CIK cells therapy (P < 0.05). Conclusion: Radiochemotherapy can inhibit the immunity in patients with malignant tumor. CIK cells therapy is safe and effective. It may improve the recent immunity and life quality of the patients, which suggesting that it may be an alternative maintenance treatment for patients with malignant tumor after radiochemotherapy.

Spatial autocorrelation calculations of the nine malignant neoplasms in Taiwan in 2005-2009: a gender comparison study

Spatial analytical techniques and models are often used in epidemiology to identify spatial anomalies (hotspots) in disease regions. These analytical approaches can be used to identify not only the location of such hotspots, but also their spatial patterns. We used spatial autocorrelation methodologies, including Global Moran's I and Local Getis-Ord statistics, to describe and map spatial clusters and areas in which nine malignant neoplasms are situated in Taiwan. In addition, we used a logistic regression model to test the characteristics of similarity and dissimilarity between males and females and to formulate the common spatial risk. The mean found by local spatial autocorrelation analysis was used to identify spatial cluster patterns. We found a significant relationship between the leading malignant neoplasms and well-documented spatial risk factors. For instance, in Taiwan, the geographic distribution of clusters where oral cavity cancer in males is prevalent was closely correspond to the locations in central Taiwan with serious metal pollution. In females, clusters of oral cavity cancer were closely related with aboriginal townships in eastern Taiwan, where cigarette smoking, alcohol drinking, and betel nut chewing are commonplace. The difference between males and females in the spatial distributions was stark. Furthermore, areas with a high morbidity of gastric cancer were clustered in aboriginal townships where the occurrence of Helicobacter pylori is frequent. Our results revealed a similarity between both males and females in spatial pattern. Cluster mapping clarified the spatial aspects of both internal and external correlations for the nine malignant neoplasms. In addition, using a method of logistic regression also enabled us to find differentiation between gender-specific spatial patterns.

^(18)F-FDG PET/CT for malignant small intestinal neoplasms

In this paper, 18F-FDG PET/CT data of 19 malignant SINs (small intestinal neoplasms) were consecutively reviewed. Nnine patients accepted PET/CT scan for preoperative diagnosis and staging, while ten patients presented follow-up after treatment and restaging. The results were correlated with abdomen enhanced CT and surgical pathological findings. Abdominal pain and weight loss were the most common findings. About 16% SINs located in the duodenum, 52% in the jejunum and 32% in the ileum. Lymphoma was the most frequent neoplasms. PET/CT revealed residual or recurrent malignant SINs in 5 patients who had negative or non-definite findings by abdomen CT and demonstrated extra-abdomen metastasis in 3 patients. Clinical decisions of treatment were changed for 6 patients after PET/CT examinations. The 18F-FDG PET/CT were better than CT in accuracy, negative predictive value and positive predictive value (89.5% vs 68.4%, 100% vs 66.7%, and 81.8% vs 69.2%, respectively). Whole body 18F-FDG PET/CT may be an effective molecular imaging method for staging and restaging of malignant SINs.

Advancing the understanding and management of blastic plasmacytoid dendritic cell neoplasm:Insights from recent case studies

We specifically discuss the mechanisms of the pathogenesis,diagnosis,and management of blastic plasmacytoid dendritic cell neoplasm(BPDCN),a rare but aggressive haematologic malignancy characterized by frequent skin manifestations and systemic dissemination.The article enriches our understanding of BPDCN through detailed case reports showing the clinical,immunophenotypic,and histopathological features that are critical for diagnosing this disease.These cases highlight the essential role of pathologists in employing advanced immunophenotyping techniques to accurately identify the disease early in its course and guide treatment decisions.Furthermore,we explore the implications of these findings for management strategies,emphasizing the use of targeted therapies such as tagraxofusp and the potential of allogeneic haematopoietic stem cell transplantation in achieving remission.The editorial underscores the importance of interdisciplinary approaches in managing BPDCN,pointing towards a future where precision medicine could significantly improve patient outcomes.

Primitive Neuroectodermal Tumor as the Second Malignant Neoplasm in a Long-Term Survivor Child of Acute Lymphoblastic Leukemia: A Case Report

Acute lymphoblastic leukemia(ALL)is a common pediatric cancer.The second malignant neoplasms(SMNs)in long-term survivors of pediatric ALL are relatively rare.Herein we report a 10-year-old girl who was diagnosed as primitive neuroectodermal tumor(PNET)5 years after the initial diagnosis of ALL with radiotherapy・free treatment.PNET is an exceedingly rare neoplasm in SMNs of survivors of childhood ALL.It is predisposed to be misdiagnosed and the pathogenesis is unclear.The outcome is poor.Long-term follow-up is necessary for the survival children of ALL.

Endoscopic stenting for inoperable malignant biliary obstruction: A systematic review and meta-analysis

AIM: To analyze through meta-analyses the benefits of two types of stents in the inoperable malignant biliary obstruction.METHODS: A systematic review of randomized clinical trials(RCT) was conducted, with the last update on March 2015, using EMBASE, CINAHL(EBSCO), MEDLINE, LILACS/CENTRAL(BVS), SCOPUS, CAPES(Brazil), and gray literature. Information of the selected studies was extracted in sight of six outcomes: primarily regarding dysfunction, complication and reintervention rates; and secondarily costs, survival, and patency time. The data about characteristics of trial participants, inclusion and exclusion criteria and types of stents were also extracted. The bias was mainly assessed through the JADAD scale. This meta-analysis was registered in the PROSPERO database by the number CRD42014015078. The analysis of the absolute risk of the outcomes was performed using the software Rev Man, by computing risk differences(RD) of dichotomous variables and mean differences(MD) of continuous variables. Data on RD and MD for each primary outcome were calculated using the MantelHaenszel test and inconsistency was qualified and reported in χ2 and the Higgins method(I2). Sensitivity analysis was performed when heterogeneity was higher than 50%, a subsequent assay was done and other findings were compiled. Student's t-test was used for the comparison of weighted arithmetic means regarding secondary outcomes.RESULTS: Initial searching identified 3660 studies; 3539 were excluded through title, repetition, and/or abstract, while 121 studies were fully assessed and were excluded mainly because they did not compare self-expanding metal stents(SEMS) and plastic stents(PS), leading to thirteen RCT selected, with 13 articles and 1133 subjects meta-analyzed. The mean age was 69.5 years old, that were affected mostly by bile duct(proximal) and pancreatic tumors(distal). The preferred SEMS diameter used was the 10 mm(30 Fr) and the preferred PS diameter used was 10 Fr. In the metaanalysis, SEMS had lower overall stent dysfunction compared to PS(21.6% vs 46.8%, P < 0.00001) and fewer re-interventions(21.6% vs 56.6%, P < 0.00001), with no difference in complications(13.7% vs 15.9%, P = 0.16). In the secondary analysis, the mean survival rate was higher in the SEMS group(182 d vs 150 d, P < 0.0001), with a higher patency period(250 d vs 124 d, P < 0.0001) and a lower cost per patient(4193.98 vs 4728.65 Euros, P < 0.0985).CONCLUSION: SEMS are associated with lower stent dysfunction, lower re-intervention rates, better survival, and higher patency time. Complications and costs showed no difference.

Malignant solitary fibrous tumor involving the liver

Solitary fibrous tumors are predominantly benign and are most commonly found in the thoracic cavity and pleura; while reports exist in the literature of malignant solitary fibrous tumors and those located in extrathoracic organs, these cases are considered extremely rare. Herein, a case is reported of a malignant solitary fibrous tumor involving the liver that was diagnosed and treated in a 62-year-old woman. The patient presented with complaints of upper abdominal pain and unintentional weight loss. Computed tomography scan of the abdomen revealed a remarkably large mass, measuring 15 cm × 10 cm × 20 cm, which appeared to be unrelated to any particular organ. The intraoperative finding of a wide communication with the left liver suggested hepatic origin, and served as an indicator for tumor resection via left hemihepatectomy. The diagnosis of solitary fibrous tumor and its malignant nature was confirmed by histological and immunohistochemical examination of the resected tissues. Hepatic solitary fibrous tumor is very rare, and surgery remains the mainstay of treatment. Due to limited reports of such tumors in the literature, little can be said about the benefit of adjuvant therapy and prognosis for the rare cases with malignant histological findings.

Extrapancreatic malignancies and intraductal papillary mucinous neoplasms of the pancreas

Over the last two decades multiple studies have demonstrated an increased incidence of additional malignancies in patients with intraductal papillary mucinous neoplasms(IPMNs).Additional malignancies have been identified in 10%-52% of patients with IPMNs.The majority of these additional cancers occur before or concurrent with the diagnosis of IPMN.The gastrointestinal tract is most commonly involved in secondary malignancies,with benign colon polyps and colon cancer commonly seen in western countries and gastric cancer commonly seen in Asian countries.Other extrapancreatic malignancies associated with IPMNs include benign and malignant esophageal neoplasms,gastrointestinal stromal tumors,carcinoid tumors,hepatobiliary cancers,breast cancers,prostate cancers,and lung cancers.There is no clear etiology for the development of secondary malignancies in patients with IPMN.Although population-based studies have shown different results from single institution studies regarding the exact incidence of additional primary cancers in IPMN patients,both have reached the same conclusion:there is a higher incidence of extrapancreatic malignancies in patients with IPMNs than in the general population.This f inding has signif icant clinical implications for both the initial evaluation and the subsequent long-term followup of patients with IPMNs.If a patient has not had recent colonoscopy,this should be performed during the evaluation of a newly diagnosed IPMN.Upper endoscopy should be performed in patients from Asian countries or for those who present with symptoms suggestive of upper gastrointestinal disease.Routine screening studies(breast and prostate) should be carried out as currently recommended for patient's age both before and after the diagnosis of IPMN.

Co-occurrence of IPMN and malignant IPNB complicated by a pancreatobiliary fistula: A case report and review of the literature

BACKGROUND Intraductal papillary neoplasm of the bile duct(IPNB) is pathologically similar to intraductal papillary mucinous neoplasm(IPMN). However, there are several significant differences between them. The rate of IPMN associated with extrapancreatic malignancies has been reported to range from 10%-40%, and it may occasionally be complicated with the presence of fistulas. IPMN associated with malignant IPNB is extremely rare and only nine cases have been reported in the literature.CASE SUMMARY We report a 52-year-old man who presented with recurrent cholangitis for nine months. Computed tomography and magnetic resonance cholangiopancreatography showed the common bile duct stricture with dilated pancreatobiliary duct without other abnormal findings. The underlying pathogenesis could not be identified based on the radiologic images. Endoscopic retrograde cholangiopancreatography revealed a pancreatobiliary fistula with dilated main pancreatic duct, biliary stricture with dilated biliary tree, and mucus discharge from the enlarged orifice of the major papilla. The patient underwent SpyGlass cholangiopancreatoscopy due to a suspected mucin-producing biliary neoplasm and indeterminate main pancreatic duct dilatation. Multiple papillary growing neoplasms with vascular images, with the extent of lesions spreading in the biliopancreatic ductal lumens, were identified by SpyGlass. In addition, the presence of a pancreatobiliary fistula was also identified. The patient was diagnosed as having benign IPMN and malignant IPNB with focal invasion by postoperative pathology. Furthermore, varying histological subtypes were present in both IPMN and IPNB. Pylorus-preserving pancreaticoduodenectomy was performed on the patient with excellent results during the 52 month followup period.CONCLUSION We deemed that pancreatography and SpyGlass allowed for an efficient diagnosis of IPMN with pancreatobiliary fistula, whereas the etiology could not be identified by radiologic imaging.

A case of primary malignant fibrous histiocytoma of the pancreas: CT and MRI findings

Primary malignant fibrous histiocytoma (MFH) of the pancreas is rare and a distinct clinical entity. We report a case of recurrence of pancreatic MFH with computed tomography (CT) and magnetic resonance imaging (MRI) findings. A 67-year-old man presented with a history of decreased body weight over the past 6 mo. Abdominal CT revealed a large, multilocular cystic mass in the head of the pancreas with obvious atrophy in the body and tail of the pancreas. After 6 mo postoperatively, MRI demonstrated a recurrent large mass in the primary area of the head of the pancreas. The lesion was heterogeneous, hypointense to the liver on T1-weighted imaging, and heterogeneously hyperintense to the liver with a hypointense area in the central part of the tumor on fat-saturated T2-weighted imaging. Contrast- enhanced T1-weighted imaging demonstrated a large multilocular cystic mass with a cystic wall, fibrous septa and enhancement of solid components. To the best of our knowledge, this is the first report on recurrence of primary MFH of the pancreas, and the first with MRI findings.

Primary intestinal malignant fibrous histiocytoma:two case reports

Malignant fibrous histiocytoma (MFH) occurs most commonly in the extremities and trunk, but rarely in the intestine. Here we report two cases of primary intestinal MFH. The first case was a 70-year old man admitted for recurrent right lower quadrant abdominal pain. At laparotomy, a tumor was found originating from the cecum, with a suspicious metastatic nodule on the surface of the right lobe of the liver. A right hemicolectomy was performed followed by an ileotransverse end-to-end anastomotic reconstruction. The second case was a 43-year old man with intussusceptions of the small intestine. An emergent laparotomy revealed 4 pedunculated masses in the small bowel and a partial resection of the small intestine was performed. Though the symptoms were not typical, based on histological and immunohistochemical studies, the patients were diagnosed as MFH of the intestine. They were not treated with chemotherapy or radiotherapy and both died within 3 mo. MFH of the intestine is an extremely rare neoplasm with an aggressive biological behavior. The pathogenesis of this disease has not been clarified to date. Complete surgical excision is preferred, adjuvant chemotherapy or radiotherapy may be advisable.