BACKGROUND Liposarcomas(LPSs)are malignant mesenchymal tumors originating from adipocytes.Myxoid LPS(MLPS),a common subtype,predominantly arises in the extremities,retroperitoneum,and deep soft tissues,with a rare occ...BACKGROUND Liposarcomas(LPSs)are malignant mesenchymal tumors originating from adipocytes.Myxoid LPS(MLPS),a common subtype,predominantly arises in the extremities,retroperitoneum,and deep soft tissues,with a rare occurrence in the gastrointestinal tract.Primary mesenteric LPS is particularly uncommon,especially in the transverse colonic mesentery.CASE SUMMARY This report describes the case of a 65-year-old female patient who presented with abdominal distension and was diagnosed with a giant mucinous LPS of the transverse colonic mesentery.Upon admission,the patient underwent a comprehensive evaluation.Contrast-enhanced computed tomography(CT)of the chest and abdomen revealed a large malignant tumor with aortic dissection,while colonoscopy identified rectal cancer.Given the patient's condition and surgical risk,an interventional procedure was first performed to manage the aortic coarctation,followed by tumor resection via laparotomy.Intraoperative and histopathological findings confirmed a giant mucinous LPS originating from the transverse colon mesentery.Postoperatively,the patient underwent chemotherapy and regular follow-up CT.Although the rectal tumor did not recur,the mesenteric tumor showed extensive recurrence,compressing the intestinal lumen and causing mechanical obstruction,which severely threatened the patient's life.A second operation temporarily relieved the obstructive symptoms;however,it had a limited effect on tumor progression.The patient died four months later due to disease progression.CONCLUSION While no standardized treatment exists for MLPS co-occurring with multiple diseases,operation remains the mainstay.However,recurrence,metastasis,and poor postoperative prognosis continue to pose serious threats to patient survival.展开更多
Appendiceal mucinous neoplasms(AMNs)are rare tumors originating from mucin-producing epithelial cells of the appendix.They can exhibit both benign and malignant behavior.They are often incidentally discovered during a...Appendiceal mucinous neoplasms(AMNs)are rare tumors originating from mucin-producing epithelial cells of the appendix.They can exhibit both benign and malignant behavior.They are often incidentally discovered during appendectomy.Clinical presentation ranges from asymptomatic to mimicking acute appendicitis.Histologically,noninvasive AMNs are classified as low-grade AMNs(LAMNs)or high-grade AMNs(HAMNs),whereas invasive tumors are categorized as mucinous adenocarcinomas.Although LAMNs and HAMNs are generally nonmalignant,rupture can lead to pseudomyxoma peritonei(PMP).Surgical resection is the primary diagnostic and therapeutic approach,with intraoperative assessment to prevent rupture.Treatment strategies vary based on findings and include appendectomy,right hemicolectomy,and cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.Histological diagnosis relies on mucin detection,and immunohistochemical markers such as cytokeratin 20(diffusely positive),cytokeratin 7(often negative),mucin 5AC,and special ATrich sequence-binding protein 2 assist in characterization.Molecular profiling frequently identifies KRAS,GNAS,and TP53 mutations.KRAS mutations are generally associated with a favorable prognosis,whereas GNAS and TP53 mutations correlate with poorer survival outcomes.These findings highlight the potential role of molecular profiling in guiding treatment strategies for AMN and PMP.展开更多
BACKGROUND Low-grade appendiceal mucinous neoplasms(LAMNs)are a class of histolo-gically well-differentiated adenomas that can proliferate outside the appendix in the form of malignant tumours,resulting in the accumul...BACKGROUND Low-grade appendiceal mucinous neoplasms(LAMNs)are a class of histolo-gically well-differentiated adenomas that can proliferate outside the appendix in the form of malignant tumours,resulting in the accumulation of external appen-diceal mucus.They may present as an extra-appendiceal mass-like bulge,mucus or polypoid tissue at the appendiceal orifice,or even with abdominal dissem-ination.The surgical approach is usually appendectomy,ileocecal resection or right hemicolectomy.It is essential to raise awareness of LAMNs and choose an appropriate treatment.CASE SUMMARY A 68-year-old man underwent a routine physical examination at our hospital on May 17,2022.The patient had no symptoms of abdominal pain,bloating,or weight loss,and his tumour marker levels were normal.The faecal occult blood test was negative,and no abnormalities were identified on physical examination.Colonoscopy revealed a submucosal protrusion at the appendiceal orifice.During endoscopic ultrasound,uneven echoes were observed in the appendix cavity.A contrast-enhanced computed tomography scan of the abdomen revealed a nodular thickening at the base of the appendix,with a diameter of approximately 1 cm.When all the examination results were considered,we suspected the lesion to be an appendiceal mucinous tumour.Laparoscopic surgery was performed using the double purse-string suture method.Postoperative pathology suggested a low-grade mucinous cystadenoma of the appendix with no involvement of the margins.A repeat colonoscopy 18 months after surgery revealed no significant abnormality at the appendiceal orifice.A contrast-enhanced computed tomography scan of the abdomen suggested only postoperative changes.CONCLUSION This study describes a case of LAMN that was treated by resecting the mass at the appendiceal orifice via the double purse-string suture technique and provides new insights on the diagnosis and treatment of LAMNs.展开更多
Mucinous cystic neoplasms of the liver(MCN-L)are rare cystic lesions characterized by mucin-producing epithelium and ovarian-like stroma.Although they constitute fewer than 5%of hepatic cystic lesions,MCN-L poses sign...Mucinous cystic neoplasms of the liver(MCN-L)are rare cystic lesions characterized by mucin-producing epithelium and ovarian-like stroma.Although they constitute fewer than 5%of hepatic cystic lesions,MCN-L poses significant diagnostic challenges due to overlapping features with other cystic lesions and their potential for malignant transformation.Early recognition and definitive surgical intervention are therefore critical to ensure optimal patient outcomes.A literature review was conducted to summarize epidemiology,clinical presentation,diagnostic modalities,and management strategies for MCN-L.Additionally,from 2019 to 2025,9 patients with MCN-L were identified at our center.Clinical data and outcomes were retrospectively analyzed.MCN-L predominantly affects middle-aged women and presents as large,multiloculated cystic lesions without biliary communication.The revised 2010 World Health Organization classification emphasizes the presence of ovarian-like stroma for definitive diagnosis.Contrast-enhanced computed tomography or magnetic resonance imaging are often suggestive but not pathognomonic,reinforcing the need for histopathological confirmation.MCN-L remains a diagnostic and therapeutic challenge due to its resemblance to other cystic liver lesions.Complete surgical resection is the treatment of choice to prevent recurrence and malignant transformation,reinforcing the importance of early intervention.Further research is needed to improve diagnostic accuracy and refine management strategies.展开更多
BACKGROUND Pancreatic neuroendocrine neoplasms(pNENs)are rare,heterogeneous tumors accounting for 1%-2%of pancreatic tumors,with significant malignant potential.Intraductal papillary mucinous neoplasm of the bile duct...BACKGROUND Pancreatic neuroendocrine neoplasms(pNENs)are rare,heterogeneous tumors accounting for 1%-2%of pancreatic tumors,with significant malignant potential.Intraductal papillary mucinous neoplasm of the bile duct(IPMN-B)is a rare precancerous lesion in the bile duct system,with potential for malignancy.The combination of pNENs and IPMN-B is exceptionally rare and often leads to misdiagnosis.This study aims to report a rare case of pNENs combined with IPMN-B treated at Yanbian University Hospital to improve understanding and management of this unusual tumor combination.CASE SUMMARY We retrospectively analyzed a case from Yanbian University Hospital.We re-viewed clinical records,imaging findings,endoscopic retrograde cholangiopan-creatography,surgical exploration,and histopathological examination.The pa-tient was diagnosed with pNENs and IPMN-B.Surgical treatment was performed,with follow-up showing effective management and no significant recurrence.CONCLUSION This case represents the first report of pNENs combined with IPMN-B.It high-lights the need for thorough diagnostic evaluation to prevent misdiagnosis and improve treatment strategies.展开更多
Intraductal papillary mucinous neoplasm(IPMN)and intraductal papillary neoplasm of the bile duct(IPNB)are mucinous cystic tumors with intraductal papillary growth and malignant potential.Their concurrent occurrence is...Intraductal papillary mucinous neoplasm(IPMN)and intraductal papillary neoplasm of the bile duct(IPNB)are mucinous cystic tumors with intraductal papillary growth and malignant potential.Their concurrent occurrence is exceptionally rare.CASE SUMMARY A 58-year-old Chinese man presented with recurrent upper abdominal pain.Imaging and laboratory tests revealed lesions consistent with IPNB and IPMN.Postoperative pathological examination confirmed IPNB with high-grade dysplasia and main-duct type IPMN with low-grade dysplasia.The patient underwent extrahepatic bile duct resection with Roux-en-Y choledochoenterostomy and distal pancreatectomy.He had an excellent prognosis with no tumor recurrence during the 30-month follow-up.CONCLUSION This case emphasizes the importance of comprehensive preoperative assessment and individualized management for these complex tumors.Further research is needed to understand their pathogenesis and improve treatment strategies.展开更多
Intraductal papillary mucinous neoplasm(IPMN) of the pancreas is a noninvasive epithelial neoplasm of mucinproducing cells arising in the main duct(MD) and/or branch ducts(BD) of the pancreas.Involved ducts are dilate...Intraductal papillary mucinous neoplasm(IPMN) of the pancreas is a noninvasive epithelial neoplasm of mucinproducing cells arising in the main duct(MD) and/or branch ducts(BD) of the pancreas.Involved ducts are dilated and filled with neoplastic papillae and mucus in variable intensity.IPMN lacks ovarian-type stroma,unlike mucinous cystic neoplasm,and is defined as a grossly visible entity(≥ 5 mm),unlike pancreatic intraepithelial neoplasm.With the use of high-resolution imaging techniques,very small IPMNs are increasingly being identified.Most IPMNs are solitary and located in the pancreatic head,although 20%-40% are multifocal.Macroscopic classification in MD type,BD type and mixed or combined type reflects biological differences with important prognostic and preoperative clinical management implications.Based on cytoarchitectural atypia,IPMN is classified into low-grade,intermediategrade and high-grade dysplasia.Based on histological features and mucin(MUC) immunophenotype,IPMNs are classified into gastric,intestinal,pancreatobiliary and oncocytic types.These different phenotypes can be observed together,with the IPMN classified according to the predominant type.Two pathways have been suggested:gastric phenotype corresponds to less aggressive uncommitted cells(MUC1-,MUC2-,MUC5 AC +,MUC6 +) with the capacity to evolve to intestinal phenotype(intestinal pathway)(MUC1-,MUC2 +,MUC5 AC +,MUC6- or weak +) or pancreatobiliary /oncocytic phenotypes(pyloropancreatic pathway)(MUC1 +,MUC 2-,MUC5 AC +,MUC 6 +) becoming more aggressive.Prognosis of IPMN is excellent but critically worsens when invasive carcinoma arises(about 40% of IPMNs),except in some cases of minimal invasion.The clinical challenge is to establish which IPMNs should be removed because of their higher risk of developing invasive cancer.Once resected,they must be extensively sampled or,much better,submitted in its entirety for microscopic study to completely rule out associated invasive carcinoma.展开更多
BACKGROUND Appendiceal mucinous neoplasms(AMNs),although not classified as rare,are relatively uncommon tumors most often discovered incidentally during colorectal surgery.Accurate identification of AMNs is difficult ...BACKGROUND Appendiceal mucinous neoplasms(AMNs),although not classified as rare,are relatively uncommon tumors most often discovered incidentally during colorectal surgery.Accurate identification of AMNs is difficult due to non-specific sym-ptoms,overlapping tumor markers with other conditions,and the potential for misdiagnosis.This underscores the urgent need for precision in diagnosis to pre-vent severe complications.CASE SUMMARY This case report describes the unexpected discovery and treatment of a low-grade AMN(LAMN)in a 74-year-old man undergoing laparoscopic hemicolectomy for transverse colon adenocarcinoma(AC).Preoperatively,non-specific gastroin-testinal symptoms and elevated tumor markers masked the presence of AMN.The tumor,presumed to be an AMN peritoneal cyst intraoperatively,was con-firmed as LAMN through histopathological examination.The neoplasm exhibited mucin accumulation and a distinct immunohistochemical profile:Positive for Ho-meobox protein CDX-2,Cytokeratin 20,special AT-rich sequence-binding protein 2,and Mucin 2 but negative for cytokeratin 7 and Paired box gene 8.This profile aids in distinguishing appendiceal and ovarian mucinous tumors.Postoperative recovery was uncomplicated,and the patient initiated adjuvant chemotherapy for the colon AC.CONCLUSION This case highlights the diagnostic complexity of AMNs,emphasizing the need for vigilant identification to avert potential complications,such as pseudomyxoma peritonei.展开更多
Background: Biliary mucinous cystic neoplasms(BMCNs) are rare hepatobiliary cystic tumors, which can be divided into noninvasive and invasive types. This study aimed to investigate the diagnosis, treatment, and progno...Background: Biliary mucinous cystic neoplasms(BMCNs) are rare hepatobiliary cystic tumors, which can be divided into noninvasive and invasive types. This study aimed to investigate the diagnosis, treatment, and prognosis of BMCNs in a large single center. Methods: We analyzed 49 patients with BMCNs confirmed by postoperative pathology at the First Afflliated Hospital, Zhejiang University School of Medicine between January 2007 and December 2021. Results: Among the 49 patients, 37 were female(75.5%), and the average age was 57.04 years. Common symptoms included abdominal discomfort, jaundice and fever, while 22 patients(44.9%) had no symptoms. Serum carbohydrate antigen(CA) 19-9 and CA125 concentrations were elevated in 34.8% and 19.6% of patients, respectively. Forty-eight patients had tumors in the intrahepatic bile ducts and only one had a tumor in the extrahepatic bile duct. Forty-eight patients with noninvasive intrahepatic BMCNs were further analyzed in terms of pathological features: 34(70.8%) had low-grade intraepithelial neoplasms(LGINs), and 14(29.2%) had high-grade intraepithelial neoplasms(HGINs). The potential immunohistochemical markers of BMCNs were cytokeratin(CK) 19, CK7, estrogen receptor and progesterone receptor. Follow-up data for 37 patients with intrahepatic BMCNs were obtained. The median overall survival(OS) of BMCNs was not reached. The longest survival time was 137 months.The 5-and 10-year OS rates were 100% and 85.4%, respectively. The 5-and 10-year recurrence-free survival(RFS) rates were 93.9% and 80.2%, respectively. Conclusions: BMCNs are rare cystic neoplasms that commonly occur in middle-aged females. BMCNs can only be diagnosed and classified by postoperative pathology, as there are no specific clinical presentations, serological indicators or imaging modalities for preoperative diagnosis. Complete surgical resection is necessary for BMCNs, and the postoperative prognosis is favorable.展开更多
BACKGROUND Low-grade appendiceal neoplasms(LAMN)are characterized by low incidence and atypical clinical presentations,often leading to misdiagnosis as acute or chronic appendicitis before surgery.The primary diagnost...BACKGROUND Low-grade appendiceal neoplasms(LAMN)are characterized by low incidence and atypical clinical presentations,often leading to misdiagnosis as acute or chronic appendicitis before surgery.The primary diagnostic tool for LAMN is abdominal computed tomography(CT)imaging.Surgical resection remains the cornerstone of LAMN management,necessitating en bloc tumor excision to minimize the risk of iatrogenic rupture.Laparoscopy,known for its minimal invasiveness,reduced postoperative discomfort,and expedited recovery,is a safe and reliable approach for LAMN treatment.Despite the possibility of pseudomyxoma peritonei development,appendectomy and partial appendectomy generally result in negative tumor margins and favorable outcomes,which can be attributed to the disease’s slow growth and lower malignancy.CASE SUMMARY A 71-year-old male patient was admitted to our hospital with a pelvic spaceoccupying lesion detected 1 mo prior.Physical examination showed a soft abdomen without tenderness or rebound and no palpable masses.No shifting dullness was noted,and digital rectal examination revealed no palpable mass.Enteroscopy revealed a raised,smooth-surfaced mass measuring 3.0 cm in the cecum.Abdominal contrast-enhanced CT showed a markedly thickened and dilated appendix with visible cystic shadows.Laparoscopic surgery was performed and revealed a significantly dilated appendix,leading to laparoscopic resection of the appendix and part of the cecum.Post-surgical pathologic analysis confirmed LAMN.The patient received symptomatic and supportive post-operative care and was discharged on postoperative day 4 without complications such as abdominal bleeding,intestinal obstruction,or incision infection.No tumor recurrence was observed during a 7-mo follow-up period.CONCLUSION LAMN is a rare disease that lacks specific clinical manifestations.Abdominal CT plays a crucial role in diagnosing LAMN,and laparoscopic surgery is a safe and effective diagnostic and therapeutic approach.展开更多
BACKGROUND Rectal mucinous adenocarcinoma(MAC)is a rare pathological type of rectal can-cer with unique pathological features and a poor prognosis.It is difficult to diag-nose and treat early because of the lack of sp...BACKGROUND Rectal mucinous adenocarcinoma(MAC)is a rare pathological type of rectal can-cer with unique pathological features and a poor prognosis.It is difficult to diag-nose and treat early because of the lack of specific manifestations in some aspects of the disease.The common metastatic organs of rectal cancer are the liver and lung;however,rectal carcinoma with metastasis to subcutaneous soft tissue is a rare finding.CASE SUMMARY In this report,the clinical data,diagnosis and treatment process,and postope-rative pathological features of a patient with left waist subcutaneous soft tissue masses were retrospectively analyzed.The patient underwent surgical treatment after admission and recovered well after surgery.The final pathological diagnosis was rectal MAC with left waist subcutaneous soft tissue metastasis.CONCLUSION Subcutaneous soft tissue metastasis of rectal MAC is rare,and it can suggest that the tumor is disseminated,and it can appear even earlier than the primary ma-lignant tumor,which is occult and leads to a missed diagnosis and misdiagnosis clinically.When a subcutaneous soft tissue mass of unknown origin appears in a patient with rectal cancer,a ma-lignant tumor should be considered.展开更多
Primary appendiceal neoplasms represent a relatively low percentage of all gastrointestinal cancers. A subset of these neoplasms, those of epithelial origin, are characterised by the production of a considerable amoun...Primary appendiceal neoplasms represent a relatively low percentage of all gastrointestinal cancers. A subset of these neoplasms, those of epithelial origin, are characterised by the production of a considerable amount of mucus, which is referred to as appendiceal mucinous neoplasms (AMN). Appendiceal mucinous neoplasms (AMN) have a low incidence, are easily misdiagnosed, depend on postoperative examination for confirmation of the diagnosis, are prone to form a “diagnosis”, and have a high incidence of the disease. Furthermore, they are prone to form peritoneal pseudomyxoma peritonei (PMP), are controversial in surgical decision-making, are prone to recurring after surgery alone, and are tricky to manage clinically. In this paper, we review the pathological characteristics, diagnosis and treatment of appendiceal mucinous tumours in the light of recent literature reports, with a view to providing certain references for the clinical diagnosis and treatment of this disease. .展开更多
BACKGROUND Early detection of advanced cystic mucinous neoplasms[(A-cMNs),defined as high-grade dysplasia or malignancy]of the pancreas is of great significance.As a simple and feasible detection method,serum tumor ma...BACKGROUND Early detection of advanced cystic mucinous neoplasms[(A-cMNs),defined as high-grade dysplasia or malignancy]of the pancreas is of great significance.As a simple and feasible detection method,serum tumor markers(STMs)may be used to predict advanced intraductal papillary mucinous neoplasms(IPMNs)and mucinous cystic neoplasms(MCNs).However,there are few studies on the usefulness of STMs other than carbohydrate antigen(CA)19-9 for early detection of A-cMNs.AIM To study the ability of five STMs-CA19-9,carcinoembryonic antigen(CEA),CA125,CA724,and CA242 to predict A-cMNs and distinguish IPMNs and MCNs.METHODS We mainly measured the levels of each STM in patients pathologically diagnosed with cMNs.The mean levels of STMs and the number of A-cMN subjects with a higher STM level than the cutoff were compared respectively to identify the ability of STMs to predict A-cMNs and distinguish MCNs from IPMNs.A receiver operating characteristic curve with the area under curve(AUC)was also created to identify the performance of the five STMs.RESULTS A total of 187 patients with cMNs were identified and 72 of them showed AcMNs.We found that CA19-9 exhibited the highest sensitivity(SE)(54.2%)and accuracy(76.5%)and a moderate ability(AUC=0.766)to predict A-cMNs.In predicting high-grade dysplasia IPMNs,the SE of CA19-9 decreased to 38.5%.The ability of CEA,CA125,and CA724 to predict A-cMNs was low(AUC=0.651,0.583,and 0.618,respectively).The predictive ability of CA242 was not identified.The combination of STMs improved the SE to 62.5%.CA125 may be specific to the diagnosis of advanced MCNs.CONCLUSION CA19-9 has a moderate ability,and CEA,CA125,and CA724 have a low ability to predict A-cMNs.The combination of STM testing could improve SE in predicting A-cMNs.展开更多
BACKGROUND Mucinous cystic neoplasm(MCN)of the pancreas is characterized by mucinproducing columnar epithelium and dense ovarian-type stroma and at risk for malignant transformation.Early diagnosis and treatment of MC...BACKGROUND Mucinous cystic neoplasm(MCN)of the pancreas is characterized by mucinproducing columnar epithelium and dense ovarian-type stroma and at risk for malignant transformation.Early diagnosis and treatment of MCN are particularly important.AIM To investigate the clinical characteristics of and management strategies for pancreatic mucinous cystadenoma(MCA)and mucinous cystadenocarcinoma(MCC).METHODS The clinical and pathological data of 82 patients with pancreatic MCA and MCC who underwent surgical resection at our department between April 2015 and March 2019 were retrospectively analyzed.RESULTS Of the 82 patients included in this study,70 had MCA and 12 had MCC.Tumor size of MCC was larger than that of MCA(P=0.049).Age and serum levels of tumor markers carcinoembryonic antigen(CEA),carbohydrate antigen(CA)19-9,and CA12-5 were significantly higher in MCC than in MCA patients(P=0.005,0.026,and 0.037,respectively).MCA tumor size was positively correlated with serum CA19-9 levels(r=0.389,P=0.001).Compared with MCC,MCA had a higher minimally invasive surgery rate(P=0.014).In the MCA group,the rate of major complications was 5.7%and that of clinically relevant pancreatic fistula was 8.6%;the corresponding rates in the MCC group were 16.7%and 16.7%,respectively.CONCLUSION Tumor size,age,and serum CEA,CA19-9,and CA12-5 levels may contribute to management of patients with MCN.Surgical resection is the primary treatment modality for MCC and MCA.展开更多
Pancreatic cystic neoplasms are being increasingly recognized, even in the absence of symptoms, in large part, due to markedly improved imaging modalities such as magnetic resonance imaging (MRI)/magnetic resonance ch...Pancreatic cystic neoplasms are being increasingly recognized, even in the absence of symptoms, in large part, due to markedly improved imaging modalities such as magnetic resonance imaging (MRI)/magnetic resonance cholangio pancreatography (MRCP) and computer tomography (CT) scanning. During the past 2 decades, better imaging of these cystic lesions has resulted in definition of different types, including pancreatic intraductal papillary mucinous neoplasms (IPMN). While IPMN represent only a distinct minority of all pancreatic cancers, they appear to be a relatively frequent neoplastic form of pancreatic cystic neoplasm. Moreover, IPMN have a much better outcome and prognosis compared to pancreatic ductal adenocarcinomas. Therefore, recognition of this entity is exceedingly important for the clinician involved in diagnosis and further evaluation of a potentially curable form of pancreatic cancer.展开更多
Intraductal papillary mucinous neoplasms （IPMNs） are a well-characterized group of rnucin-producing cystic neoplasms of the clear malignant potential type. We report here two cases of intraductal papillary mucinous ...Intraductal papillary mucinous neoplasms （IPMNs） are a well-characterized group of rnucin-producing cystic neoplasms of the clear malignant potential type. We report here two cases of intraductal papillary mucinous carcinoma （IPMC） with atypical manifestations. In one case, we discussed a pseudomyxoma peritonei caused by a ruptured IPMC. In the other case we discussed the fistulization of IPMC into the stomach and duodenum. These two cases suggest that IPMN can either spontaneously rupture causing mucinous materials to spill into the free abdominal cavity or directly invade adjacent organs resulting in fistula development.展开更多
Recently, a novel and distinct pancreatic cystic tumor termed 'mudnous nonneoplastic' cyst was described in the literature. We report our experience with a 71-year-old female with a cystic tumor in the body of...Recently, a novel and distinct pancreatic cystic tumor termed 'mudnous nonneoplastic' cyst was described in the literature. We report our experience with a 71-year-old female with a cystic tumor in the body of the pancreas demonstrating features suggestive of this diagnosis. We also review the literature regarding this 'novel' pathological entity and discuss critically its existence and its differential diagnoses.展开更多
Cystic neoplasms of the pancreas are relatively rare, comprising 10 percent of pancreatic cysts and only 1 percent of pancreatic cancers. Cystic neoplasms include mucinous cystic neoplasms, serous cystadenomas, papill...Cystic neoplasms of the pancreas are relatively rare, comprising 10 percent of pancreatic cysts and only 1 percent of pancreatic cancers. Cystic neoplasms include mucinous cystic neoplasms, serous cystadenomas, papillary cystic tumors, cystic islet cell tumors and intraductal papillary mucinous neoplasms of the pancreas (IPMNs). IPMN was first described in 1982. It has been most commonly described in 60 to 70 years old males, and represents a relatively ''new'' but increasingly recognized disease. The improvement and widespread use of modern imaging equipments and heightened awareness of physicians contribute to the increasing incidence of IPMN. The majority of IPMNs are located in the pancreatic head (75%) while the rest involves the body/tail regions. Multifocal IPMNs have been hypothesized, but the true presence of multifocality is unknown. Here we present a 72-yearold male diagnosed with IPMN (carcinoma in situ ) in the pancreatic head and a branch duct type IPMN (duct atypia) in the pancreatic body and tail. The patient underwent a Whipple intervention and a distal pancreatectomy. A three-year disease-free survival has been observed so far.展开更多
Cystic hepatic neoplasms are rare tumors,and are classified into two separate entities:mucinous cystic neoplasms(MCNs)and intraductal papillary mucinous neoplasms of the bile duct(IPMN-B).We report the case of a 56-ye...Cystic hepatic neoplasms are rare tumors,and are classified into two separate entities:mucinous cystic neoplasms(MCNs)and intraductal papillary mucinous neoplasms of the bile duct(IPMN-B).We report the case of a 56-year-old woman who presented with abdominal pain and jaundice due to the presence of a large hepatic multilocular cystic tumor associated with an intraductal tumor.Partial hepatectomy with resection of extrahepatic bile ducts demonstrated an intrahepatic MCN and an intraductal IPMN-B.This is the first report of the simultaneous occurrence of these two histologically distinct entities in the liver.展开更多
We present a rare case of synchronous mucinous borderline tumor of the ovary and low-grade appendiceal mucinous neoplasm (LAMN). We performed a left adnexectomy to diagnose the left ovarian borderline malignancy and a...We present a rare case of synchronous mucinous borderline tumor of the ovary and low-grade appendiceal mucinous neoplasm (LAMN). We performed a left adnexectomy to diagnose the left ovarian borderline malignancy and an ileostomy because of the swollen appendix during the operation. It was diagnosed as left ovarian mucinous borderline malignancy and LAMN. LAMN causes peritoneal dissemination, ovarian metastasis, and peritoneal pseudomyxoma. The appendix and ovary are close to each other anatomically and can metastasize if there is a tumor in either. For ovarian mucinous tumors, it is necessary to search the gastrointestinal tract, especially the appendix, as the primary lesion. For appendix tumors, it is necessary to search for the ovary. Since LAMN may be associated with borderline ovarian malignancies, as in this case, there is a possibility of the duplication of tumors when searching for ovarian mucinous tumors as the primary tumor and if ovarian tumors are found. Since LAMN and mucinous ovarian tumors have similar histological features, immunohistochemical staining is useful for their differentiation because they show different immunostaining patterns.展开更多
基金Supported by The Shandong Natural Science Foundation of China,No.ZR2021QH046.
文摘BACKGROUND Liposarcomas(LPSs)are malignant mesenchymal tumors originating from adipocytes.Myxoid LPS(MLPS),a common subtype,predominantly arises in the extremities,retroperitoneum,and deep soft tissues,with a rare occurrence in the gastrointestinal tract.Primary mesenteric LPS is particularly uncommon,especially in the transverse colonic mesentery.CASE SUMMARY This report describes the case of a 65-year-old female patient who presented with abdominal distension and was diagnosed with a giant mucinous LPS of the transverse colonic mesentery.Upon admission,the patient underwent a comprehensive evaluation.Contrast-enhanced computed tomography(CT)of the chest and abdomen revealed a large malignant tumor with aortic dissection,while colonoscopy identified rectal cancer.Given the patient's condition and surgical risk,an interventional procedure was first performed to manage the aortic coarctation,followed by tumor resection via laparotomy.Intraoperative and histopathological findings confirmed a giant mucinous LPS originating from the transverse colon mesentery.Postoperatively,the patient underwent chemotherapy and regular follow-up CT.Although the rectal tumor did not recur,the mesenteric tumor showed extensive recurrence,compressing the intestinal lumen and causing mechanical obstruction,which severely threatened the patient's life.A second operation temporarily relieved the obstructive symptoms;however,it had a limited effect on tumor progression.The patient died four months later due to disease progression.CONCLUSION While no standardized treatment exists for MLPS co-occurring with multiple diseases,operation remains the mainstay.However,recurrence,metastasis,and poor postoperative prognosis continue to pose serious threats to patient survival.
文摘Appendiceal mucinous neoplasms(AMNs)are rare tumors originating from mucin-producing epithelial cells of the appendix.They can exhibit both benign and malignant behavior.They are often incidentally discovered during appendectomy.Clinical presentation ranges from asymptomatic to mimicking acute appendicitis.Histologically,noninvasive AMNs are classified as low-grade AMNs(LAMNs)or high-grade AMNs(HAMNs),whereas invasive tumors are categorized as mucinous adenocarcinomas.Although LAMNs and HAMNs are generally nonmalignant,rupture can lead to pseudomyxoma peritonei(PMP).Surgical resection is the primary diagnostic and therapeutic approach,with intraoperative assessment to prevent rupture.Treatment strategies vary based on findings and include appendectomy,right hemicolectomy,and cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.Histological diagnosis relies on mucin detection,and immunohistochemical markers such as cytokeratin 20(diffusely positive),cytokeratin 7(often negative),mucin 5AC,and special ATrich sequence-binding protein 2 assist in characterization.Molecular profiling frequently identifies KRAS,GNAS,and TP53 mutations.KRAS mutations are generally associated with a favorable prognosis,whereas GNAS and TP53 mutations correlate with poorer survival outcomes.These findings highlight the potential role of molecular profiling in guiding treatment strategies for AMN and PMP.
基金Supported by Jinhua City Science and Technology Plan Projects“Improve the Polarization of Cancer-related Fatigue:Clinical Efficacy Study”,No.2018-4-049.
文摘BACKGROUND Low-grade appendiceal mucinous neoplasms(LAMNs)are a class of histolo-gically well-differentiated adenomas that can proliferate outside the appendix in the form of malignant tumours,resulting in the accumulation of external appen-diceal mucus.They may present as an extra-appendiceal mass-like bulge,mucus or polypoid tissue at the appendiceal orifice,or even with abdominal dissem-ination.The surgical approach is usually appendectomy,ileocecal resection or right hemicolectomy.It is essential to raise awareness of LAMNs and choose an appropriate treatment.CASE SUMMARY A 68-year-old man underwent a routine physical examination at our hospital on May 17,2022.The patient had no symptoms of abdominal pain,bloating,or weight loss,and his tumour marker levels were normal.The faecal occult blood test was negative,and no abnormalities were identified on physical examination.Colonoscopy revealed a submucosal protrusion at the appendiceal orifice.During endoscopic ultrasound,uneven echoes were observed in the appendix cavity.A contrast-enhanced computed tomography scan of the abdomen revealed a nodular thickening at the base of the appendix,with a diameter of approximately 1 cm.When all the examination results were considered,we suspected the lesion to be an appendiceal mucinous tumour.Laparoscopic surgery was performed using the double purse-string suture method.Postoperative pathology suggested a low-grade mucinous cystadenoma of the appendix with no involvement of the margins.A repeat colonoscopy 18 months after surgery revealed no significant abnormality at the appendiceal orifice.A contrast-enhanced computed tomography scan of the abdomen suggested only postoperative changes.CONCLUSION This study describes a case of LAMN that was treated by resecting the mass at the appendiceal orifice via the double purse-string suture technique and provides new insights on the diagnosis and treatment of LAMNs.
文摘Mucinous cystic neoplasms of the liver(MCN-L)are rare cystic lesions characterized by mucin-producing epithelium and ovarian-like stroma.Although they constitute fewer than 5%of hepatic cystic lesions,MCN-L poses significant diagnostic challenges due to overlapping features with other cystic lesions and their potential for malignant transformation.Early recognition and definitive surgical intervention are therefore critical to ensure optimal patient outcomes.A literature review was conducted to summarize epidemiology,clinical presentation,diagnostic modalities,and management strategies for MCN-L.Additionally,from 2019 to 2025,9 patients with MCN-L were identified at our center.Clinical data and outcomes were retrospectively analyzed.MCN-L predominantly affects middle-aged women and presents as large,multiloculated cystic lesions without biliary communication.The revised 2010 World Health Organization classification emphasizes the presence of ovarian-like stroma for definitive diagnosis.Contrast-enhanced computed tomography or magnetic resonance imaging are often suggestive but not pathognomonic,reinforcing the need for histopathological confirmation.MCN-L remains a diagnostic and therapeutic challenge due to its resemblance to other cystic liver lesions.Complete surgical resection is the treatment of choice to prevent recurrence and malignant transformation,reinforcing the importance of early intervention.Further research is needed to improve diagnostic accuracy and refine management strategies.
文摘BACKGROUND Pancreatic neuroendocrine neoplasms(pNENs)are rare,heterogeneous tumors accounting for 1%-2%of pancreatic tumors,with significant malignant potential.Intraductal papillary mucinous neoplasm of the bile duct(IPMN-B)is a rare precancerous lesion in the bile duct system,with potential for malignancy.The combination of pNENs and IPMN-B is exceptionally rare and often leads to misdiagnosis.This study aims to report a rare case of pNENs combined with IPMN-B treated at Yanbian University Hospital to improve understanding and management of this unusual tumor combination.CASE SUMMARY We retrospectively analyzed a case from Yanbian University Hospital.We re-viewed clinical records,imaging findings,endoscopic retrograde cholangiopan-creatography,surgical exploration,and histopathological examination.The pa-tient was diagnosed with pNENs and IPMN-B.Surgical treatment was performed,with follow-up showing effective management and no significant recurrence.CONCLUSION This case represents the first report of pNENs combined with IPMN-B.It high-lights the need for thorough diagnostic evaluation to prevent misdiagnosis and improve treatment strategies.
文摘Intraductal papillary mucinous neoplasm(IPMN)and intraductal papillary neoplasm of the bile duct(IPNB)are mucinous cystic tumors with intraductal papillary growth and malignant potential.Their concurrent occurrence is exceptionally rare.CASE SUMMARY A 58-year-old Chinese man presented with recurrent upper abdominal pain.Imaging and laboratory tests revealed lesions consistent with IPNB and IPMN.Postoperative pathological examination confirmed IPNB with high-grade dysplasia and main-duct type IPMN with low-grade dysplasia.The patient underwent extrahepatic bile duct resection with Roux-en-Y choledochoenterostomy and distal pancreatectomy.He had an excellent prognosis with no tumor recurrence during the 30-month follow-up.CONCLUSION This case emphasizes the importance of comprehensive preoperative assessment and individualized management for these complex tumors.Further research is needed to understand their pathogenesis and improve treatment strategies.
文摘Intraductal papillary mucinous neoplasm(IPMN) of the pancreas is a noninvasive epithelial neoplasm of mucinproducing cells arising in the main duct(MD) and/or branch ducts(BD) of the pancreas.Involved ducts are dilated and filled with neoplastic papillae and mucus in variable intensity.IPMN lacks ovarian-type stroma,unlike mucinous cystic neoplasm,and is defined as a grossly visible entity(≥ 5 mm),unlike pancreatic intraepithelial neoplasm.With the use of high-resolution imaging techniques,very small IPMNs are increasingly being identified.Most IPMNs are solitary and located in the pancreatic head,although 20%-40% are multifocal.Macroscopic classification in MD type,BD type and mixed or combined type reflects biological differences with important prognostic and preoperative clinical management implications.Based on cytoarchitectural atypia,IPMN is classified into low-grade,intermediategrade and high-grade dysplasia.Based on histological features and mucin(MUC) immunophenotype,IPMNs are classified into gastric,intestinal,pancreatobiliary and oncocytic types.These different phenotypes can be observed together,with the IPMN classified according to the predominant type.Two pathways have been suggested:gastric phenotype corresponds to less aggressive uncommitted cells(MUC1-,MUC2-,MUC5 AC +,MUC6 +) with the capacity to evolve to intestinal phenotype(intestinal pathway)(MUC1-,MUC2 +,MUC5 AC +,MUC6- or weak +) or pancreatobiliary /oncocytic phenotypes(pyloropancreatic pathway)(MUC1 +,MUC 2-,MUC5 AC +,MUC 6 +) becoming more aggressive.Prognosis of IPMN is excellent but critically worsens when invasive carcinoma arises(about 40% of IPMNs),except in some cases of minimal invasion.The clinical challenge is to establish which IPMNs should be removed because of their higher risk of developing invasive cancer.Once resected,they must be extensively sampled or,much better,submitted in its entirety for microscopic study to completely rule out associated invasive carcinoma.
文摘BACKGROUND Appendiceal mucinous neoplasms(AMNs),although not classified as rare,are relatively uncommon tumors most often discovered incidentally during colorectal surgery.Accurate identification of AMNs is difficult due to non-specific sym-ptoms,overlapping tumor markers with other conditions,and the potential for misdiagnosis.This underscores the urgent need for precision in diagnosis to pre-vent severe complications.CASE SUMMARY This case report describes the unexpected discovery and treatment of a low-grade AMN(LAMN)in a 74-year-old man undergoing laparoscopic hemicolectomy for transverse colon adenocarcinoma(AC).Preoperatively,non-specific gastroin-testinal symptoms and elevated tumor markers masked the presence of AMN.The tumor,presumed to be an AMN peritoneal cyst intraoperatively,was con-firmed as LAMN through histopathological examination.The neoplasm exhibited mucin accumulation and a distinct immunohistochemical profile:Positive for Ho-meobox protein CDX-2,Cytokeratin 20,special AT-rich sequence-binding protein 2,and Mucin 2 but negative for cytokeratin 7 and Paired box gene 8.This profile aids in distinguishing appendiceal and ovarian mucinous tumors.Postoperative recovery was uncomplicated,and the patient initiated adjuvant chemotherapy for the colon AC.CONCLUSION This case highlights the diagnostic complexity of AMNs,emphasizing the need for vigilant identification to avert potential complications,such as pseudomyxoma peritonei.
基金supported by grants from Zhejiang Natural Science Foundation (GF21H030024)the General Scientific Re-search Project of Zhejiang Provincial Department of Education (Y202146219)the Postgraduate Education Research Project of Zhejiang University (20220326)。
文摘Background: Biliary mucinous cystic neoplasms(BMCNs) are rare hepatobiliary cystic tumors, which can be divided into noninvasive and invasive types. This study aimed to investigate the diagnosis, treatment, and prognosis of BMCNs in a large single center. Methods: We analyzed 49 patients with BMCNs confirmed by postoperative pathology at the First Afflliated Hospital, Zhejiang University School of Medicine between January 2007 and December 2021. Results: Among the 49 patients, 37 were female(75.5%), and the average age was 57.04 years. Common symptoms included abdominal discomfort, jaundice and fever, while 22 patients(44.9%) had no symptoms. Serum carbohydrate antigen(CA) 19-9 and CA125 concentrations were elevated in 34.8% and 19.6% of patients, respectively. Forty-eight patients had tumors in the intrahepatic bile ducts and only one had a tumor in the extrahepatic bile duct. Forty-eight patients with noninvasive intrahepatic BMCNs were further analyzed in terms of pathological features: 34(70.8%) had low-grade intraepithelial neoplasms(LGINs), and 14(29.2%) had high-grade intraepithelial neoplasms(HGINs). The potential immunohistochemical markers of BMCNs were cytokeratin(CK) 19, CK7, estrogen receptor and progesterone receptor. Follow-up data for 37 patients with intrahepatic BMCNs were obtained. The median overall survival(OS) of BMCNs was not reached. The longest survival time was 137 months.The 5-and 10-year OS rates were 100% and 85.4%, respectively. The 5-and 10-year recurrence-free survival(RFS) rates were 93.9% and 80.2%, respectively. Conclusions: BMCNs are rare cystic neoplasms that commonly occur in middle-aged females. BMCNs can only be diagnosed and classified by postoperative pathology, as there are no specific clinical presentations, serological indicators or imaging modalities for preoperative diagnosis. Complete surgical resection is necessary for BMCNs, and the postoperative prognosis is favorable.
基金Scientific Research Fund of National Health Commission of China,Key Health Science and Technology Program of Zhejiang Province,No.WKJ-ZJ-2201Key Project of Social Welfare Program of Zhejiang Science and Technology Department,“Lingyan”Program,No.2022C03099and Clinical Research Fund Project of Zhejiang Medical Association,No.2021ZYC-A173.
文摘BACKGROUND Low-grade appendiceal neoplasms(LAMN)are characterized by low incidence and atypical clinical presentations,often leading to misdiagnosis as acute or chronic appendicitis before surgery.The primary diagnostic tool for LAMN is abdominal computed tomography(CT)imaging.Surgical resection remains the cornerstone of LAMN management,necessitating en bloc tumor excision to minimize the risk of iatrogenic rupture.Laparoscopy,known for its minimal invasiveness,reduced postoperative discomfort,and expedited recovery,is a safe and reliable approach for LAMN treatment.Despite the possibility of pseudomyxoma peritonei development,appendectomy and partial appendectomy generally result in negative tumor margins and favorable outcomes,which can be attributed to the disease’s slow growth and lower malignancy.CASE SUMMARY A 71-year-old male patient was admitted to our hospital with a pelvic spaceoccupying lesion detected 1 mo prior.Physical examination showed a soft abdomen without tenderness or rebound and no palpable masses.No shifting dullness was noted,and digital rectal examination revealed no palpable mass.Enteroscopy revealed a raised,smooth-surfaced mass measuring 3.0 cm in the cecum.Abdominal contrast-enhanced CT showed a markedly thickened and dilated appendix with visible cystic shadows.Laparoscopic surgery was performed and revealed a significantly dilated appendix,leading to laparoscopic resection of the appendix and part of the cecum.Post-surgical pathologic analysis confirmed LAMN.The patient received symptomatic and supportive post-operative care and was discharged on postoperative day 4 without complications such as abdominal bleeding,intestinal obstruction,or incision infection.No tumor recurrence was observed during a 7-mo follow-up period.CONCLUSION LAMN is a rare disease that lacks specific clinical manifestations.Abdominal CT plays a crucial role in diagnosing LAMN,and laparoscopic surgery is a safe and effective diagnostic and therapeutic approach.
文摘BACKGROUND Rectal mucinous adenocarcinoma(MAC)is a rare pathological type of rectal can-cer with unique pathological features and a poor prognosis.It is difficult to diag-nose and treat early because of the lack of specific manifestations in some aspects of the disease.The common metastatic organs of rectal cancer are the liver and lung;however,rectal carcinoma with metastasis to subcutaneous soft tissue is a rare finding.CASE SUMMARY In this report,the clinical data,diagnosis and treatment process,and postope-rative pathological features of a patient with left waist subcutaneous soft tissue masses were retrospectively analyzed.The patient underwent surgical treatment after admission and recovered well after surgery.The final pathological diagnosis was rectal MAC with left waist subcutaneous soft tissue metastasis.CONCLUSION Subcutaneous soft tissue metastasis of rectal MAC is rare,and it can suggest that the tumor is disseminated,and it can appear even earlier than the primary ma-lignant tumor,which is occult and leads to a missed diagnosis and misdiagnosis clinically.When a subcutaneous soft tissue mass of unknown origin appears in a patient with rectal cancer,a ma-lignant tumor should be considered.
文摘Primary appendiceal neoplasms represent a relatively low percentage of all gastrointestinal cancers. A subset of these neoplasms, those of epithelial origin, are characterised by the production of a considerable amount of mucus, which is referred to as appendiceal mucinous neoplasms (AMN). Appendiceal mucinous neoplasms (AMN) have a low incidence, are easily misdiagnosed, depend on postoperative examination for confirmation of the diagnosis, are prone to form a “diagnosis”, and have a high incidence of the disease. Furthermore, they are prone to form peritoneal pseudomyxoma peritonei (PMP), are controversial in surgical decision-making, are prone to recurring after surgery alone, and are tricky to manage clinically. In this paper, we review the pathological characteristics, diagnosis and treatment of appendiceal mucinous tumours in the light of recent literature reports, with a view to providing certain references for the clinical diagnosis and treatment of this disease. .
基金National Natural Science Foundation of China,No.81770642the Shanghai Association for Science and Technology,China,No.19411951602.
文摘BACKGROUND Early detection of advanced cystic mucinous neoplasms[(A-cMNs),defined as high-grade dysplasia or malignancy]of the pancreas is of great significance.As a simple and feasible detection method,serum tumor markers(STMs)may be used to predict advanced intraductal papillary mucinous neoplasms(IPMNs)and mucinous cystic neoplasms(MCNs).However,there are few studies on the usefulness of STMs other than carbohydrate antigen(CA)19-9 for early detection of A-cMNs.AIM To study the ability of five STMs-CA19-9,carcinoembryonic antigen(CEA),CA125,CA724,and CA242 to predict A-cMNs and distinguish IPMNs and MCNs.METHODS We mainly measured the levels of each STM in patients pathologically diagnosed with cMNs.The mean levels of STMs and the number of A-cMN subjects with a higher STM level than the cutoff were compared respectively to identify the ability of STMs to predict A-cMNs and distinguish MCNs from IPMNs.A receiver operating characteristic curve with the area under curve(AUC)was also created to identify the performance of the five STMs.RESULTS A total of 187 patients with cMNs were identified and 72 of them showed AcMNs.We found that CA19-9 exhibited the highest sensitivity(SE)(54.2%)and accuracy(76.5%)and a moderate ability(AUC=0.766)to predict A-cMNs.In predicting high-grade dysplasia IPMNs,the SE of CA19-9 decreased to 38.5%.The ability of CEA,CA125,and CA724 to predict A-cMNs was low(AUC=0.651,0.583,and 0.618,respectively).The predictive ability of CA242 was not identified.The combination of STMs improved the SE to 62.5%.CA125 may be specific to the diagnosis of advanced MCNs.CONCLUSION CA19-9 has a moderate ability,and CEA,CA125,and CA724 have a low ability to predict A-cMNs.The combination of STM testing could improve SE in predicting A-cMNs.
文摘BACKGROUND Mucinous cystic neoplasm(MCN)of the pancreas is characterized by mucinproducing columnar epithelium and dense ovarian-type stroma and at risk for malignant transformation.Early diagnosis and treatment of MCN are particularly important.AIM To investigate the clinical characteristics of and management strategies for pancreatic mucinous cystadenoma(MCA)and mucinous cystadenocarcinoma(MCC).METHODS The clinical and pathological data of 82 patients with pancreatic MCA and MCC who underwent surgical resection at our department between April 2015 and March 2019 were retrospectively analyzed.RESULTS Of the 82 patients included in this study,70 had MCA and 12 had MCC.Tumor size of MCC was larger than that of MCA(P=0.049).Age and serum levels of tumor markers carcinoembryonic antigen(CEA),carbohydrate antigen(CA)19-9,and CA12-5 were significantly higher in MCC than in MCA patients(P=0.005,0.026,and 0.037,respectively).MCA tumor size was positively correlated with serum CA19-9 levels(r=0.389,P=0.001).Compared with MCC,MCA had a higher minimally invasive surgery rate(P=0.014).In the MCA group,the rate of major complications was 5.7%and that of clinically relevant pancreatic fistula was 8.6%;the corresponding rates in the MCC group were 16.7%and 16.7%,respectively.CONCLUSION Tumor size,age,and serum CEA,CA19-9,and CA12-5 levels may contribute to management of patients with MCN.Surgical resection is the primary treatment modality for MCC and MCA.
文摘Pancreatic cystic neoplasms are being increasingly recognized, even in the absence of symptoms, in large part, due to markedly improved imaging modalities such as magnetic resonance imaging (MRI)/magnetic resonance cholangio pancreatography (MRCP) and computer tomography (CT) scanning. During the past 2 decades, better imaging of these cystic lesions has resulted in definition of different types, including pancreatic intraductal papillary mucinous neoplasms (IPMN). While IPMN represent only a distinct minority of all pancreatic cancers, they appear to be a relatively frequent neoplastic form of pancreatic cystic neoplasm. Moreover, IPMN have a much better outcome and prognosis compared to pancreatic ductal adenocarcinomas. Therefore, recognition of this entity is exceedingly important for the clinician involved in diagnosis and further evaluation of a potentially curable form of pancreatic cancer.
基金Supported by the funds from researching and developing business for overcoming cancer,No. 07-2005-038-0
文摘Intraductal papillary mucinous neoplasms （IPMNs） are a well-characterized group of rnucin-producing cystic neoplasms of the clear malignant potential type. We report here two cases of intraductal papillary mucinous carcinoma （IPMC） with atypical manifestations. In one case, we discussed a pseudomyxoma peritonei caused by a ruptured IPMC. In the other case we discussed the fistulization of IPMC into the stomach and duodenum. These two cases suggest that IPMN can either spontaneously rupture causing mucinous materials to spill into the free abdominal cavity or directly invade adjacent organs resulting in fistula development.
文摘Recently, a novel and distinct pancreatic cystic tumor termed 'mudnous nonneoplastic' cyst was described in the literature. We report our experience with a 71-year-old female with a cystic tumor in the body of the pancreas demonstrating features suggestive of this diagnosis. We also review the literature regarding this 'novel' pathological entity and discuss critically its existence and its differential diagnoses.
文摘Cystic neoplasms of the pancreas are relatively rare, comprising 10 percent of pancreatic cysts and only 1 percent of pancreatic cancers. Cystic neoplasms include mucinous cystic neoplasms, serous cystadenomas, papillary cystic tumors, cystic islet cell tumors and intraductal papillary mucinous neoplasms of the pancreas (IPMNs). IPMN was first described in 1982. It has been most commonly described in 60 to 70 years old males, and represents a relatively ''new'' but increasingly recognized disease. The improvement and widespread use of modern imaging equipments and heightened awareness of physicians contribute to the increasing incidence of IPMN. The majority of IPMNs are located in the pancreatic head (75%) while the rest involves the body/tail regions. Multifocal IPMNs have been hypothesized, but the true presence of multifocality is unknown. Here we present a 72-yearold male diagnosed with IPMN (carcinoma in situ ) in the pancreatic head and a branch duct type IPMN (duct atypia) in the pancreatic body and tail. The patient underwent a Whipple intervention and a distal pancreatectomy. A three-year disease-free survival has been observed so far.
文摘Cystic hepatic neoplasms are rare tumors,and are classified into two separate entities:mucinous cystic neoplasms(MCNs)and intraductal papillary mucinous neoplasms of the bile duct(IPMN-B).We report the case of a 56-year-old woman who presented with abdominal pain and jaundice due to the presence of a large hepatic multilocular cystic tumor associated with an intraductal tumor.Partial hepatectomy with resection of extrahepatic bile ducts demonstrated an intrahepatic MCN and an intraductal IPMN-B.This is the first report of the simultaneous occurrence of these two histologically distinct entities in the liver.
文摘We present a rare case of synchronous mucinous borderline tumor of the ovary and low-grade appendiceal mucinous neoplasm (LAMN). We performed a left adnexectomy to diagnose the left ovarian borderline malignancy and an ileostomy because of the swollen appendix during the operation. It was diagnosed as left ovarian mucinous borderline malignancy and LAMN. LAMN causes peritoneal dissemination, ovarian metastasis, and peritoneal pseudomyxoma. The appendix and ovary are close to each other anatomically and can metastasize if there is a tumor in either. For ovarian mucinous tumors, it is necessary to search the gastrointestinal tract, especially the appendix, as the primary lesion. For appendix tumors, it is necessary to search for the ovary. Since LAMN may be associated with borderline ovarian malignancies, as in this case, there is a possibility of the duplication of tumors when searching for ovarian mucinous tumors as the primary tumor and if ovarian tumors are found. Since LAMN and mucinous ovarian tumors have similar histological features, immunohistochemical staining is useful for their differentiation because they show different immunostaining patterns.
