BACKGROUND Pancreatic mixed serous-neuroendocrine neoplasms(MSNNs)are mixed tumors containing two components with different pathologies,namely,pancreatic serous cystic neoplasm(PSCN)and pancreatic neuroendocrine tumor...BACKGROUND Pancreatic mixed serous-neuroendocrine neoplasms(MSNNs)are mixed tumors containing two components with different pathologies,namely,pancreatic serous cystic neoplasm(PSCN)and pancreatic neuroendocrine tumor(PanNET).For MSNNs,diffuse PSCN involving the whole pancreas is extremely rare,with only eight previous case reports.CASE SUMMARY A 45-year-old Chinese woman,with a free previous medical history and no obvious symptoms,was found to have a pancreatic neoplasm and admitted to our hospital for further diagnosis in March 2018.Abdominal palpation revealed a painless,mobile mass in the epigastrium,and no abnormalities were observed in an examination of the nervous system and ocular system.A computed tomography scan showed multiple cystic lesions involving the whole pancreas ranging in diameter from 0.4 to 2 cm and also revealed an enhanced mass,2.2 cm in diameter,in the head of the pancreas.Moreover,multiple cysts were found in the kidneys bilaterally,and the right lobe of the liver contained a small cyst.A Whipple operation with total pancreatectomy and splenectomy was performed.A diagnosis of pancreatic MSNN was established,consisting of diffuse serous microcystic cystadenoma with a concomitant grade 2 PanNET.Of note,the patient had no personal or family history of Von Hippel-Lindau syndrome or other disease.CONCLUSION We report the first case of MSNN with a diffuse PSCN component involving the entire pancreas in a Chinese woman.It is important to be aware of its relationship with VHL syndrome,and close clinical follow-up is recommended.展开更多
Mixed neuroendocrine non-neuroendocrine neoplasms(MiNENs)are a hetero-geneous group of malignant neoplasms that can settle in the gastroenteropan-creatic tract.They are composed of a neuroendocrine(NE)and a non-NE com...Mixed neuroendocrine non-neuroendocrine neoplasms(MiNENs)are a hetero-geneous group of malignant neoplasms that can settle in the gastroenteropan-creatic tract.They are composed of a neuroendocrine(NE)and a non-NE compo-nent in at least 30%of each tumour.The non-NE component can include different histological combinations of glandular,squamous,mucinous and sarcomatoid phenotypes,and one or both of the components can be low-or high grade malignant.Recent changes in the nomenclature of these neoplasms might lead to great deal of confusion,and the lack of specific clinical trials is the main reason why their management is difficult.The review aims to clarify the definition of MiNEN and analyze available evidence about their diagnosis and treatment options according to their location and extension through careful analysis of the available data.It would be important to reach a general consensus on their diagnosis in order to construct a classification that remains stable over time and facilitates the design of clinical trials that,due to their low incidence,will require long recruitment periods.展开更多
Here we report a rare case of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs). A 51-year-old lady with no known family history of malignancies developed this rare type of malignancy without any active gastr...Here we report a rare case of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs). A 51-year-old lady with no known family history of malignancies developed this rare type of malignancy without any active gastrointestinal symptoms. However, during a routine health check, physicians noticed a raised Carcinoembryonic Antigen (CEA) level and the patient subsequently was referred to the surgical department for further management. A colonoscopy and CECT abdomen were done and she was electively admitted for a left hemicolectomy operation for a splenic flexure tumour. The histopathological report revealed the tumour is a case of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) [moderately differentiated adenocarcinoma and neuroendocrine tumour grade 3]. TNM (8th edition, 2016): pT3, pN2 (20/21), pMX. Both resection margins were clear from malignant cells. Colorectal MiNENs, constitute a rare group of gastrointestinal tumours composed of both neuroendocrine and non-neuroendocrine components. Given their non-diagnostic macroscopic features, specific histological features and lack of disease awareness which are responsible for the underestimated incidence and conflicting data. In this case, a multidisciplinary team approach is important in managing patients with this malignancy to achieve the best outcome.展开更多
In this editorial we comment on the manuscript describing a case of adenocarcinoma mixed with a neuroendocrine carcinoma of the gastroesophageal junction.Mixed neuroendocrine and non-neuroendocrine neoplasms of the ga...In this editorial we comment on the manuscript describing a case of adenocarcinoma mixed with a neuroendocrine carcinoma of the gastroesophageal junction.Mixed neuroendocrine and non-neuroendocrine neoplasms of the gastrointestinal system are rare heterogeneous group of tumors characterized by a high malignant potential,rapid growth,and poor prognosis.Due to the rarity of these cancers,the standard therapy is poorly defined.The diagnosis of these tumors is based on combination of morphological features,immunohistochemical and neuroendocrine and epithelial cell markers.Both endocrine and epithelial cell components can act independently of each other and thus,careful grading of each component separately is required.These cancers are aggressive in nature and the potential of each component has paramount importance in the choice of treatment and response.Regardless of the organ of origin,these tumors portend poor prognosis with increased proportion of neuroendocrine component.Multidisciplinary services and strategies are required for the management of these mixed malignancies to provide the best oncological outcomes.The etiopathogenesis of these mixed tumors remains obscure but poses interesting question.We briefly discuss a few salient points in this editorial.展开更多
BACKGROUND Mixed neuroendocrine non-neuroendocrine neoplasm(MiNEN)is a rare diagnosis,mainly encountered in the gastro-entero-pancreatic tract.There is limited knowledge of its epidemiology,prognosis and biology,and t...BACKGROUND Mixed neuroendocrine non-neuroendocrine neoplasm(MiNEN)is a rare diagnosis,mainly encountered in the gastro-entero-pancreatic tract.There is limited knowledge of its epidemiology,prognosis and biology,and the best management for affected patients is still to be defined.AIM To investigate clinical-pathological characteristics,treatment modalities and survival outcomes of a retrospective cohort of patients with a diagnosis of MiNEN.METHODS Consecutive patients with a histologically proven diagnosis of MiNEN were identified at 5 European centres.Patient data were retrospectively collected from medical records.Pathological samples were reviewed to ascertain compliance with the 2017 World Health Organisation definition of MiNEN.Tumour responses to systemic treatment were assessed according to the Response Evaluation Criteria in Solid Tumours 1.1.Kaplan-Meier analysis was applied to estimate survival outcomes.Associations between clinical-pathological characteristics and survival outcomes were explored using Log-rank test for equality of survivors functions(univariate)and Cox-regression analysis(multivariable).RESULTS Sixty-nine consecutive patients identified;Median age at diagnosis:64 years.Males:63.8%.Localised disease(curable):53.6%.Commonest sites of origin:colon-rectum(43.5%)and oesophagus/oesophagogastric junction(15.9%).The neuroendocrine component was;predominant in 58.6%,poorly differentiated in 86.3%,and large cell in 81.25%,of cases analysed.Most distant metastases analysed(73.4%)were occupied only by a poorly differentiated neuroendocrine component.Ninety-four percent of patients with localised disease underwent curative surgery;53%also received perioperative treatment,most often in line with protocols for adenocarcinomas from the same sites of origin.Chemotherapy was offered to most patients(68.1%)with advanced disease,and followed protocols for pure neuroendocrine carcinomas or adenocarcinomas in equal proportion.In localised cases,median recurrence free survival(RFS);14.0 months(95%CI:9.2-24.4),and median overall survival(OS):28.6 months(95%CI:18.3-41.1).On univariate analysis,receipt of perioperative treatment(vs surgery alone)did not improve RFS(P=0.375),or OS(P=0.240).In advanced cases,median progression free survival(PFS);5.6 months(95%CI:4.4-7.4),and median OS;9.0 months(95%CI:5.2-13.4).On univariate analysis,receipt of palliative active treatment(vs best supportive care)prolonged PFS and OS(both,P<0.001).CONCLUSION MiNEN is most commonly driven by a poorly differentiated neuroendocrine component,and has poor prognosis.Advances in its biological understanding are needed to identify effective treatments and improve patient outcomes.展开更多
Augmented-and mixed-reality technologies have pioneered the realization of real-time fusion and interactive projection for laparoscopic surgeries.Indocyanine green fluorescence imaging technology has enabled anatomica...Augmented-and mixed-reality technologies have pioneered the realization of real-time fusion and interactive projection for laparoscopic surgeries.Indocyanine green fluorescence imaging technology has enabled anatomical,functional,and radical hepatectomy through tumor identification and localization of target hepatic segments,driving a transformative shift in themanagement of hepatic surgical diseases,moving away from traditional,empirical diagnostic and treatment approaches toward digital,intelligent ones.The Hepatic Surgery Group of the Surgery Branch of the Chinese Medical Association,Digital Medicine Branch of the Chinese Medical Association,Digital Intelligent Surgery Committee of the Chinese Society of ResearchHospitals,and Liver Cancer Committee of the Chinese Medical Doctor Association organized the relevant experts in China to formulate this consensus.This consensus provides a comprehensive outline of the principles,advantages,processes,and key considerations associated with the application of augmented reality and mixed-reality technology combined with indocyanine green fluorescence imaging technology for hepatic segmental and subsegmental resection.The purpose is to streamline and standardize the application of these technologies.展开更多
BACKGROUND Mixed tumors of the colon and rectum,composed of a combination of epithelial and endocrine elements of benign and malignant potential are rare neoplasms.These can occur anywhere in the gastrointestinal trac...BACKGROUND Mixed tumors of the colon and rectum,composed of a combination of epithelial and endocrine elements of benign and malignant potential are rare neoplasms.These can occur anywhere in the gastrointestinal tract and are often diagnosed incidentally.Though they have been a well-documented entity in the pancreas,where the exocrine-endocrine mixed tumors have been known for a while,recognition and accurate diagnosis of these tumors in the colon and rectum,to date,remains a challenge.This is further compounded by the different terminologies that have been attributed to these lesions over the years adding to increased confusion and misclassification.Therefore,dedicated literature reviews of these lesions in the colon and rectum are inconsistent and are predominantly limited to case reports and case series of limited case numbers.Though,most of these tumors are high grade and of advanced stage,intermediate and low grade lesions of these mixed tumors are also increasingly been reported.There are no established independent consensus based guidelines for the therapeutic patient management of these unique lesions.AIM To provide a comprehensive targeted literature review of these complex mixed tumors in the colon and rectum that chronicles the evolution over time with summarization of historical perspectives of terminology and to further our understanding regarding their pathogenesis including genomic landscape,clinicoradiological features,pathology,treatment,prognosis,the current status of the management of the primary lesions,their recurrences and metastases.METHODS A comprehensive review of the published English literature was conducted using the search engines PubMed,MEDLINE and GOOGLE scholar.The following search terms[“mixed tumors colon”OR mixed endocrine/neuroendocrine tumor/neoplasm/lesion colon OR adenocarcinoma and endocrine/neuroendocrine tumor colon OR mixed adenocarcinoma and endocrine/neuroendocrine carcinoma colon OR Amphicrine tumors OR Collision tumors]were used.Eligibility criteria were defined and all potential relevant items,including full articles and/or abstracts were independently reviewed,assessed and agreed upon items were selected for in-depth analysis.RESULTS In total 237 full articles/abstracts documents were considered for eligibility of which 45 articles were illegible resulting in a total of 192 articles that were assessed for eligibility of which 139 have been selected for reference in this current review.This seminal manuscript is a one stop article that provides a detailed outlook on the evolution over time with summarization of historical perspectives,nomenclature,clinicoradiological features,pathology,treatment,prognosis and the current status of the management of both the primary lesions,their recurrences and metastases.Gaps in knowledge have also been identified and discussed.An important outcome of this manuscript is the justified proposal for a new,simple,clinically relevant,non-ambiguous terminology for these lesions to be referred to as mixed epithelial endocrine neoplasms(MEENs).CONCLUSION MEEN of the colon and rectum are poorly understood rare entities that encompass an extensive range of heterogeneous tumors with a wide variety of combinations leading to tumors of high,intermediate or low grade malignant potential.This proposed new revised terminology of MEEN will solve the biggest hurdle of confusion and misclassification that plagues these rare unique colorectal neoplasms thus facilitating the future design of multi institutional prospective randomized controlled clinical trials to develop and evaluate newer therapeutic strategies that are recommended for continued improved understanding and personal optimization of clinical management of these unique colorectal neoplasms.展开更多
BACKGROUND The incidence of mixed neuroendocrine-non-neuroendocrine neoplasms(MiNEN)is low.To improve our understanding of this rare tumor type and optimally guide clinical treatment,associated risk factors,clinical m...BACKGROUND The incidence of mixed neuroendocrine-non-neuroendocrine neoplasms(MiNEN)is low.To improve our understanding of this rare tumor type and optimally guide clinical treatment,associated risk factors,clinical manifestations,and prognosis must be explored.AIM To identify risk factors that influence the prognosis of patients with gastroenteropancreatic MiNEN(GEP-MiNEN).METHODS We retrospectively analyzed the clinical data of 46 patients who were diagnosed with GEP-MiNEN at the First Affiliated Hospital of Bengbu Medical College(Anhui,China)between January 2013 and December 2017.Risk factors influencing the prognosis of the patients were assessed using Kaplan-Meier curves and cox regression models.We compared the results with 55 randomly selected patients with gastroenteropancreatic GEP neuroendocrine tumors,47 with neuroendocrine carcinomas(NEC),and 58 with poorly differentiated adenocarcinoma.RESULTS Among the 46 patients with GEP-MiNEN,thirty-five had gastric tumors,nine had intestinal tumors(four in the small intestine and five in the colon and rectum),and two had pancreatic tumors.The median age of the patients was 66(41-84)years,and the male-to-female ratio was 2.83.Thirty-three(71.7%)patients had clinical stage III and IV cancers.Distant metastasis occurred in 14 patients,of which 13 had metastasis to the liver.The follow-up period was 11-72 mo,and the median overall survival was 30 mo.Ki-67 index≥50%,high proportion of NEC,lymph node involvement,distant metastasis,and higher clinical stage were independent risk factors affecting the prognosis of patients with GEP-MiNEN.The median overall survival was shorter for patients with NEC than for those with MiNEN(14 mo vs 30 mo,P=0.001),but did not significantly differ from those with poorly differentiated adenocarcinoma and MiNEN(30 mo vs 18 mo,P=0.453).CONCLUSION A poor prognosis is associated with rare,aggressive GEP-MiNEN.Ki-67 index,tumor composition,lymph node involvement,distant metastasis,and clinical stage are important factors for patient prognosis.展开更多
Mixed neuroendocrine-non-neuroendocrine neoplasms(MiNENs)are rare mixed tumors containing both neuroendocrine(NE)and non-NE components.Each component must occupy at least 30%of the tumor volume by definition.Recent mo...Mixed neuroendocrine-non-neuroendocrine neoplasms(MiNENs)are rare mixed tumors containing both neuroendocrine(NE)and non-NE components.Each component must occupy at least 30%of the tumor volume by definition.Recent molecular evidence suggests MiNENs are clonal neoplasms and potentially harbor targetable mutations similar to conventional carcinomas.There have been multiple changes in the nomenclature and classification of MiNENs which has created some confusion among pathologists on how to integrate the contributions of each component in a MiNEN,an issue which in turn has resulted in confusion in communication with front-line treating oncologists.This mini review summarizes our current understanding of MiNENs and outline diagnosis,prognosis,and management of these neoplasms.The authors emphasize the importance of treating the most aggressive component of the tumor regardless of its percentage volume.展开更多
Mixed neuroendocrine-nonneuroendocrine neoplasms(Mi NENs)of the digestive tract are a rare heterogeneous group of tumors that present many challenges in terms of diagnosis and treatment.Over the years,the diagnostic c...Mixed neuroendocrine-nonneuroendocrine neoplasms(Mi NENs)of the digestive tract are a rare heterogeneous group of tumors that present many challenges in terms of diagnosis and treatment.Over the years,the diagnostic criteria,classification,and clinical behavior of these tumors have been the subjects of ongoing debate,and the various changes in their nomenclature have strengthened the challenges associated with Mi NENs.This review is performed to provide an understanding of the key factors involved in the evolution of the designation of these tumors as Mi NEN,highlight the current diagnostic criteria,summarize the latest data on pathogenesis and provide information on available treatments.Moreover,this work seeks to increase the awareness about these rare neoplasms by presenting the clinicopathological features and prognostic factors that play important roles in their behavior and discussing their different regions of origin in the gastrointestinal system(GIS).Currently,the Mi NEN category also includes tumors in the GIS with a nonneuroendocrine component and epithelial tumors other than adenocarcinoma,depending on the organ of origin.Diagnosis is based on the presence of both morphological components in more than 30%of the tumor.However,this value needs to be reconfirmed with further studies and may be a limiting factor in the diagnosis of Mi NEN by biopsy.Furthermore,available clinicopathological data suggest that the inclusion of amphicrine tumors in the definition of Mi NEN is not supportive and warrants further investigation.The diagnosis of these tumors is not solely based on immunohistochemical findings.They are not hybrid tumors and both components can act independently;thus,careful grading of each component separately is required.In addition to parameters such as the metastatic state of the tumor at the time of diagnosis and the feasibility of surgical resection,the aggressive potential of both components has paramount importance in the choice of treatment.Regardless of the organ of origin within the GIS,almost Mi NENs are tumors with poor prognosis and are frequently encountered in the elderly and men.They are most frequently reported in the colorectum,where data from molecular studies indicate a monoclonal origin;however,further studies are required to provide additional support for this origin.展开更多
BACKGROUND Mixed neuroendocrine-nonneuroendocrine neoplasm(MiNEN)is a rare tumor that occurs in the gastrointestinal tract and pancreas,usually composed of adenocarcinoma and neuroendocrine carcinoma.MiNEN occurring i...BACKGROUND Mixed neuroendocrine-nonneuroendocrine neoplasm(MiNEN)is a rare tumor that occurs in the gastrointestinal tract and pancreas,usually composed of adenocarcinoma and neuroendocrine carcinoma.MiNEN occurring in ampulla is even rarer.We report 4 cases of MiNEN in ampulla,combined with literature review to summarize the clinical features and treatment of the disease,in order to improve the understanding of the disease.CASE SUMMARY A retrospective analysis was performed in 4 cases of MiNEN of the ampulla diagnosed by pathology from 2014 to 2021.The 4 patients were all male,aged 67-81 years(average 72.25 years).Among them,2 patients had jaundice,1 patient had abdominal pain,and 1 patient had jaundice with abdominal pain as the first symptom.All 4 patients underwent enhanced CT or MRI,which all indicated that the tumors were located in the ampulla.Two patients underwent duodenoscopy,and a biopsy revealed ampullary adenocarcinoma.All 4 patients underwent radical pancreaticoduodenectomy.Four cases were followed up:One patient developed severe complications after the operation,his condition deteriorated,and he survived for 1 mo.In the other 3 patients,tumor recurrence was observed during follow-up,and 2 of them survived for 29 mo and 22 mo respectively.One case survived and is still being followed up.CONCLUSION MiNEN of the ampulla are extremely rare,lacking typical clinical symptoms and imaging features,and are usually diagnosed after postoperative histopathological and immunohistochemical examinations.The main treatment is radical surgical resection,which can be combined with chemotherapy.The best method of diagnosis and treatment needs further research.展开更多
BACKGROUND Gastric mixed neuroendocrine-non-neuroendocrine neoplasm(MiNEN),which consists of neuroendocrine and non-neuroendocrine components,is quite rare.Until now,most data on gastric MiNEN come from clinical cases...BACKGROUND Gastric mixed neuroendocrine-non-neuroendocrine neoplasm(MiNEN),which consists of neuroendocrine and non-neuroendocrine components,is quite rare.Until now,most data on gastric MiNEN come from clinical cases,without largescale retrospective studies or controlled clinical trials.Consequently,no consensus regarding the origin,molecular characteristics,or appropriate treatment of MiNEN has been reached so far.We conducted chemotherapy of irinotecan plus cisplatin(IP regimen)and surgery in two patients with gastric MiNEN,which had never been used in treating this kind of tumor,leading to their long-term survival for more than 3 and 7 years,respectively.CASE SUMMARY We present two patients(one male and one female)with gastric MiNEN,with the primary manifestation of recurrent upper abdominal pain.After they were referred to our hospital,a diagnosis of gastric MiNEN was defined with the help of CT scan,and histopathological and immunohistochemical examinations on the samples of gastrointestinal endoscopy or radical surgery.The male patient(case 1)were found to have metastases in the reginal lymph nodes and the left liver.He received four cycles of IP regimens first,then the gastrectomy and partial left liver resection,followed by additional two cycles of IP chemotherapy.The female patient(case 2)underwent a laparoscopic gastrectomy,and received six cycles of IP regimen.She was found to have metastatic lesions in the right lung 2 years after that,and underwent video-assisted thoracoscopic surgery(VATS)of the lower lobe of the right lung.The two patients have now survived for more than 3 years and 7 years,respectively,without any evidence of recurrence or metastases.CONCLUSION IP regimen,combined with curative-intent surgery if feasible,could be considered as the priority in the choice of front-line chemotherapy for gastric MiNEN.展开更多
BACKGROUND Neuroendocrine carcinoma(NEC)of the extrahepatic bile duct is very rare,and the treatment and prognosis are unclear.Herein,we report the case of a middleaged female with primary large cell NEC(LCNEC)of the ...BACKGROUND Neuroendocrine carcinoma(NEC)of the extrahepatic bile duct is very rare,and the treatment and prognosis are unclear.Herein,we report the case of a middleaged female with primary large cell NEC(LCNEC)of the common hepatic duct combined with distal cholangiocarcinoma(dCCA).Additionally,after a review of the relevant literature,we summarize and compare mixed neuroendocrine-nonneuroendocrine neoplasm(MiNEN)and pure NEC to provide a reference for selecting the appropriate treatment and predicting the prognosis of this rare disease.CASE SUMMARY A 62-year-old female presented to the hospital due to recurrent abdominal pain for 2 months.Physical examination showed mild tenderness in the upper abdomen and a positive Courvoisier sign.Blood tests showed elevated liver transaminase and carbohydrate antigen 199 levels.Imaging examination revealed node dissection was performed,and hepatic duct tumours were unexpectedly found during surgery.Pathology suggested poorly differentiated LCNEC(approximately 0.5 cm×0.5 cm×0.4 cm),Ki-67(50%),synaptophysin+,and chromogranin A+.dCCA pathology suggested moderately differentiated adenocarcinoma.The patient eventually developed lymph node metastasis in the liver,bone,peritoneum,and abdominal cavity and died 24 months after surgery.Gene sequencing methods were used to compare gene mutations in the two primary bile duct tumours.CONCLUSION The prognosis of MiNEN and pure NEC alone is different,and the selection of treatment options needs to be differentiated.展开更多
目的探讨肾混合性上皮间质肿瘤(m ixed ep ithelial and strom al tumor of the k idney,MESTK)的临床病理学特点、免疫表型和鉴别诊断。方法运用光镜和免疫组化方法分析4例MESTK,并复习有关文献。结果女性3例、男性1例,平均发病年龄为3...目的探讨肾混合性上皮间质肿瘤(m ixed ep ithelial and strom al tumor of the k idney,MESTK)的临床病理学特点、免疫表型和鉴别诊断。方法运用光镜和免疫组化方法分析4例MESTK,并复习有关文献。结果女性3例、男性1例,平均发病年龄为39岁。临床表现为腰痛、肉眼或镜下血尿。巨检肿瘤境界清楚,呈实性。镜检以不等量增生、囊性扩张腺上皮与不同排列方式的梭形细胞间质混合组成为特征。免疫表型为梭形细胞vim entin(3/3)、desm in(2/4)、SMA(3/4)、ER(2/3)、PR(3/3)呈阳性表达,不表达CD34(0/3)、S-100蛋白(0/2)、HMB45(0/2);上皮细胞CKpan(4/4)、EMA(4/4)呈阳性表达。结论MESTK是一种少见的肾良性混合性肿瘤,其诊断主要依靠组织病理学和免疫组化标记。展开更多
基金National Natural Science Foundation of China,No.81702596
文摘BACKGROUND Pancreatic mixed serous-neuroendocrine neoplasms(MSNNs)are mixed tumors containing two components with different pathologies,namely,pancreatic serous cystic neoplasm(PSCN)and pancreatic neuroendocrine tumor(PanNET).For MSNNs,diffuse PSCN involving the whole pancreas is extremely rare,with only eight previous case reports.CASE SUMMARY A 45-year-old Chinese woman,with a free previous medical history and no obvious symptoms,was found to have a pancreatic neoplasm and admitted to our hospital for further diagnosis in March 2018.Abdominal palpation revealed a painless,mobile mass in the epigastrium,and no abnormalities were observed in an examination of the nervous system and ocular system.A computed tomography scan showed multiple cystic lesions involving the whole pancreas ranging in diameter from 0.4 to 2 cm and also revealed an enhanced mass,2.2 cm in diameter,in the head of the pancreas.Moreover,multiple cysts were found in the kidneys bilaterally,and the right lobe of the liver contained a small cyst.A Whipple operation with total pancreatectomy and splenectomy was performed.A diagnosis of pancreatic MSNN was established,consisting of diffuse serous microcystic cystadenoma with a concomitant grade 2 PanNET.Of note,the patient had no personal or family history of Von Hippel-Lindau syndrome or other disease.CONCLUSION We report the first case of MSNN with a diffuse PSCN component involving the entire pancreas in a Chinese woman.It is important to be aware of its relationship with VHL syndrome,and close clinical follow-up is recommended.
文摘Mixed neuroendocrine non-neuroendocrine neoplasms(MiNENs)are a hetero-geneous group of malignant neoplasms that can settle in the gastroenteropan-creatic tract.They are composed of a neuroendocrine(NE)and a non-NE compo-nent in at least 30%of each tumour.The non-NE component can include different histological combinations of glandular,squamous,mucinous and sarcomatoid phenotypes,and one or both of the components can be low-or high grade malignant.Recent changes in the nomenclature of these neoplasms might lead to great deal of confusion,and the lack of specific clinical trials is the main reason why their management is difficult.The review aims to clarify the definition of MiNEN and analyze available evidence about their diagnosis and treatment options according to their location and extension through careful analysis of the available data.It would be important to reach a general consensus on their diagnosis in order to construct a classification that remains stable over time and facilitates the design of clinical trials that,due to their low incidence,will require long recruitment periods.
文摘Here we report a rare case of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs). A 51-year-old lady with no known family history of malignancies developed this rare type of malignancy without any active gastrointestinal symptoms. However, during a routine health check, physicians noticed a raised Carcinoembryonic Antigen (CEA) level and the patient subsequently was referred to the surgical department for further management. A colonoscopy and CECT abdomen were done and she was electively admitted for a left hemicolectomy operation for a splenic flexure tumour. The histopathological report revealed the tumour is a case of mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) [moderately differentiated adenocarcinoma and neuroendocrine tumour grade 3]. TNM (8th edition, 2016): pT3, pN2 (20/21), pMX. Both resection margins were clear from malignant cells. Colorectal MiNENs, constitute a rare group of gastrointestinal tumours composed of both neuroendocrine and non-neuroendocrine components. Given their non-diagnostic macroscopic features, specific histological features and lack of disease awareness which are responsible for the underestimated incidence and conflicting data. In this case, a multidisciplinary team approach is important in managing patients with this malignancy to achieve the best outcome.
文摘In this editorial we comment on the manuscript describing a case of adenocarcinoma mixed with a neuroendocrine carcinoma of the gastroesophageal junction.Mixed neuroendocrine and non-neuroendocrine neoplasms of the gastrointestinal system are rare heterogeneous group of tumors characterized by a high malignant potential,rapid growth,and poor prognosis.Due to the rarity of these cancers,the standard therapy is poorly defined.The diagnosis of these tumors is based on combination of morphological features,immunohistochemical and neuroendocrine and epithelial cell markers.Both endocrine and epithelial cell components can act independently of each other and thus,careful grading of each component separately is required.These cancers are aggressive in nature and the potential of each component has paramount importance in the choice of treatment and response.Regardless of the organ of origin,these tumors portend poor prognosis with increased proportion of neuroendocrine component.Multidisciplinary services and strategies are required for the management of these mixed malignancies to provide the best oncological outcomes.The etiopathogenesis of these mixed tumors remains obscure but poses interesting question.We briefly discuss a few salient points in this editorial.
文摘BACKGROUND Mixed neuroendocrine non-neuroendocrine neoplasm(MiNEN)is a rare diagnosis,mainly encountered in the gastro-entero-pancreatic tract.There is limited knowledge of its epidemiology,prognosis and biology,and the best management for affected patients is still to be defined.AIM To investigate clinical-pathological characteristics,treatment modalities and survival outcomes of a retrospective cohort of patients with a diagnosis of MiNEN.METHODS Consecutive patients with a histologically proven diagnosis of MiNEN were identified at 5 European centres.Patient data were retrospectively collected from medical records.Pathological samples were reviewed to ascertain compliance with the 2017 World Health Organisation definition of MiNEN.Tumour responses to systemic treatment were assessed according to the Response Evaluation Criteria in Solid Tumours 1.1.Kaplan-Meier analysis was applied to estimate survival outcomes.Associations between clinical-pathological characteristics and survival outcomes were explored using Log-rank test for equality of survivors functions(univariate)and Cox-regression analysis(multivariable).RESULTS Sixty-nine consecutive patients identified;Median age at diagnosis:64 years.Males:63.8%.Localised disease(curable):53.6%.Commonest sites of origin:colon-rectum(43.5%)and oesophagus/oesophagogastric junction(15.9%).The neuroendocrine component was;predominant in 58.6%,poorly differentiated in 86.3%,and large cell in 81.25%,of cases analysed.Most distant metastases analysed(73.4%)were occupied only by a poorly differentiated neuroendocrine component.Ninety-four percent of patients with localised disease underwent curative surgery;53%also received perioperative treatment,most often in line with protocols for adenocarcinomas from the same sites of origin.Chemotherapy was offered to most patients(68.1%)with advanced disease,and followed protocols for pure neuroendocrine carcinomas or adenocarcinomas in equal proportion.In localised cases,median recurrence free survival(RFS);14.0 months(95%CI:9.2-24.4),and median overall survival(OS):28.6 months(95%CI:18.3-41.1).On univariate analysis,receipt of perioperative treatment(vs surgery alone)did not improve RFS(P=0.375),or OS(P=0.240).In advanced cases,median progression free survival(PFS);5.6 months(95%CI:4.4-7.4),and median OS;9.0 months(95%CI:5.2-13.4).On univariate analysis,receipt of palliative active treatment(vs best supportive care)prolonged PFS and OS(both,P<0.001).CONCLUSION MiNEN is most commonly driven by a poorly differentiated neuroendocrine component,and has poor prognosis.Advances in its biological understanding are needed to identify effective treatments and improve patient outcomes.
基金National Key Research and Development Program(2016YFC0106500800)NationalMajor Scientific Instruments and Equipments Development Project of National Natural Science Foundation of China(81627805)+3 种基金National Natural Science Foundation of China-Guangdong Joint Fund Key Program(U1401254)National Natural Science Foundation of China Mathematics Tianyuan Foundation(12026602)Guangdong Provincial Natural Science Foundation Team Project(6200171)Guangdong Provincial Health Appropriate Technology Promotion Project(20230319214525105,20230322152307666).
文摘Augmented-and mixed-reality technologies have pioneered the realization of real-time fusion and interactive projection for laparoscopic surgeries.Indocyanine green fluorescence imaging technology has enabled anatomical,functional,and radical hepatectomy through tumor identification and localization of target hepatic segments,driving a transformative shift in themanagement of hepatic surgical diseases,moving away from traditional,empirical diagnostic and treatment approaches toward digital,intelligent ones.The Hepatic Surgery Group of the Surgery Branch of the Chinese Medical Association,Digital Medicine Branch of the Chinese Medical Association,Digital Intelligent Surgery Committee of the Chinese Society of ResearchHospitals,and Liver Cancer Committee of the Chinese Medical Doctor Association organized the relevant experts in China to formulate this consensus.This consensus provides a comprehensive outline of the principles,advantages,processes,and key considerations associated with the application of augmented reality and mixed-reality technology combined with indocyanine green fluorescence imaging technology for hepatic segmental and subsegmental resection.The purpose is to streamline and standardize the application of these technologies.
文摘BACKGROUND Mixed tumors of the colon and rectum,composed of a combination of epithelial and endocrine elements of benign and malignant potential are rare neoplasms.These can occur anywhere in the gastrointestinal tract and are often diagnosed incidentally.Though they have been a well-documented entity in the pancreas,where the exocrine-endocrine mixed tumors have been known for a while,recognition and accurate diagnosis of these tumors in the colon and rectum,to date,remains a challenge.This is further compounded by the different terminologies that have been attributed to these lesions over the years adding to increased confusion and misclassification.Therefore,dedicated literature reviews of these lesions in the colon and rectum are inconsistent and are predominantly limited to case reports and case series of limited case numbers.Though,most of these tumors are high grade and of advanced stage,intermediate and low grade lesions of these mixed tumors are also increasingly been reported.There are no established independent consensus based guidelines for the therapeutic patient management of these unique lesions.AIM To provide a comprehensive targeted literature review of these complex mixed tumors in the colon and rectum that chronicles the evolution over time with summarization of historical perspectives of terminology and to further our understanding regarding their pathogenesis including genomic landscape,clinicoradiological features,pathology,treatment,prognosis,the current status of the management of the primary lesions,their recurrences and metastases.METHODS A comprehensive review of the published English literature was conducted using the search engines PubMed,MEDLINE and GOOGLE scholar.The following search terms[“mixed tumors colon”OR mixed endocrine/neuroendocrine tumor/neoplasm/lesion colon OR adenocarcinoma and endocrine/neuroendocrine tumor colon OR mixed adenocarcinoma and endocrine/neuroendocrine carcinoma colon OR Amphicrine tumors OR Collision tumors]were used.Eligibility criteria were defined and all potential relevant items,including full articles and/or abstracts were independently reviewed,assessed and agreed upon items were selected for in-depth analysis.RESULTS In total 237 full articles/abstracts documents were considered for eligibility of which 45 articles were illegible resulting in a total of 192 articles that were assessed for eligibility of which 139 have been selected for reference in this current review.This seminal manuscript is a one stop article that provides a detailed outlook on the evolution over time with summarization of historical perspectives,nomenclature,clinicoradiological features,pathology,treatment,prognosis and the current status of the management of both the primary lesions,their recurrences and metastases.Gaps in knowledge have also been identified and discussed.An important outcome of this manuscript is the justified proposal for a new,simple,clinically relevant,non-ambiguous terminology for these lesions to be referred to as mixed epithelial endocrine neoplasms(MEENs).CONCLUSION MEEN of the colon and rectum are poorly understood rare entities that encompass an extensive range of heterogeneous tumors with a wide variety of combinations leading to tumors of high,intermediate or low grade malignant potential.This proposed new revised terminology of MEEN will solve the biggest hurdle of confusion and misclassification that plagues these rare unique colorectal neoplasms thus facilitating the future design of multi institutional prospective randomized controlled clinical trials to develop and evaluate newer therapeutic strategies that are recommended for continued improved understanding and personal optimization of clinical management of these unique colorectal neoplasms.
基金The Natural Science Foundation of Anhui Province,No.1908085MH275the Key Project of Science and Technology Development Foundation of Bengbu Medical College,No.BYKF201710the Graduate Innovation Program of Bengbu Medical College,No.Byycx20064.
文摘BACKGROUND The incidence of mixed neuroendocrine-non-neuroendocrine neoplasms(MiNEN)is low.To improve our understanding of this rare tumor type and optimally guide clinical treatment,associated risk factors,clinical manifestations,and prognosis must be explored.AIM To identify risk factors that influence the prognosis of patients with gastroenteropancreatic MiNEN(GEP-MiNEN).METHODS We retrospectively analyzed the clinical data of 46 patients who were diagnosed with GEP-MiNEN at the First Affiliated Hospital of Bengbu Medical College(Anhui,China)between January 2013 and December 2017.Risk factors influencing the prognosis of the patients were assessed using Kaplan-Meier curves and cox regression models.We compared the results with 55 randomly selected patients with gastroenteropancreatic GEP neuroendocrine tumors,47 with neuroendocrine carcinomas(NEC),and 58 with poorly differentiated adenocarcinoma.RESULTS Among the 46 patients with GEP-MiNEN,thirty-five had gastric tumors,nine had intestinal tumors(four in the small intestine and five in the colon and rectum),and two had pancreatic tumors.The median age of the patients was 66(41-84)years,and the male-to-female ratio was 2.83.Thirty-three(71.7%)patients had clinical stage III and IV cancers.Distant metastasis occurred in 14 patients,of which 13 had metastasis to the liver.The follow-up period was 11-72 mo,and the median overall survival was 30 mo.Ki-67 index≥50%,high proportion of NEC,lymph node involvement,distant metastasis,and higher clinical stage were independent risk factors affecting the prognosis of patients with GEP-MiNEN.The median overall survival was shorter for patients with NEC than for those with MiNEN(14 mo vs 30 mo,P=0.001),but did not significantly differ from those with poorly differentiated adenocarcinoma and MiNEN(30 mo vs 18 mo,P=0.453).CONCLUSION A poor prognosis is associated with rare,aggressive GEP-MiNEN.Ki-67 index,tumor composition,lymph node involvement,distant metastasis,and clinical stage are important factors for patient prognosis.
文摘Mixed neuroendocrine-non-neuroendocrine neoplasms(MiNENs)are rare mixed tumors containing both neuroendocrine(NE)and non-NE components.Each component must occupy at least 30%of the tumor volume by definition.Recent molecular evidence suggests MiNENs are clonal neoplasms and potentially harbor targetable mutations similar to conventional carcinomas.There have been multiple changes in the nomenclature and classification of MiNENs which has created some confusion among pathologists on how to integrate the contributions of each component in a MiNEN,an issue which in turn has resulted in confusion in communication with front-line treating oncologists.This mini review summarizes our current understanding of MiNENs and outline diagnosis,prognosis,and management of these neoplasms.The authors emphasize the importance of treating the most aggressive component of the tumor regardless of its percentage volume.
文摘Mixed neuroendocrine-nonneuroendocrine neoplasms(Mi NENs)of the digestive tract are a rare heterogeneous group of tumors that present many challenges in terms of diagnosis and treatment.Over the years,the diagnostic criteria,classification,and clinical behavior of these tumors have been the subjects of ongoing debate,and the various changes in their nomenclature have strengthened the challenges associated with Mi NENs.This review is performed to provide an understanding of the key factors involved in the evolution of the designation of these tumors as Mi NEN,highlight the current diagnostic criteria,summarize the latest data on pathogenesis and provide information on available treatments.Moreover,this work seeks to increase the awareness about these rare neoplasms by presenting the clinicopathological features and prognostic factors that play important roles in their behavior and discussing their different regions of origin in the gastrointestinal system(GIS).Currently,the Mi NEN category also includes tumors in the GIS with a nonneuroendocrine component and epithelial tumors other than adenocarcinoma,depending on the organ of origin.Diagnosis is based on the presence of both morphological components in more than 30%of the tumor.However,this value needs to be reconfirmed with further studies and may be a limiting factor in the diagnosis of Mi NEN by biopsy.Furthermore,available clinicopathological data suggest that the inclusion of amphicrine tumors in the definition of Mi NEN is not supportive and warrants further investigation.The diagnosis of these tumors is not solely based on immunohistochemical findings.They are not hybrid tumors and both components can act independently;thus,careful grading of each component separately is required.In addition to parameters such as the metastatic state of the tumor at the time of diagnosis and the feasibility of surgical resection,the aggressive potential of both components has paramount importance in the choice of treatment.Regardless of the organ of origin within the GIS,almost Mi NENs are tumors with poor prognosis and are frequently encountered in the elderly and men.They are most frequently reported in the colorectum,where data from molecular studies indicate a monoclonal origin;however,further studies are required to provide additional support for this origin.
基金Supported by Funded Project of"Mountain Climbing"Cultivation Project of Yijishan Hospital of Wannan Medical College,No.KPF2019011
文摘BACKGROUND Mixed neuroendocrine-nonneuroendocrine neoplasm(MiNEN)is a rare tumor that occurs in the gastrointestinal tract and pancreas,usually composed of adenocarcinoma and neuroendocrine carcinoma.MiNEN occurring in ampulla is even rarer.We report 4 cases of MiNEN in ampulla,combined with literature review to summarize the clinical features and treatment of the disease,in order to improve the understanding of the disease.CASE SUMMARY A retrospective analysis was performed in 4 cases of MiNEN of the ampulla diagnosed by pathology from 2014 to 2021.The 4 patients were all male,aged 67-81 years(average 72.25 years).Among them,2 patients had jaundice,1 patient had abdominal pain,and 1 patient had jaundice with abdominal pain as the first symptom.All 4 patients underwent enhanced CT or MRI,which all indicated that the tumors were located in the ampulla.Two patients underwent duodenoscopy,and a biopsy revealed ampullary adenocarcinoma.All 4 patients underwent radical pancreaticoduodenectomy.Four cases were followed up:One patient developed severe complications after the operation,his condition deteriorated,and he survived for 1 mo.In the other 3 patients,tumor recurrence was observed during follow-up,and 2 of them survived for 29 mo and 22 mo respectively.One case survived and is still being followed up.CONCLUSION MiNEN of the ampulla are extremely rare,lacking typical clinical symptoms and imaging features,and are usually diagnosed after postoperative histopathological and immunohistochemical examinations.The main treatment is radical surgical resection,which can be combined with chemotherapy.The best method of diagnosis and treatment needs further research.
文摘BACKGROUND Gastric mixed neuroendocrine-non-neuroendocrine neoplasm(MiNEN),which consists of neuroendocrine and non-neuroendocrine components,is quite rare.Until now,most data on gastric MiNEN come from clinical cases,without largescale retrospective studies or controlled clinical trials.Consequently,no consensus regarding the origin,molecular characteristics,or appropriate treatment of MiNEN has been reached so far.We conducted chemotherapy of irinotecan plus cisplatin(IP regimen)and surgery in two patients with gastric MiNEN,which had never been used in treating this kind of tumor,leading to their long-term survival for more than 3 and 7 years,respectively.CASE SUMMARY We present two patients(one male and one female)with gastric MiNEN,with the primary manifestation of recurrent upper abdominal pain.After they were referred to our hospital,a diagnosis of gastric MiNEN was defined with the help of CT scan,and histopathological and immunohistochemical examinations on the samples of gastrointestinal endoscopy or radical surgery.The male patient(case 1)were found to have metastases in the reginal lymph nodes and the left liver.He received four cycles of IP regimens first,then the gastrectomy and partial left liver resection,followed by additional two cycles of IP chemotherapy.The female patient(case 2)underwent a laparoscopic gastrectomy,and received six cycles of IP regimen.She was found to have metastatic lesions in the right lung 2 years after that,and underwent video-assisted thoracoscopic surgery(VATS)of the lower lobe of the right lung.The two patients have now survived for more than 3 years and 7 years,respectively,without any evidence of recurrence or metastases.CONCLUSION IP regimen,combined with curative-intent surgery if feasible,could be considered as the priority in the choice of front-line chemotherapy for gastric MiNEN.
基金Medical Health Science and Technology Project of Zhejiang Provincial Health Commission,No.2022KY1246Science and Technology Bureau of Jiaxing City,No.2023AZ31002 and No.2022AZ10009.
文摘BACKGROUND Neuroendocrine carcinoma(NEC)of the extrahepatic bile duct is very rare,and the treatment and prognosis are unclear.Herein,we report the case of a middleaged female with primary large cell NEC(LCNEC)of the common hepatic duct combined with distal cholangiocarcinoma(dCCA).Additionally,after a review of the relevant literature,we summarize and compare mixed neuroendocrine-nonneuroendocrine neoplasm(MiNEN)and pure NEC to provide a reference for selecting the appropriate treatment and predicting the prognosis of this rare disease.CASE SUMMARY A 62-year-old female presented to the hospital due to recurrent abdominal pain for 2 months.Physical examination showed mild tenderness in the upper abdomen and a positive Courvoisier sign.Blood tests showed elevated liver transaminase and carbohydrate antigen 199 levels.Imaging examination revealed node dissection was performed,and hepatic duct tumours were unexpectedly found during surgery.Pathology suggested poorly differentiated LCNEC(approximately 0.5 cm×0.5 cm×0.4 cm),Ki-67(50%),synaptophysin+,and chromogranin A+.dCCA pathology suggested moderately differentiated adenocarcinoma.The patient eventually developed lymph node metastasis in the liver,bone,peritoneum,and abdominal cavity and died 24 months after surgery.Gene sequencing methods were used to compare gene mutations in the two primary bile duct tumours.CONCLUSION The prognosis of MiNEN and pure NEC alone is different,and the selection of treatment options needs to be differentiated.
文摘目的探讨肾混合性上皮间质肿瘤(m ixed ep ithelial and strom al tumor of the k idney,MESTK)的临床病理学特点、免疫表型和鉴别诊断。方法运用光镜和免疫组化方法分析4例MESTK,并复习有关文献。结果女性3例、男性1例,平均发病年龄为39岁。临床表现为腰痛、肉眼或镜下血尿。巨检肿瘤境界清楚,呈实性。镜检以不等量增生、囊性扩张腺上皮与不同排列方式的梭形细胞间质混合组成为特征。免疫表型为梭形细胞vim entin(3/3)、desm in(2/4)、SMA(3/4)、ER(2/3)、PR(3/3)呈阳性表达,不表达CD34(0/3)、S-100蛋白(0/2)、HMB45(0/2);上皮细胞CKpan(4/4)、EMA(4/4)呈阳性表达。结论MESTK是一种少见的肾良性混合性肿瘤,其诊断主要依靠组织病理学和免疫组化标记。