BACKGROUND: The management of metastatic disease in pancreatic endocrine tumors (PETs) demands a multidisciplinary approach and the cooperation of several medical specialties. The role of surgery is critical, even whe...BACKGROUND: The management of metastatic disease in pancreatic endocrine tumors (PETs) demands a multidisciplinary approach and the cooperation of several medical specialties. The role of surgery is critical, even when a radical excision cannot always be achieved. DATA SOURCES: A PubMed search of relevant articles published up to February 2011 was performed to identify current information about PET liver metastases regarding diagnosis and management, with an emphasis on surgery. RESULTS: The early diagnosis of metastases and their accurate localization, most commonly in the liver, is very important. Surgical options include radical excision, and palliative excision to relieve symptoms in case of failure of medical treatment. The goal of the radical excision is to remove the primary tumor bulk and all liver metastases at the same time, but unfortunately it is not feasible in most cases. Palliative excisions include aggressive tumor debulking surgeries in well-differentiated carcinomas, trying to remove at least 90% of the tumor mass, combined with other additional destructive techniques such as hepatic artery embolization or chemoembolization to treat metastases or chemoembolization to relieve symptoms in cases of rapidly growing tumors. The combination of chemoembolization and systemic chemotherapy results in better response and survival rates. Other local destructive techniques include ethanol injection, cryotherapy and radiofrequency ablation. CONCLUSION: It seems that the current management of PETs can achieve important improvements, even in advanced cases.展开更多
AIM:To investigate differential points of solid-pseudo-papillary neoplasm (SPN) of the pancreas and pancre-atic endocrine tumor (PET).METHODS:Ten cases of SPN and fourteen cases of PET were studied in this retrospecti...AIM:To investigate differential points of solid-pseudo-papillary neoplasm (SPN) of the pancreas and pancre-atic endocrine tumor (PET).METHODS:Ten cases of SPN and fourteen cases of PET were studied in this retrospective study. Clinical and pathologic features,immunostaining reactions and β-catenin gene mutations were analyzed.RESULTS:The mean age of SPN patients was 25.6 years and these patients had no specific symptoms. The mean diameter of the tumors was 11.0 cm,9/10 cases were cystic or a mixture of solid and cystic structures,and there was hemorrhage and necrosis on the cut surface in 8/10 (80%) cases. Characteristic pseudo-papillary structure and discohesive appearance of the neoplastic cells were observed in all 10 (100%) cases. The results of immunostaining showed that nuclear expression of β-catenin and loss of E-cadherin in all the cases,was only seen in SPN. Molecular studies discov-ered that 9/10 (90%) cases harbored a point mutation of exon 3 in β-catenin gene. On the other hand,the mean age of PET patients was 43.1 years. Eight of 14 cases presented with symptoms caused by hypoglyce-mia,and the other 6 cases presented with symptoms similar to those of SPN. The mean size of the tumors was 2.9 cm,most of the tumors were solid,only 3/14 (21%) were a mixture of solid and cystic structures,and macroscopic hemorrhage and necrosis were much less common (3/14,21%). Histologically,tumor cells were arranged in trabecular,acinar or solid patterns and demonstrated no pseudopapillary structure and discohesive appearance in all 14 (100%) cases. The results of immunostaining and mutation detection were completely different with SPN that membrane and cytoplastic expression of β-catenin without loss of E-cadherin,as well as no mutation in β-catenin gene in all the cases. CONCLUSION:Both macroscopic and microscopic features of SPN are quite characteristic. It is not difficult to distinguish it from PET. If necessary,immunos-taining of β-catenin and E-cadherin is quite helpful to make the differential diagnosis.展开更多
AIM： To investigate the expression of cyclin-dependent kinase inhibitors p21 and p27 in gastric well differentiated endocrine tumors （GWDET） （ECLocell carcinoids）.METHODS： The expressions of p21 and p27 were exa...AIM： To investigate the expression of cyclin-dependent kinase inhibitors p21 and p27 in gastric well differentiated endocrine tumors （GWDET） （ECLocell carcinoids）.METHODS： The expressions of p21 and p27 were examined immunhistochemically in endoscopic biopsy specimens from 16 patients matching the diagnostic criteria of GWDET. Percentage of positive nuclear staining either weak or strong was noted. The association of immunoexpressions with age, gender, tumor localization, multifocality and accompanying chronic atrophic gastritis, neuroendocrine cell hyperplasia （NEH）, neuroendocrine dysplasia （NED）, intestinal metaplasia （IM）, Ki-67 proliferation index and clinical outcome were also evaluated.RESULTS： All cases expressed p27 with a mean expression score of 43.6%, while 31.3% of the cases showed any p21 expression, p21 and p27 immunoexpressions were significantly correlated with each other （P 〈 0.01）, and the p21-expressing group had higher p27 expression scores （68% vs 22%）. p21 and p27 expressions were lower in women, in non-atrophic mucosa and cases whose tumors were located somewhere other than fundus without submucosal extension. On contrary, p21 and p27 expressions were higher in males and the patients with submucosal extension and atrophic gastritis. Cases presenting lower p27 scores had solitary tumors showing neither NEH-NED nor IM. Despite, cases with lower p21 expression presented multifocal tumors accompanied by NEH-NED. However, no correlation of p21 and p27 expressions was found with age and Ki-67 expression.CONCLUSION： p27 is widely expressed in GWDETs, while p21 expression is sparse and observed in two thirds of the cases. Loss of p21 and p27 expressions may be correlated with different carcinoid tumor subtypes; however,more studies are needed to assess the role of these prospective markers in gastrointestinal endocrine tumors.展开更多
BACKGROUND Hepatectomy is the first choice for treating neuroendocrine tumor liver metastases.However,most patients with neuroendocrine tumor liver metastases are not suitable for hepatectomy.Ablation combined with he...BACKGROUND Hepatectomy is the first choice for treating neuroendocrine tumor liver metastases.However,most patients with neuroendocrine tumor liver metastases are not suitable for hepatectomy.Ablation combined with hepatectomy can be an alternative to liver resection.AIM To explore the clinical effect of microwave ablation combined with hepatectomy for the treatment of neuroendocrine tumor liver metastases.METHODS In this study,the data of patients who underwent microwave ablation combined with hepatectomy for the treatment of neuroendocrine tumor liver metastases from June 2015 to January 2018 were reviewed.Before the operation,the patients did not receive any treatment for liver neuroendocrine tumors.After a multidisciplinary expert group discussion,all patients were deemed unsuitable for liver resection.All patients were diagnosed with neuroendocrine tumors by pathology.The overall survival time and progression-free survival time were followed by telephone calls and outpatient visits after surgery.RESULTS Eleven patients with neuroendocrine tumor liver metastases were treated by microwave ablation combined with hepatectomy between June 2015 and January 2018.The median number of liver metastatic nodules was 4(range,2 to 43).The median number of lesions resected was 1(range,1 to 18),and the median number of lesions ablated was 3(range,1 to 38).The mean operation time was 405.6(±39.4)min.The median intraoperative blood loss was 600 mL(range,50 to 3000).Ten patients had a fever after surgery.The median duration of fever was 3 d(range,0 to 21).Elevated bilirubin levels occurred in all patients after surgery.The median bilirubin on the first day after surgery was 28.5(range,10.7 to 98.9)μmol/L.One patient developed respiratory failure,renal insufficiency,and pneumonia after the operation.No patient died postoperatively during hospitalization.The mean overall survival time after surgery was 34.1(±3.7)mo,and the median progression-free survival time was 8(range,2 to 51)mo.One year after surgery,ten patients survived and five patients survived without progression.Three year after surgery,eight patients survived and two patients survived without progression.CONCLUSION Microwave ablation combined with hepatectomy not only makes the patients obtain a survival rate similar to that of patients undergoing hepatectomy,but also has a low incidence of postoperative complications.展开更多
We report 2 cases of pancreatic endocrine tumors that caused obstruction of the main pancreatic duct(MPD).A 49-year-old asymptomatic man was referred to our institution because dilation of the MPD was revealed by abdo...We report 2 cases of pancreatic endocrine tumors that caused obstruction of the main pancreatic duct(MPD).A 49-year-old asymptomatic man was referred to our institution because dilation of the MPD was revealed by abdominal ultrasonography(US).No tumor was detected by endoscopic ultrasonography,computed tomography(CT),and magnetic resonance imaging(MRI).The diameter of the MPD was > 20 mm at the body,and no dilation was noted at the head.Although malignancy was not confirmed through cytology or imaging,pancreatic cancer was strongly suspected.Pancreaticoduo-denectomy was performed.Pathological and immunohistochemical examination revealed a 5 mm × 3 mm serotonin-positive endocrine tumor.Fibrosis was present around the MPD and seemed to cause stricture.A 32-year-old asymptomatic man had elevated serum amylase,and US demonstrated dilation of the MPD.No tumor was detected by CT and MRI.Pancreatic cancer was suspected due to stricture and dilation of the MPD.Pancreatectomy of middle part of pancreas was performed.Pathological and immunohistochemical examination revealed a serotonin-positive endocrine tumor sized 5 mm × 4 mm.We report 2 cases of serotonin-positive pancreatic endocrine tumors that caused stricture of the MPD in spite of the small size of the tumor.展开更多
Objective： The aim of our study was to analysis the pictures of conventional magnetic resonance imaging （MR）, diffusion-weighted imaging （DWl） and dynamic enhanced magnetic resonance imaging （DEMRI） of pancreat...Objective： The aim of our study was to analysis the pictures of conventional magnetic resonance imaging （MR）, diffusion-weighted imaging （DWl） and dynamic enhanced magnetic resonance imaging （DEMRI） of pancreatic endocrine tumors （PETs）, and evaluate diagnostic value of MR, DWl and DEMRI for diagnosing PETs. Methods： DWl and DEMRI scanning toward 13 patients with PETs being confirmed by surgical pathology before surgery on the basis of conventional MR scanning were carried out, and MR findings was analyzed retrospectively. Results： Of 13 patients with PETs there was 11 cases with single lesion, 2 with multiple, and had 15 lesions altogether, of which there were 3 lesions in pancreatic head, 1 in its neck, 2 in its body, 4 in its body and tail, 5 in its tail. MR findings： （1） T1WI signal was low or slightly lower （9/15）, and equal ones （5/15）; （2） T2WI showed high or slightly higher signal （10/15）, and equal ones （5/15）; （3） T1WI with fat suppression： the signal was low （11/15）, mixed signal （2/15）, and equal ones （2/15）; （4） DWI： normal pancreatic tissue exhibited homogeneous intermediate signal, all 15 lesions were high or slightly higher signal, the measured ADC values of tissue of PETs was （1.124 ± 0.252） × 104 mm2/s, and the ADC value of normal pancreatic tissue （1.873 ± 0.157） × 10^3 mm2/s; （5） Enhanced （M3D/LAVA） scanning： among 13 patients with PETs there were 12 pancreatic lesions with significantly enhanced signals in the arterial phase in all 15, and significantly higher than normal pancreatic tissue, and two slight enhancement was slightly higher signal; and 1 no enhancement. Enhanced pattern： homogeneous enhancement were 6 lesions, and the heterogeneous 4, and the edge ring 5. Conclusion： MR and DWl combining with DEMRI help qualitative diagnosis of pancreatic endocrine tumors.展开更多
Autoimmune pancreatitis(AIP) is a rare cause of recurrent acute pancreatitis or chronic pancreatitis in middleaged patients,and is characterised by a marked infiltration of lymphocytes and plasma cells in pancreatic t...Autoimmune pancreatitis(AIP) is a rare cause of recurrent acute pancreatitis or chronic pancreatitis in middleaged patients,and is characterised by a marked infiltration of lymphocytes and plasma cells in pancreatic tissue.Diagnosis of focal forms can be diff icult as AIP may mimic pancreatic adenocarcinoma.Pediatric cases of AIP are exceptional.We report the case of a 15-yearold girl who had a focal AIP and associated cholangitis,with a very unusual vascularized mass that mimicked a pancreatic endocrine tumor.The diagnosis was obtained by a pancreatic biopsy,thus avoiding surgical resection,and all the clinical,biological and radiological abnormalities resolved after steroid therapy with 6 mo of follow-up.展开更多
To investigate clinicopathological features,diagnosis and treatment of malignant pancreatic tumors.Methods A retrospective clinical analysis was made in 27 cases of malignant pancreatic tumors admitted from 1989 to 20...To investigate clinicopathological features,diagnosis and treatment of malignant pancreatic tumors.Methods A retrospective clinical analysis was made in 27 cases of malignant pancreatic tumors admitted from 1989 to 2003.Results Tumors were surgically resected in 22 patients.Tumor infiltration into surrounding organs was found in 13 cases.Four patients had lymph nodes metastasis and 5 had liver metastasis.Two cases were found tumor embolus in vascular or lymph tube.Neural invasion was found in 3.Conclusion Malignant pancreatic tumors has good prognosis.Aggressive attempts of surgical management should be performed.6 refs,1 tab.展开更多
This review delved into the intricate relationship between circadian clocks and physiological processes,emphasizing their critical role in maintaining homeo-stasis.Orchestrated by interlocked clock genes,the circadian...This review delved into the intricate relationship between circadian clocks and physiological processes,emphasizing their critical role in maintaining homeo-stasis.Orchestrated by interlocked clock genes,the circadian timekeeping system regulates fundamental processes like the sleep-wake cycle,energy metabolism,immune function,and cell proliferation.The central oscillator in the hypothalamic suprachiasmatic nucleus synchronizes with light-dark cycles,while peripheral tissue clocks are influenced by cues such as feeding times.Circadian disruption,linked to modern lifestyle factors like night shift work,correlates with adverse health outcomes,including metabolic syndrome,cardiovascular diseases,infec-tions,and cancer.We explored the molecular mechanisms of circadian clock genes and their impact on metabolic disorders and cancer pathogenesis.Specific associ-ations between circadian disruption and endocrine tumors,spanning breast,ovarian,testicular,prostate,thyroid,pituitary,and adrenal gland cancers,are highlighted.Shift work is associated with increased breast cancer risk,with PER genes influencing tumor progression and drug resistance.CLOCK gene expression correlates with cisplatin resistance in ovarian cancer,while factors like aging and intermittent fasting affect prostate cancer.Our review underscored the intricate interplay between circadian rhythms and cancer,involving the regulation of the cell cycle,DNA repair,metabolism,immune function,and the tumor microenvir-onment.We advocated for integrating biological timing into clinical consider-ations for personalized healthcare,proposing that understanding these connec-tions could lead to novel therapeutic approaches.Evidence supports circadian rhythm-focused therapies,particularly chronotherapy,for treating endocrine tumors.Our review called for further research to uncover detailed connections between circadian clocks and cancer,providing essential insights for targeted treatments.We emphasized the importance of public health interventions to mitigate lifestyle-related circadian disruptions and underscored the critical role of circadian rhythms in disease mechanisms and therapeutic interventions.展开更多
Background Pancreatic endocrine tumors （PETs） are rare and their surgical treatment is often debated. The purpose of this retrospective study was to analyze the diagnosis and surgical strategy of functioning and non...Background Pancreatic endocrine tumors （PETs） are rare and their surgical treatment is often debated. The purpose of this retrospective study was to analyze the diagnosis and surgical strategy of functioning and non-functioning PETs. Methods From May 1980 to March 2006, 36 patients with pancreatic endocrine tumors at the Second Affiliated Hospital of Zhejiang University were retrospectively studied. Results Among the 36 patients, 29 （81%） had functioning tumors, and 7 （19%） had nonfunctioning tumors. Ninety-two percent of insulinomas were benign, whereas 4 （57%） of nonfunctioning PETs were malignant. The size of functioning tumors was （2.3i-0.3） cm, that of nonfunctioning tumors was less than （5.1^-0.5） cm. The combination CT and transabdominal ultrasonography resulted in a diagnostic sensitivity of 84%. Thirty-three primary lesions were precisely located in 32 patients （89%）. Atypical tumor resection was performed for 73% of functioning tumors, while typical pancreatectomy was performed for 6 （85%） of nonfunctioning tumors. Moreover, 5 liver resections and 1 lymph node dissection were performed. During the follow-up, fifteen complications occurred in 12 （36%） patients after operation. The 5-year survival rate for patients with benign tumors was 92% compared to 50% for those with malignant tumors. Surgical cure was achieved in 95% of patients with benign insulinomas. Conclusions Surgical strategy for PETs depends on the size and location of the tumor and the risk of malignancy. The optimal surgical procedure is key to prevent postoperative complication. Radical resection including initial and metastatic lesion may benefit patients with maliqnant PETs.展开更多
Pancreatic endocrine tumors are rare but are among the most common neuroendocrine neoplasms of the abdomen.At diagnosis many of them are already advanced and diff icult to treat.We report on an initially inoperable ma...Pancreatic endocrine tumors are rare but are among the most common neuroendocrine neoplasms of the abdomen.At diagnosis many of them are already advanced and diff icult to treat.We report on an initially inoperable malignant pancreatic endocrine tumor in a 33-year-old woman,who received neoadjuvant peptide receptor radionuclide therapy(PRRT)as firstline treatment.This resulted in a signif icant downstaging of the tumor and allowed its subsequent complete surgical removal.Follow-up for eighteen months revealed a complete remission.This is the first report on neoadjuvant PRRT in a neuroendocrine neoplasm with subsequent successful complete resection.展开更多
Objective: To study the express of some nonhormonal antigens in pancreatic endocrine tumors. Methods: The nonhormonal antigens including Alpha subunit of human chorionic gonadotropin (α HCG), progesterone receptor...Objective: To study the express of some nonhormonal antigens in pancreatic endocrine tumors. Methods: The nonhormonal antigens including Alpha subunit of human chorionic gonadotropin (α HCG), progesterone receptors (PR), 7B2, HISL 19, in normal pancreatic islets and in 52 cases of pancreatic endocrine tumors (PET) were investigated by immunohistochemistry. Results: It was found that HCG can be detected in PET but not in normal islet cells. HCG immunoreactivity was expressed by 3 of 28 (10.7%) benign PET and by 14 of 24 (58.3%) malignant PET.PR was found by 20 of 28(71.4%) benign PET and by 7 of 24 (29%) malignant PET. 7B2 was detected by 23 of 28 (82.1%) benign PET and by 13 of 24 (54.2%) malignant PET. HISL 19 was appeared by 23 of 28 benign PET and by 11 of 24 (46%) malignant PET. Golgitype persisted in 87.5% malignant tumors. Conclusion: The assay of nonhormonal antigens may be well defined the clinico pathological characteristics of PET.展开更多
The antigen Alpha subunit of human chorionic gonadotropin (α HCG) and a HCGmRNA in pancreatic endocrine tumors (PET) were investigated by immuno histochemistry and in situ hybridization. It was found that α HC...The antigen Alpha subunit of human chorionic gonadotropin (α HCG) and a HCGmRNA in pancreatic endocrine tumors (PET) were investigated by immuno histochemistry and in situ hybridization. It was found that α HCG can be detected in PET buy not in normal islet cells. α HCG immunoreactivity was expressed by 5 of 28 (18%) in benign PET and 14 of 24 (58.3%) in malignant PET. Using in situ hybridization of α HCGmRNA, a strong signal in PET was obtained. The clinico pathological significance of α HCG in PET was discussed.展开更多
Large cell neuroendocrine carcinoma (LCNEC) in the biliary system is a poorly differentiated, high-grade neuroendocrine tumor. These tumors exhibit aggressive behavior and an increased tendency for early nodal and dis...Large cell neuroendocrine carcinoma (LCNEC) in the biliary system is a poorly differentiated, high-grade neuroendocrine tumor. These tumors exhibit aggressive behavior and an increased tendency for early nodal and distant metastases. Herein, we report an unusual case of a pure primary LCNEC of the common bile duct (CBD). A 75-year-old female presented with nausea and jaundice. The patient underwent a CBD excision with lymph node dissection. Upon histological and immunohistochemical examination, the tumor exhibited pure large cell-type neuroendocrine features. Metastases were noted in two of the eight lymph nodes. The patient was administered adjuvant chemotherapy. The patient’s cancer recurred 7 mo after surgery, and the patient died from liver failure 5 mo after recurrence. The prognosis of LCNEC of CBD remains poor despite curative resection and adjuvant chemotherapy. The role of additional therapies, such as multimodal treatment including radiation therapy, must be further studied to improve the prognoses of patients.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:Carcinoid tumors of the extrahepatic biliary tree are extremely rare malignancies,accounting for 0.2%-2%of all gastrointestinal carcinoid tumors,while carcinoids of the cystic duct are an uncommon entity an...BACKGROUND:Carcinoid tumors of the extrahepatic biliary tree are extremely rare malignancies,accounting for 0.2%-2%of all gastrointestinal carcinoid tumors,while carcinoids of the cystic duct are an uncommon entity and an extremely unusual cause of bile duct obstruction.METHODS:After an extensive literature review,we retrospectively analysed 61 cases of carcinoid tumor of the biliary tree as well as one additional case of a 60-yearold female with symptoms and laboratory/imaging examination findings compatible with those of a malignant biliary tract obstruction.At laparotomy,resection of the gallbladder and common bile duct was performed.Histological study revealed a well-differentiated neuroendocrine carcinoma of the cystic duct.The patient remained disease-free at 16 months.RESULTS:Our presentation is the seventh case reported in the world literature.Compared to cholangiocarcinoma,analysis of the reviewed group indicates an increased incidence of extrahepatic carcinoid tumors in younger persons along with a slight female predominance.Statistically,the most common anatomic location is the common bile duct,followed by the perihilar region and the cystic duct.Jaundice is the most common finding.Curative surgery was realized in the majority of cases and longterm disease-free survival was achieved when surgery was curative.CONCLUSIONS:Carcinoid tumors obstructing the biliary tree are extremely difficult to diagnose preoperatively,and nearly impossible to differentiate from non-neuroendocrine tumors.As surgery offers the only potential cure for both biliary carcinoids and cholangiocarcinoma,we recommend aggressive surgical therapy as the treatment of choice in every case of potentially resectable biliary tumor.展开更多
Gastric carcinoids(GCs),which originate from gastric enterochromaffin-like(ECL) mucosal cells and account for 2.4% of all carcinoids,are found increasingly in the course of upper gastrointestinal tract endoscopy.Curre...Gastric carcinoids(GCs),which originate from gastric enterochromaffin-like(ECL) mucosal cells and account for 2.4% of all carcinoids,are found increasingly in the course of upper gastrointestinal tract endoscopy.Current nosography includes those occurring in chronic conditions with hypergastrinemia,as the type 1 associated with chronic atrophic gastritis,and the type 2 associated with Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1,and type 3,which is unrelated to hypergastrinemia and is frequently malignant,with distant metastases.The optimal clinical approach to GCs remains to be elucidated,depending upon type,size and number of carcinoids.While there is agreement concerning the treatment of type 3 carcinoids,for types 1 and 2,current possibilities include simple surveillance,endoscopic polypectomy,surgical excision,associated or not with surgical antrectomy,or total gastrectomy.Moreover,the recent introduction of somatostatin analogues represents a therapeutic option of possibly outstanding relevance.展开更多
Gastrinomas are defined as gastrin secreting tumors that are associated with Zollinger-Ellison syndrome （ZES）. ZES is characterized by elevated fasting gastrin serum levels, positive secretin stimulation test and cl...Gastrinomas are defined as gastrin secreting tumors that are associated with Zollinger-Ellison syndrome （ZES）. ZES is characterized by elevated fasting gastrin serum levels, positive secretin stimulation test and clinical symptoms such as recurrent peptic ulcer disease, gastroesophageal reflux disease and occasional diarrhea. Genetically, nonhereditary （sporadic） gastrinomas are distinguished from hereditary gastrinomas, which are associated with multiple endocrine neoplasia type 1 （MEN1） syndrome. In general, duodenal gastrinomas are small and solitary if they are sporadic and multiple as well as hereditary. The sporadic gastrinomas occur in the duodenum or in the pancreas while the hereditary gastrinomas almost all occur in the duodenum. Our series of 77 sporadic duodenal neuroendocrine tumors （NETs） includes 18 patients （23.4%） with gastrinomas and ZES. Of 535 sporadic NETs in the pancreas collected from the NET archives of the departments of pathology in Zürich, Switzerland, and Kiel, Germany, 24 patients （4.5%） suffered from sporadic pancreatic gastrinomas and ZES. These NETs have to be distinguished from tumors with immunohistochemical positivity for gastrin but without evidence of ZES. An additional 19 patients suffered from MEN1 and ZES. These patients showed exclusively duodenal gastrinomas, but not pancreatic gastrinomas. The prognosis of sporadic and MEN1- associated duodenal gastrinomas is better than that of pancreatic gastrinomas, since they progress slowly to liver metastasis. In summary, sporadic and MEN1- associated gastrinomas in the duodenum and pancreas show different clinico-pathological and genetic features. The incidence of sporadic duodenal gastrin-producing tumors is increasing, possibly due to optimized diagnostic procedures. In contrast, pancreatic MEN1- associated gastrinomas seem to be extremely rare. A considerable subset of tumors with immunohistochemical expression of gastrin but without evidence of ZES should be designated as functionally inactive NETs expressing gastrin, but not as gastrinomas.展开更多
Objective To explore tissue expression of cyclin-dependent kinase inhibitor p27Kip1 andβ-catenin in multiple endocrine neoplasia type1（MEN1）-related parathyroid tumors（MHPT）.Methods Immunohistochemistry was perfo...Objective To explore tissue expression of cyclin-dependent kinase inhibitor p27Kip1 andβ-catenin in multiple endocrine neoplasia type1（MEN1）-related parathyroid tumors（MHPT）.Methods Immunohistochemistry was performed to analyze the expression of p27Kip1 andβ-catenin in parathyroid glands from 31 subjects展开更多
von Hippel-Lindau(VHL)disease is a rare,inherited neoplastic disease characterized by hemangioblastomas(HBL)of the central nervous system(CNS),retinal angiomas,renal cell carcinomas(RCC),pancreatic endocrine tumors(PE...von Hippel-Lindau(VHL)disease is a rare,inherited neoplastic disease characterized by hemangioblastomas(HBL)of the central nervous system(CNS),retinal angiomas,renal cell carcinomas(RCC),pancreatic endocrine tumors(PETs),pheochromocytomas,paragangliomas,and visceral cysts.We encountered a large VHL family in northwest China and conducted a systematic screening of the family members based on their epidemiological and clinical characteristics.A self-designed questionnaire was used to collect the general sociodemographic and health information of the family members.For the preliminary family screening,physical examination and abdomen B ultrasonography were performed.The suspected patients were subjected to cranial computerized tomography and fundus examination.The clinical data of the patients with confirmed VHL disease were collected from hospital records.A total of 63 lineal descendants in six generations were observed in the family(generations O,A,B,C,D,E),including 9 dead suspected cases(6 males,3 females)and 10 living cases(2 males,8 females).Among the 10 living cases,4,2,1,3,4,8,and 2 manifested HBLs of the CNS,PETs,RCC,pancreatic cysts,renal cysts,pheochromocytomas(4 hemi and 4 bilateral),and paragangliomas,respectively.Data showed that the morbidity of VHL disease in generation C was lower than that in generation B,but the age of onset was younger.This study is the first to report VHL disease in northwest China and VHL-associated PET cases in Chinese.Therefore,follow-up checkups of the family should be focused on younger generations.Proper family screening protocols should be followed for the treatment of patients with VHL disease.展开更多
文摘BACKGROUND: The management of metastatic disease in pancreatic endocrine tumors (PETs) demands a multidisciplinary approach and the cooperation of several medical specialties. The role of surgery is critical, even when a radical excision cannot always be achieved. DATA SOURCES: A PubMed search of relevant articles published up to February 2011 was performed to identify current information about PET liver metastases regarding diagnosis and management, with an emphasis on surgery. RESULTS: The early diagnosis of metastases and their accurate localization, most commonly in the liver, is very important. Surgical options include radical excision, and palliative excision to relieve symptoms in case of failure of medical treatment. The goal of the radical excision is to remove the primary tumor bulk and all liver metastases at the same time, but unfortunately it is not feasible in most cases. Palliative excisions include aggressive tumor debulking surgeries in well-differentiated carcinomas, trying to remove at least 90% of the tumor mass, combined with other additional destructive techniques such as hepatic artery embolization or chemoembolization to treat metastases or chemoembolization to relieve symptoms in cases of rapidly growing tumors. The combination of chemoembolization and systemic chemotherapy results in better response and survival rates. Other local destructive techniques include ethanol injection, cryotherapy and radiofrequency ablation. CONCLUSION: It seems that the current management of PETs can achieve important improvements, even in advanced cases.
基金Supported by Department of Pathology, Xiangya Basic Medical School, Central-south University
文摘AIM:To investigate differential points of solid-pseudo-papillary neoplasm (SPN) of the pancreas and pancre-atic endocrine tumor (PET).METHODS:Ten cases of SPN and fourteen cases of PET were studied in this retrospective study. Clinical and pathologic features,immunostaining reactions and β-catenin gene mutations were analyzed.RESULTS:The mean age of SPN patients was 25.6 years and these patients had no specific symptoms. The mean diameter of the tumors was 11.0 cm,9/10 cases were cystic or a mixture of solid and cystic structures,and there was hemorrhage and necrosis on the cut surface in 8/10 (80%) cases. Characteristic pseudo-papillary structure and discohesive appearance of the neoplastic cells were observed in all 10 (100%) cases. The results of immunostaining showed that nuclear expression of β-catenin and loss of E-cadherin in all the cases,was only seen in SPN. Molecular studies discov-ered that 9/10 (90%) cases harbored a point mutation of exon 3 in β-catenin gene. On the other hand,the mean age of PET patients was 43.1 years. Eight of 14 cases presented with symptoms caused by hypoglyce-mia,and the other 6 cases presented with symptoms similar to those of SPN. The mean size of the tumors was 2.9 cm,most of the tumors were solid,only 3/14 (21%) were a mixture of solid and cystic structures,and macroscopic hemorrhage and necrosis were much less common (3/14,21%). Histologically,tumor cells were arranged in trabecular,acinar or solid patterns and demonstrated no pseudopapillary structure and discohesive appearance in all 14 (100%) cases. The results of immunostaining and mutation detection were completely different with SPN that membrane and cytoplastic expression of β-catenin without loss of E-cadherin,as well as no mutation in β-catenin gene in all the cases. CONCLUSION:Both macroscopic and microscopic features of SPN are quite characteristic. It is not difficult to distinguish it from PET. If necessary,immunos-taining of β-catenin and E-cadherin is quite helpful to make the differential diagnosis.
文摘AIM： To investigate the expression of cyclin-dependent kinase inhibitors p21 and p27 in gastric well differentiated endocrine tumors （GWDET） （ECLocell carcinoids）.METHODS： The expressions of p21 and p27 were examined immunhistochemically in endoscopic biopsy specimens from 16 patients matching the diagnostic criteria of GWDET. Percentage of positive nuclear staining either weak or strong was noted. The association of immunoexpressions with age, gender, tumor localization, multifocality and accompanying chronic atrophic gastritis, neuroendocrine cell hyperplasia （NEH）, neuroendocrine dysplasia （NED）, intestinal metaplasia （IM）, Ki-67 proliferation index and clinical outcome were also evaluated.RESULTS： All cases expressed p27 with a mean expression score of 43.6%, while 31.3% of the cases showed any p21 expression, p21 and p27 immunoexpressions were significantly correlated with each other （P 〈 0.01）, and the p21-expressing group had higher p27 expression scores （68% vs 22%）. p21 and p27 expressions were lower in women, in non-atrophic mucosa and cases whose tumors were located somewhere other than fundus without submucosal extension. On contrary, p21 and p27 expressions were higher in males and the patients with submucosal extension and atrophic gastritis. Cases presenting lower p27 scores had solitary tumors showing neither NEH-NED nor IM. Despite, cases with lower p21 expression presented multifocal tumors accompanied by NEH-NED. However, no correlation of p21 and p27 expressions was found with age and Ki-67 expression.CONCLUSION： p27 is widely expressed in GWDETs, while p21 expression is sparse and observed in two thirds of the cases. Loss of p21 and p27 expressions may be correlated with different carcinoid tumor subtypes; however,more studies are needed to assess the role of these prospective markers in gastrointestinal endocrine tumors.
基金Peking University People’s Hospital Scientific Research Development Funds,No.RDY2017-28.
文摘BACKGROUND Hepatectomy is the first choice for treating neuroendocrine tumor liver metastases.However,most patients with neuroendocrine tumor liver metastases are not suitable for hepatectomy.Ablation combined with hepatectomy can be an alternative to liver resection.AIM To explore the clinical effect of microwave ablation combined with hepatectomy for the treatment of neuroendocrine tumor liver metastases.METHODS In this study,the data of patients who underwent microwave ablation combined with hepatectomy for the treatment of neuroendocrine tumor liver metastases from June 2015 to January 2018 were reviewed.Before the operation,the patients did not receive any treatment for liver neuroendocrine tumors.After a multidisciplinary expert group discussion,all patients were deemed unsuitable for liver resection.All patients were diagnosed with neuroendocrine tumors by pathology.The overall survival time and progression-free survival time were followed by telephone calls and outpatient visits after surgery.RESULTS Eleven patients with neuroendocrine tumor liver metastases were treated by microwave ablation combined with hepatectomy between June 2015 and January 2018.The median number of liver metastatic nodules was 4(range,2 to 43).The median number of lesions resected was 1(range,1 to 18),and the median number of lesions ablated was 3(range,1 to 38).The mean operation time was 405.6(±39.4)min.The median intraoperative blood loss was 600 mL(range,50 to 3000).Ten patients had a fever after surgery.The median duration of fever was 3 d(range,0 to 21).Elevated bilirubin levels occurred in all patients after surgery.The median bilirubin on the first day after surgery was 28.5(range,10.7 to 98.9)μmol/L.One patient developed respiratory failure,renal insufficiency,and pneumonia after the operation.No patient died postoperatively during hospitalization.The mean overall survival time after surgery was 34.1(±3.7)mo,and the median progression-free survival time was 8(range,2 to 51)mo.One year after surgery,ten patients survived and five patients survived without progression.Three year after surgery,eight patients survived and two patients survived without progression.CONCLUSION Microwave ablation combined with hepatectomy not only makes the patients obtain a survival rate similar to that of patients undergoing hepatectomy,but also has a low incidence of postoperative complications.
文摘We report 2 cases of pancreatic endocrine tumors that caused obstruction of the main pancreatic duct(MPD).A 49-year-old asymptomatic man was referred to our institution because dilation of the MPD was revealed by abdominal ultrasonography(US).No tumor was detected by endoscopic ultrasonography,computed tomography(CT),and magnetic resonance imaging(MRI).The diameter of the MPD was > 20 mm at the body,and no dilation was noted at the head.Although malignancy was not confirmed through cytology or imaging,pancreatic cancer was strongly suspected.Pancreaticoduo-denectomy was performed.Pathological and immunohistochemical examination revealed a 5 mm × 3 mm serotonin-positive endocrine tumor.Fibrosis was present around the MPD and seemed to cause stricture.A 32-year-old asymptomatic man had elevated serum amylase,and US demonstrated dilation of the MPD.No tumor was detected by CT and MRI.Pancreatic cancer was suspected due to stricture and dilation of the MPD.Pancreatectomy of middle part of pancreas was performed.Pathological and immunohistochemical examination revealed a serotonin-positive endocrine tumor sized 5 mm × 4 mm.We report 2 cases of serotonin-positive pancreatic endocrine tumors that caused stricture of the MPD in spite of the small size of the tumor.
文摘Objective： The aim of our study was to analysis the pictures of conventional magnetic resonance imaging （MR）, diffusion-weighted imaging （DWl） and dynamic enhanced magnetic resonance imaging （DEMRI） of pancreatic endocrine tumors （PETs）, and evaluate diagnostic value of MR, DWl and DEMRI for diagnosing PETs. Methods： DWl and DEMRI scanning toward 13 patients with PETs being confirmed by surgical pathology before surgery on the basis of conventional MR scanning were carried out, and MR findings was analyzed retrospectively. Results： Of 13 patients with PETs there was 11 cases with single lesion, 2 with multiple, and had 15 lesions altogether, of which there were 3 lesions in pancreatic head, 1 in its neck, 2 in its body, 4 in its body and tail, 5 in its tail. MR findings： （1） T1WI signal was low or slightly lower （9/15）, and equal ones （5/15）; （2） T2WI showed high or slightly higher signal （10/15）, and equal ones （5/15）; （3） T1WI with fat suppression： the signal was low （11/15）, mixed signal （2/15）, and equal ones （2/15）; （4） DWI： normal pancreatic tissue exhibited homogeneous intermediate signal, all 15 lesions were high or slightly higher signal, the measured ADC values of tissue of PETs was （1.124 ± 0.252） × 104 mm2/s, and the ADC value of normal pancreatic tissue （1.873 ± 0.157） × 10^3 mm2/s; （5） Enhanced （M3D/LAVA） scanning： among 13 patients with PETs there were 12 pancreatic lesions with significantly enhanced signals in the arterial phase in all 15, and significantly higher than normal pancreatic tissue, and two slight enhancement was slightly higher signal; and 1 no enhancement. Enhanced pattern： homogeneous enhancement were 6 lesions, and the heterogeneous 4, and the edge ring 5. Conclusion： MR and DWl combining with DEMRI help qualitative diagnosis of pancreatic endocrine tumors.
文摘Autoimmune pancreatitis(AIP) is a rare cause of recurrent acute pancreatitis or chronic pancreatitis in middleaged patients,and is characterised by a marked infiltration of lymphocytes and plasma cells in pancreatic tissue.Diagnosis of focal forms can be diff icult as AIP may mimic pancreatic adenocarcinoma.Pediatric cases of AIP are exceptional.We report the case of a 15-yearold girl who had a focal AIP and associated cholangitis,with a very unusual vascularized mass that mimicked a pancreatic endocrine tumor.The diagnosis was obtained by a pancreatic biopsy,thus avoiding surgical resection,and all the clinical,biological and radiological abnormalities resolved after steroid therapy with 6 mo of follow-up.
文摘To investigate clinicopathological features,diagnosis and treatment of malignant pancreatic tumors.Methods A retrospective clinical analysis was made in 27 cases of malignant pancreatic tumors admitted from 1989 to 2003.Results Tumors were surgically resected in 22 patients.Tumor infiltration into surrounding organs was found in 13 cases.Four patients had lymph nodes metastasis and 5 had liver metastasis.Two cases were found tumor embolus in vascular or lymph tube.Neural invasion was found in 3.Conclusion Malignant pancreatic tumors has good prognosis.Aggressive attempts of surgical management should be performed.6 refs,1 tab.
文摘This review delved into the intricate relationship between circadian clocks and physiological processes,emphasizing their critical role in maintaining homeo-stasis.Orchestrated by interlocked clock genes,the circadian timekeeping system regulates fundamental processes like the sleep-wake cycle,energy metabolism,immune function,and cell proliferation.The central oscillator in the hypothalamic suprachiasmatic nucleus synchronizes with light-dark cycles,while peripheral tissue clocks are influenced by cues such as feeding times.Circadian disruption,linked to modern lifestyle factors like night shift work,correlates with adverse health outcomes,including metabolic syndrome,cardiovascular diseases,infec-tions,and cancer.We explored the molecular mechanisms of circadian clock genes and their impact on metabolic disorders and cancer pathogenesis.Specific associ-ations between circadian disruption and endocrine tumors,spanning breast,ovarian,testicular,prostate,thyroid,pituitary,and adrenal gland cancers,are highlighted.Shift work is associated with increased breast cancer risk,with PER genes influencing tumor progression and drug resistance.CLOCK gene expression correlates with cisplatin resistance in ovarian cancer,while factors like aging and intermittent fasting affect prostate cancer.Our review underscored the intricate interplay between circadian rhythms and cancer,involving the regulation of the cell cycle,DNA repair,metabolism,immune function,and the tumor microenvir-onment.We advocated for integrating biological timing into clinical consider-ations for personalized healthcare,proposing that understanding these connec-tions could lead to novel therapeutic approaches.Evidence supports circadian rhythm-focused therapies,particularly chronotherapy,for treating endocrine tumors.Our review called for further research to uncover detailed connections between circadian clocks and cancer,providing essential insights for targeted treatments.We emphasized the importance of public health interventions to mitigate lifestyle-related circadian disruptions and underscored the critical role of circadian rhythms in disease mechanisms and therapeutic interventions.
文摘Background Pancreatic endocrine tumors （PETs） are rare and their surgical treatment is often debated. The purpose of this retrospective study was to analyze the diagnosis and surgical strategy of functioning and non-functioning PETs. Methods From May 1980 to March 2006, 36 patients with pancreatic endocrine tumors at the Second Affiliated Hospital of Zhejiang University were retrospectively studied. Results Among the 36 patients, 29 （81%） had functioning tumors, and 7 （19%） had nonfunctioning tumors. Ninety-two percent of insulinomas were benign, whereas 4 （57%） of nonfunctioning PETs were malignant. The size of functioning tumors was （2.3i-0.3） cm, that of nonfunctioning tumors was less than （5.1^-0.5） cm. The combination CT and transabdominal ultrasonography resulted in a diagnostic sensitivity of 84%. Thirty-three primary lesions were precisely located in 32 patients （89%）. Atypical tumor resection was performed for 73% of functioning tumors, while typical pancreatectomy was performed for 6 （85%） of nonfunctioning tumors. Moreover, 5 liver resections and 1 lymph node dissection were performed. During the follow-up, fifteen complications occurred in 12 （36%） patients after operation. The 5-year survival rate for patients with benign tumors was 92% compared to 50% for those with malignant tumors. Surgical cure was achieved in 95% of patients with benign insulinomas. Conclusions Surgical strategy for PETs depends on the size and location of the tumor and the risk of malignancy. The optimal surgical procedure is key to prevent postoperative complication. Radical resection including initial and metastatic lesion may benefit patients with maliqnant PETs.
文摘Pancreatic endocrine tumors are rare but are among the most common neuroendocrine neoplasms of the abdomen.At diagnosis many of them are already advanced and diff icult to treat.We report on an initially inoperable malignant pancreatic endocrine tumor in a 33-year-old woman,who received neoadjuvant peptide receptor radionuclide therapy(PRRT)as firstline treatment.This resulted in a signif icant downstaging of the tumor and allowed its subsequent complete surgical removal.Follow-up for eighteen months revealed a complete remission.This is the first report on neoadjuvant PRRT in a neuroendocrine neoplasm with subsequent successful complete resection.
文摘Objective: To study the express of some nonhormonal antigens in pancreatic endocrine tumors. Methods: The nonhormonal antigens including Alpha subunit of human chorionic gonadotropin (α HCG), progesterone receptors (PR), 7B2, HISL 19, in normal pancreatic islets and in 52 cases of pancreatic endocrine tumors (PET) were investigated by immunohistochemistry. Results: It was found that HCG can be detected in PET but not in normal islet cells. HCG immunoreactivity was expressed by 3 of 28 (10.7%) benign PET and by 14 of 24 (58.3%) malignant PET.PR was found by 20 of 28(71.4%) benign PET and by 7 of 24 (29%) malignant PET. 7B2 was detected by 23 of 28 (82.1%) benign PET and by 13 of 24 (54.2%) malignant PET. HISL 19 was appeared by 23 of 28 benign PET and by 11 of 24 (46%) malignant PET. Golgitype persisted in 87.5% malignant tumors. Conclusion: The assay of nonhormonal antigens may be well defined the clinico pathological characteristics of PET.
文摘The antigen Alpha subunit of human chorionic gonadotropin (α HCG) and a HCGmRNA in pancreatic endocrine tumors (PET) were investigated by immuno histochemistry and in situ hybridization. It was found that α HCG can be detected in PET buy not in normal islet cells. α HCG immunoreactivity was expressed by 5 of 28 (18%) in benign PET and 14 of 24 (58.3%) in malignant PET. Using in situ hybridization of α HCGmRNA, a strong signal in PET was obtained. The clinico pathological significance of α HCG in PET was discussed.
文摘Large cell neuroendocrine carcinoma (LCNEC) in the biliary system is a poorly differentiated, high-grade neuroendocrine tumor. These tumors exhibit aggressive behavior and an increased tendency for early nodal and distant metastases. Herein, we report an unusual case of a pure primary LCNEC of the common bile duct (CBD). A 75-year-old female presented with nausea and jaundice. The patient underwent a CBD excision with lymph node dissection. Upon histological and immunohistochemical examination, the tumor exhibited pure large cell-type neuroendocrine features. Metastases were noted in two of the eight lymph nodes. The patient was administered adjuvant chemotherapy. The patient’s cancer recurred 7 mo after surgery, and the patient died from liver failure 5 mo after recurrence. The prognosis of LCNEC of CBD remains poor despite curative resection and adjuvant chemotherapy. The role of additional therapies, such as multimodal treatment including radiation therapy, must be further studied to improve the prognoses of patients.
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.
文摘BACKGROUND:Carcinoid tumors of the extrahepatic biliary tree are extremely rare malignancies,accounting for 0.2%-2%of all gastrointestinal carcinoid tumors,while carcinoids of the cystic duct are an uncommon entity and an extremely unusual cause of bile duct obstruction.METHODS:After an extensive literature review,we retrospectively analysed 61 cases of carcinoid tumor of the biliary tree as well as one additional case of a 60-yearold female with symptoms and laboratory/imaging examination findings compatible with those of a malignant biliary tract obstruction.At laparotomy,resection of the gallbladder and common bile duct was performed.Histological study revealed a well-differentiated neuroendocrine carcinoma of the cystic duct.The patient remained disease-free at 16 months.RESULTS:Our presentation is the seventh case reported in the world literature.Compared to cholangiocarcinoma,analysis of the reviewed group indicates an increased incidence of extrahepatic carcinoid tumors in younger persons along with a slight female predominance.Statistically,the most common anatomic location is the common bile duct,followed by the perihilar region and the cystic duct.Jaundice is the most common finding.Curative surgery was realized in the majority of cases and longterm disease-free survival was achieved when surgery was curative.CONCLUSIONS:Carcinoid tumors obstructing the biliary tree are extremely difficult to diagnose preoperatively,and nearly impossible to differentiate from non-neuroendocrine tumors.As surgery offers the only potential cure for both biliary carcinoids and cholangiocarcinoma,we recommend aggressive surgical therapy as the treatment of choice in every case of potentially resectable biliary tumor.
文摘Gastric carcinoids(GCs),which originate from gastric enterochromaffin-like(ECL) mucosal cells and account for 2.4% of all carcinoids,are found increasingly in the course of upper gastrointestinal tract endoscopy.Current nosography includes those occurring in chronic conditions with hypergastrinemia,as the type 1 associated with chronic atrophic gastritis,and the type 2 associated with Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1,and type 3,which is unrelated to hypergastrinemia and is frequently malignant,with distant metastases.The optimal clinical approach to GCs remains to be elucidated,depending upon type,size and number of carcinoids.While there is agreement concerning the treatment of type 3 carcinoids,for types 1 and 2,current possibilities include simple surveillance,endoscopic polypectomy,surgical excision,associated or not with surgical antrectomy,or total gastrectomy.Moreover,the recent introduction of somatostatin analogues represents a therapeutic option of possibly outstanding relevance.
基金Supported by the Hensel Stiftung Kiel (F370011, MA and GK),the Swiss National Foundation (SNF 31-61884, AP and PK) andthe German Society of Pathology (MA). Tobias Henopp has afellowship sponsored by Ipsen GMBH, Ettlingen, Germany
文摘Gastrinomas are defined as gastrin secreting tumors that are associated with Zollinger-Ellison syndrome （ZES）. ZES is characterized by elevated fasting gastrin serum levels, positive secretin stimulation test and clinical symptoms such as recurrent peptic ulcer disease, gastroesophageal reflux disease and occasional diarrhea. Genetically, nonhereditary （sporadic） gastrinomas are distinguished from hereditary gastrinomas, which are associated with multiple endocrine neoplasia type 1 （MEN1） syndrome. In general, duodenal gastrinomas are small and solitary if they are sporadic and multiple as well as hereditary. The sporadic gastrinomas occur in the duodenum or in the pancreas while the hereditary gastrinomas almost all occur in the duodenum. Our series of 77 sporadic duodenal neuroendocrine tumors （NETs） includes 18 patients （23.4%） with gastrinomas and ZES. Of 535 sporadic NETs in the pancreas collected from the NET archives of the departments of pathology in Zürich, Switzerland, and Kiel, Germany, 24 patients （4.5%） suffered from sporadic pancreatic gastrinomas and ZES. These NETs have to be distinguished from tumors with immunohistochemical positivity for gastrin but without evidence of ZES. An additional 19 patients suffered from MEN1 and ZES. These patients showed exclusively duodenal gastrinomas, but not pancreatic gastrinomas. The prognosis of sporadic and MEN1- associated duodenal gastrinomas is better than that of pancreatic gastrinomas, since they progress slowly to liver metastasis. In summary, sporadic and MEN1- associated gastrinomas in the duodenum and pancreas show different clinico-pathological and genetic features. The incidence of sporadic duodenal gastrin-producing tumors is increasing, possibly due to optimized diagnostic procedures. In contrast, pancreatic MEN1- associated gastrinomas seem to be extremely rare. A considerable subset of tumors with immunohistochemical expression of gastrin but without evidence of ZES should be designated as functionally inactive NETs expressing gastrin, but not as gastrinomas.
文摘Objective To explore tissue expression of cyclin-dependent kinase inhibitor p27Kip1 andβ-catenin in multiple endocrine neoplasia type1（MEN1）-related parathyroid tumors（MHPT）.Methods Immunohistochemistry was performed to analyze the expression of p27Kip1 andβ-catenin in parathyroid glands from 31 subjects
文摘von Hippel-Lindau(VHL)disease is a rare,inherited neoplastic disease characterized by hemangioblastomas(HBL)of the central nervous system(CNS),retinal angiomas,renal cell carcinomas(RCC),pancreatic endocrine tumors(PETs),pheochromocytomas,paragangliomas,and visceral cysts.We encountered a large VHL family in northwest China and conducted a systematic screening of the family members based on their epidemiological and clinical characteristics.A self-designed questionnaire was used to collect the general sociodemographic and health information of the family members.For the preliminary family screening,physical examination and abdomen B ultrasonography were performed.The suspected patients were subjected to cranial computerized tomography and fundus examination.The clinical data of the patients with confirmed VHL disease were collected from hospital records.A total of 63 lineal descendants in six generations were observed in the family(generations O,A,B,C,D,E),including 9 dead suspected cases(6 males,3 females)and 10 living cases(2 males,8 females).Among the 10 living cases,4,2,1,3,4,8,and 2 manifested HBLs of the CNS,PETs,RCC,pancreatic cysts,renal cysts,pheochromocytomas(4 hemi and 4 bilateral),and paragangliomas,respectively.Data showed that the morbidity of VHL disease in generation C was lower than that in generation B,but the age of onset was younger.This study is the first to report VHL disease in northwest China and VHL-associated PET cases in Chinese.Therefore,follow-up checkups of the family should be focused on younger generations.Proper family screening protocols should be followed for the treatment of patients with VHL disease.
