BACKGROUND Pancreatic mixed neuroendocrine-non-neuroendocrine neoplasms(MiNENs)are rare malignancies affecting the pancreas.The World Health Organization defines MiNENs as neoplasms composed of morphologically recogni...BACKGROUND Pancreatic mixed neuroendocrine-non-neuroendocrine neoplasms(MiNENs)are rare malignancies affecting the pancreas.The World Health Organization defines MiNENs as neoplasms composed of morphologically recognizable neuroendocrine and non-neuroendocrine components,each constituting 30%or more of the tumor volume.Adenocarcinoma-neuroendocrine carcinoma is the most frequent MiNEN combination.A well-differentiated neuroendocrine tumor(NET)component is rarely reported in MiNENs.CASE SUMMARY Here we report a rare case with intermingled components of ductal adenocarcinoma and grade 1 well-differentiated NET in the pancreas.The two tumors show distinct histology and significant differentiation discrepancy(poorly differentiated high grade adenocarcinoma and well-differentiated low grade NET),and also present as metastases in separate lymph nodes.Next generation sequencing of the two components demonstrates KRAS and TP53 mutations in the ductal adenocarcinoma,but no genetic alterations in the NET,suggesting divergent origins for these two components.Although tumors like this meet the diagnostic criteria for MiNEN,clinicians often find the diagnosis and staging confusing and impractical for clinical management.CONCLUSION Mixed NET/non-NET tumors with distinct histology and molecular profiles might be better classified as collision tumors rather than MiNENs.展开更多
We describe a rare case of the transformation of a dysplastic nodule into well-differentiated hepato- cellular carcinoma (HCC) in a 56-year-old man with alcoholrelated liver cirrhosis. Ultrasound (US) disclosed a 10 m...We describe a rare case of the transformation of a dysplastic nodule into well-differentiated hepato- cellular carcinoma (HCC) in a 56-year-old man with alcoholrelated liver cirrhosis. Ultrasound (US) disclosed a 10 mm hypoechoic nodule and contrast enhanced US revealed a hypovascular nodule, both in segment seven. US-guided biopsy revealed a high-grade dysplastic nodule characterized by enhanced cellularity with a high N/C ratio, increased cytoplasmic eosinophilia, and slight cell atypia. One year later, the US pattern of the nodule changed from hypoechoic to hyperechoic without any change in size or hypovascularity. US-guided biopsy revealed well-differentiated HCC of the same features as shown in the first biopsy, but with additional pseudoglandular formation and moderate cell atypia. Moreover, immunohistochemical staining of cyclase- associated protein 2, a new molecular marker of well- differentiated HCC, turned positive. This is the first case of multistep hepatocarcinogenesis from a dysplastic nodule to well-differentiated HCC within one year in alcohol-related liver cirrhosis.展开更多
AIM:To assess the clinicopathological characteristics of duodenal well-differentiated endocrine tumors.METHODS:We examined clinicopathological characteristics in 11 consecutive patients with duodenal well-differentiat...AIM:To assess the clinicopathological characteristics of duodenal well-differentiated endocrine tumors.METHODS:We examined clinicopathological characteristics in 11 consecutive patients with duodenal well-differentiated endocrine tumors treated by endoscopic therapy or surgery in our hospital from 1992 through 2007.Patients with well-differentiated endocrine tu-mors of the papilla of Vater or with gastrinoma were excluded.RESULTS:Three patients received endoscopic treatment,and 8 underwent surgery.In patients who received endoscopic treatment,the tumor diameter was less than 1.0 cm,with no histopathological evidence of lymphovascular invasion or invasion of the muscularis.There were no complications such as late bleedingor perforation after treatment.Among 8 patients with tumors less than 1.0 cm in diameter,3 underwent partial resection,and 2 underwent radical surgery.Three patients had lymphovascular invasion,1 had invasion of the muscularis,and 1 had proximal lymph node metastasis.Among 3 patients with tumors 1.0 cm or more in diameter,1 underwent partial resection,and 2 under-went radical surgery.One patient had lymphovascular invasion,with no lymph node metastasis.After treatment,all patients are alive and have remained free of metastasis and recurrence.CONCLUSION:Duodenal well-differentiated endocrine tumors less than 1.0 cm in diameter have a risk of lym-phovascular invasion,invasion of the muscularis,and lymph node metastasis,irrespective of procedural prob-lems.展开更多
BACKGROUND Villous adenomas of the urinary tract are uncommon. They are morphologically similar to and difficult to differentiate from their counterpart in the colon. The histogenesis and malignant potential are uncer...BACKGROUND Villous adenomas of the urinary tract are uncommon. They are morphologically similar to and difficult to differentiate from their counterpart in the colon. The histogenesis and malignant potential are uncertain.CASE SUMMARY A 63-year-old woman was admitted to our hospital with a mass in the urethral orifice. Gross and microscopic pathological examination was suggestive of urethral villous adenoma with focal well-differentiated adenocarcinoma. The whole urethra and part of the bladder were excised. No further treatment was offered. Carcinoembryonic antigen, cytokeratin 7, cytokeratin 20, epithelial membrane antigen, and p53 protein were positive, and the ratio of Ki-67 was 60%. After follow-up at 11 mo, the patient was cured and had no recurrence.CONCLUSION Immunohistochemistry is important for differential diagnosis of villous adenoma of the urinary system. Complete surgical resection of the urinary tract is curative.展开更多
Background:The incidence of well-differentiated gastric neuroendocrine tumors(G-NET)is increasing annually,and while they have a good prognosis and low mortality rate,their high recurrence rate makes treatment options...Background:The incidence of well-differentiated gastric neuroendocrine tumors(G-NET)is increasing annually,and while they have a good prognosis and low mortality rate,their high recurrence rate makes treatment options controversial.This study aims to determine the relationship between individualized treatment plans and the recurrence of G-NET.Methods:We performed a multicenter,retrospective study of 94 patients with highly differentiated G-NET and treated at Peking Union Medical College Hospital,Yantai Yuhuangding Hospital,and Beijing Zhong-Neng-Jian Hospital from November 2015 to September 2023.Risk factors for recurrence of G-NETs were investigated using chi-squared test and multifactorial logistic regression analysis.Results:After a median follow-up of 49 months,the overall recurrence rate among the 94 G-NET patients was 14%(13/94).The recurrence rates of endoscopic mucosal resection(EMR),endoscopic submucosal dissection(ESD),somatostatin analog(SSA)therapy,and surgery were 43%(6/14),10%(5/49),5%(1/22),and 11%(1/9),respectively.Post-treatment recurrence rates were significantly different(P=0.014)among four treatments(EMR,ESD,SSA,and surgery),and further subgroup comparisons revealed lower recurrence rates in the ESD and SSA groups than in the EMR group.From the second month onward,SSA therapy considerably reduced the gastrin levels from 1081.0(571.5,2472.8)pg/mL to 461.5(255.3,795.0)pg/mL(Z=-3.521,P<0.001).Both chi-squared test and multifactorial logistic regression analysis suggested that among the clinicopathological parameters studied,only the pre-treatment gastrin level(P=0.018 and 0.005)and the type of treatment(P=0.014 and 0.017)were significantly associated with G-NET recurrence.Conclusions:Individualized treatment strategies may reduce the risk of relapse after G-NET treatment.Long-term SSA therapy may be a secure and efficacious treatment option for type 1 G-NET with more than six lesions,and it substantially decreases the incidence of post-treatment recurrence.展开更多
BACKGROUND Numerous studies have indicated that the temozolomide and capecitabine regimen(TEMCAP)exhibits a certain level of efficacy in treating advanced,welldifferentiated gastroenteropancreatic neuroendocrine tumor...BACKGROUND Numerous studies have indicated that the temozolomide and capecitabine regimen(TEMCAP)exhibits a certain level of efficacy in treating advanced,welldifferentiated gastroenteropancreatic neuroendocrine tumors(GEP-NET).However,published data from Peru are limited.We hypothesize that this regimen could be a viable therapeutic option for advanced GEP-NET in the Peruvian population.AIM To evaluate overall survival(OS)in patients diagnosed with advanced GEP-NET treated with TEMCAP at the Instituto Nacional de Enfermedades Neoplásicas(INEN)in Lima-Perú.METHODS A retrospective review was conducted to identify patients with GEP-NEN treated with the TEMCAP regimen between 2011 and 2021 at the INEN.A total of thirtyeight patients were included in the final analysis:Thirty-five received TEMCAP as a first-line treatment,and three as a second-line treatment.The primary objective was to evaluate OS.The efficacy and safety of TEMCAP were assessed until the occurrence of unacceptable toxicity or disease progression.Survival outcomes were estimated using the Kaplan-Meier method.RESULTS The median age of the patients was 52 years(range 24-77 years),and 53.3%were female.The most common symptoms at diagnosis were abdominal pain in 31 patients(81.6%).Primary tumors included 12 in the rectum(31.6%),11 in the pancreas(28.9%),3 in the ileum(7.9%),2 in the mesentery(5.3%),2 in the small intestine(5.3%),1 in the appendix(2.6%),1 in the stomach(2.6%)and 6 cases of liver metastasis of unknown primary(15.8%).Five were neuroendocrine tumors(NET)G1(13.2%),33 were NET G2(86.8%),five had Ki67<3%(13.2%),and 33 had Ki67 between 3%and 20%(86.8%).TEMCAP was administered to 35(92.1%)patients as first-line treatment.OS at 12,36,and 60 months was estimated in 80%,66%,and 42%,respectively,with a median OS of 49 months.CONCLUSION TEMCAP therapy is a viable first-line option regarding efficacy and tolerability in areas where standard therapy is inaccessible.展开更多
AIM:To elucidate the variety of ways early-stage hepatocellular carcinoma(HCC)can appear on magnetic resonance(MR)imaging by analyzing T1-weighted,T2-weighted,and gadolinium-enhanced dynamic studies.METHODS:Seventy-th...AIM:To elucidate the variety of ways early-stage hepatocellular carcinoma(HCC)can appear on magnetic resonance(MR)imaging by analyzing T1-weighted,T2-weighted,and gadolinium-enhanced dynamic studies.METHODS:Seventy-three patients with well-differentiated HCC(wHCC)or dysplastic nodules were retrospectively identified from medical records,and new histological sections were prepared and reviewed.The tumor nodules were categorized into three groups:dysplastic nodule(DN),wHCC compatible with Edmondson-Steiner grade I HCC(w1-HCC),and wHCC compatible with Edmondson-Steiner gradeⅡHCC(w2-HCC).The signal intensity on pre-contrast MR imaging and the enhancing pattern for each tumor were recorded and compared between the three tumor groups.RESULTS:Among the 73 patients,14 were diagnosed as having DN,40 were diagnosed as having w1-HCC,and 19 were diagnosed as having w2-HCC.Hyperintensity measurements on T2-weighted axial images(T2WI)were statistically significant between DNs and wHCC(P=0.006)and between DN and w1-HCC(P=0.02).The other imaging features revealed no significant differences between DN and wHCC or between DN and w1-HCC.Hyperintensity on both T1W out-phase imaging(P=0.007)and arterial enhancement on dynamic study(P=0.005)showed statistically significant differences between w1-HCC and w2-HCC.The other imaging features revealed no significant differences between w1-HCC and w2-HCC.CONCLUSION:In the follow-up for a cirrhotic nodule,increased signal intensity on T2WI may be a sign of malignant transformation.Furthermore,a noted loss of hyperintensity on T1WI and the detection of arterial enhancement might indicate further progression of the histological grade.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
Liposarcoma is the most common soft tissue sarcoma and accounts for 15%-20% of all mesenchymal malignancies. The tumor occurs most frequently in the limbs, retroperitoneum and rarely has a visceral location. We report...Liposarcoma is the most common soft tissue sarcoma and accounts for 15%-20% of all mesenchymal malignancies. The tumor occurs most frequently in the limbs, retroperitoneum and rarely has a visceral location. We report a case of a gastric liposarcoma in a male patient. A 68 years old male patient was admitted to hospital for abdominal discomfort and fullness lasting for a month. He reported rare episodes of vomiting. The CT examination revealed a large epigastric mass （8 cm × 4 cm） involving the lesser curvature of the stomach, in contact with the pancreas and gallbladder. Fatty areas within the mass were evident. A total gastrectomy together with cholecystectomy was performed. The histopathological diagnosis was a well differentiated liposarcoma. The patient did not undergo any adjuvant treatment, he is under close follow up and two years later he is disease free. We report this case due to the rarity of this tumor in the stomach （nine cases reported in the literature）.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
BACKGROUND Well-differentiated liposarcoma is the second most common pathologic type of retroperitoneal sarcoma.It is characterized by a huge mass,but multiple organ invasions are common.Surgery is the only treatment ...BACKGROUND Well-differentiated liposarcoma is the second most common pathologic type of retroperitoneal sarcoma.It is characterized by a huge mass,but multiple organ invasions are common.Surgery is the only treatment option for potential cure.Hyper-accuracy three-dimensional(3D)reconstruction is widely used in robotic partly nephrectomy owing to its ability to visualize overlapping anatomy.CASE SUMMARY A 54-year-old man was admitted for progressive abdominal distension over the preceding 2 mo.Computed tomography revealed a 32 cm×21 cm×12 cm lipomatous mass.Hyper-accuracy 3D reconstruction was performed because of the complex relationship between the mass and nearby tissue.The patient underwent surgical resection,and the tumor did not recur for over 16 mo.CONCLUSION Hyper-accuracy 3D reconstruction is useful for operative planning owing to its intuitiveness and precise determination of anatomical structures in both tumors and nearby tissues.展开更多
BACKGROUND Retroperitoneal lipomas are extremely rare tumors and tend to be large in size(>10 cm)when diagnosed,causing various clinical manifestations.Preoperative diagnosis of retroperitoneal lipomas is difficult...BACKGROUND Retroperitoneal lipomas are extremely rare tumors and tend to be large in size(>10 cm)when diagnosed,causing various clinical manifestations.Preoperative diagnosis of retroperitoneal lipomas is difficult.There is a lack of relevant information about the management and prognosis of these benign tumors due to limited reports.CASE SUMMARY A 53-year-old woman who complained about progressive abdominal distention and aggravating satiety was referred to the gynecological outpatient department of Peking Union Medical College Hospital.Computerized tomography(CT)revealed an immense mass with fat density,measuring 28.6 cm×16.6 cm in size.Adjacent organs,including the intestinal tract and uterus,were squeezed to the right side of the abdomen.An exploratory laparotomy was performed with suspicion of liposarcoma.Intraoperatively,a giant yellowish lobulated mass was found occupying the retroperitoneum and it was removed by tumor debulking.Postoperative histopathological results confirmed the diagnosis of retroperitoneal lipoma.CONCLUSION Retroperitoneal lipoma is a very rare condition and is difficult to differentiate from well-differentiated liposarcoma.Radiographic investigations,especially CT and magnetic resonance imaging,are important for preoperative diagnosis.Surgical resection is the fundamental treatment,which is difficult due to its size and relation to neighboring structures.展开更多
Liposarcomas (LS) are the second most common type of soft tissue malignancies in adults;they arise from mesenchymal cells and account for 1% of all adult cancers and 15% of all pediatric cancers. The site of origin ca...Liposarcomas (LS) are the second most common type of soft tissue malignancies in adults;they arise from mesenchymal cells and account for 1% of all adult cancers and 15% of all pediatric cancers. The site of origin can be from anywhere there is fat in the human body. LS are classified based on the primary site of origin, and mediastinal LS are extremely rare. When mediastinal neoplasms are stratified based on histology, they represent less than 1% of all </span><span style="font-family:Verdana;">mediastinal tumors. To date, less than 150 cases have been reported in the</span><span style="font-family:Verdana;"> English literature. This article aims to present an unusual case of an extremely rare malignancy and perform a systematic review of the latest literature. In </span><span style="font-family:Verdana;">this report, our group is documenting the presentation, management, and</span><span style="font-family:Verdana;"> outcome of a 65-year-old male patient with a massive anterior mediastinal primary LS. The tumor was displacing the mediastinum into the right chest, occupying most of the left chest, and pushing the diaphragm into the abdomen. Mediastinal liposarcomas are extremely rare malignancies and can prove to be challenging to diagnose and treat. Aggressive surgical treatment with R0 resection is the gold standard, however, tumor biology in many cases is associated with variable growth rates and encroachment of adjacent vital structures and blood vessels. When complex anatomical structures preclude an R0 resection, there is a high incidence of local recurrence. In cases where there is a high risk of recurrence, radiotherapy is indicated and chemotherapy has a more limited role.展开更多
The incidence of nodal metastasis is quite common in well-differentiated thyroid cancer.However,its clinical significance is generally quite minimal.The adverse pathological features need to be recognized.The debate c...The incidence of nodal metastasis is quite common in well-differentiated thyroid cancer.However,its clinical significance is generally quite minimal.The adverse pathological features need to be recognized.The debate continues over prophylactic central compartment dissection.However,it needs to be re-evaluated in terms of complications of elective procedure.The extent of lateral neck dissection is standardized from level II through level V.Recurrent nodal disease is more likely to be persistent nodal disease.Appropriate preoperative imaging is very crucial.Surgery for recurrent disease needs to be considered based on nodal prognostic factors and location of the disease.The approach of using active surveillance and continuous monitoring is reasonable,especially for recurrence below 1 cm.展开更多
文摘BACKGROUND Pancreatic mixed neuroendocrine-non-neuroendocrine neoplasms(MiNENs)are rare malignancies affecting the pancreas.The World Health Organization defines MiNENs as neoplasms composed of morphologically recognizable neuroendocrine and non-neuroendocrine components,each constituting 30%or more of the tumor volume.Adenocarcinoma-neuroendocrine carcinoma is the most frequent MiNEN combination.A well-differentiated neuroendocrine tumor(NET)component is rarely reported in MiNENs.CASE SUMMARY Here we report a rare case with intermingled components of ductal adenocarcinoma and grade 1 well-differentiated NET in the pancreas.The two tumors show distinct histology and significant differentiation discrepancy(poorly differentiated high grade adenocarcinoma and well-differentiated low grade NET),and also present as metastases in separate lymph nodes.Next generation sequencing of the two components demonstrates KRAS and TP53 mutations in the ductal adenocarcinoma,but no genetic alterations in the NET,suggesting divergent origins for these two components.Although tumors like this meet the diagnostic criteria for MiNEN,clinicians often find the diagnosis and staging confusing and impractical for clinical management.CONCLUSION Mixed NET/non-NET tumors with distinct histology and molecular profiles might be better classified as collision tumors rather than MiNENs.
文摘We describe a rare case of the transformation of a dysplastic nodule into well-differentiated hepato- cellular carcinoma (HCC) in a 56-year-old man with alcoholrelated liver cirrhosis. Ultrasound (US) disclosed a 10 mm hypoechoic nodule and contrast enhanced US revealed a hypovascular nodule, both in segment seven. US-guided biopsy revealed a high-grade dysplastic nodule characterized by enhanced cellularity with a high N/C ratio, increased cytoplasmic eosinophilia, and slight cell atypia. One year later, the US pattern of the nodule changed from hypoechoic to hyperechoic without any change in size or hypovascularity. US-guided biopsy revealed well-differentiated HCC of the same features as shown in the first biopsy, but with additional pseudoglandular formation and moderate cell atypia. Moreover, immunohistochemical staining of cyclase- associated protein 2, a new molecular marker of well- differentiated HCC, turned positive. This is the first case of multistep hepatocarcinogenesis from a dysplastic nodule to well-differentiated HCC within one year in alcohol-related liver cirrhosis.
文摘AIM:To assess the clinicopathological characteristics of duodenal well-differentiated endocrine tumors.METHODS:We examined clinicopathological characteristics in 11 consecutive patients with duodenal well-differentiated endocrine tumors treated by endoscopic therapy or surgery in our hospital from 1992 through 2007.Patients with well-differentiated endocrine tu-mors of the papilla of Vater or with gastrinoma were excluded.RESULTS:Three patients received endoscopic treatment,and 8 underwent surgery.In patients who received endoscopic treatment,the tumor diameter was less than 1.0 cm,with no histopathological evidence of lymphovascular invasion or invasion of the muscularis.There were no complications such as late bleedingor perforation after treatment.Among 8 patients with tumors less than 1.0 cm in diameter,3 underwent partial resection,and 2 underwent radical surgery.Three patients had lymphovascular invasion,1 had invasion of the muscularis,and 1 had proximal lymph node metastasis.Among 3 patients with tumors 1.0 cm or more in diameter,1 underwent partial resection,and 2 under-went radical surgery.One patient had lymphovascular invasion,with no lymph node metastasis.After treatment,all patients are alive and have remained free of metastasis and recurrence.CONCLUSION:Duodenal well-differentiated endocrine tumors less than 1.0 cm in diameter have a risk of lym-phovascular invasion,invasion of the muscularis,and lymph node metastasis,irrespective of procedural prob-lems.
文摘BACKGROUND Villous adenomas of the urinary tract are uncommon. They are morphologically similar to and difficult to differentiate from their counterpart in the colon. The histogenesis and malignant potential are uncertain.CASE SUMMARY A 63-year-old woman was admitted to our hospital with a mass in the urethral orifice. Gross and microscopic pathological examination was suggestive of urethral villous adenoma with focal well-differentiated adenocarcinoma. The whole urethra and part of the bladder were excised. No further treatment was offered. Carcinoembryonic antigen, cytokeratin 7, cytokeratin 20, epithelial membrane antigen, and p53 protein were positive, and the ratio of Ki-67 was 60%. After follow-up at 11 mo, the patient was cured and had no recurrence.CONCLUSION Immunohistochemistry is important for differential diagnosis of villous adenoma of the urinary system. Complete surgical resection of the urinary tract is curative.
基金supported by grants from National High-Level Hospital Clinical Research Funding(No.2022-PUMCH-C-055)National High-Level Hospital Clinical Research Funding(No.2022-PUMCH-D-002).
文摘Background:The incidence of well-differentiated gastric neuroendocrine tumors(G-NET)is increasing annually,and while they have a good prognosis and low mortality rate,their high recurrence rate makes treatment options controversial.This study aims to determine the relationship between individualized treatment plans and the recurrence of G-NET.Methods:We performed a multicenter,retrospective study of 94 patients with highly differentiated G-NET and treated at Peking Union Medical College Hospital,Yantai Yuhuangding Hospital,and Beijing Zhong-Neng-Jian Hospital from November 2015 to September 2023.Risk factors for recurrence of G-NETs were investigated using chi-squared test and multifactorial logistic regression analysis.Results:After a median follow-up of 49 months,the overall recurrence rate among the 94 G-NET patients was 14%(13/94).The recurrence rates of endoscopic mucosal resection(EMR),endoscopic submucosal dissection(ESD),somatostatin analog(SSA)therapy,and surgery were 43%(6/14),10%(5/49),5%(1/22),and 11%(1/9),respectively.Post-treatment recurrence rates were significantly different(P=0.014)among four treatments(EMR,ESD,SSA,and surgery),and further subgroup comparisons revealed lower recurrence rates in the ESD and SSA groups than in the EMR group.From the second month onward,SSA therapy considerably reduced the gastrin levels from 1081.0(571.5,2472.8)pg/mL to 461.5(255.3,795.0)pg/mL(Z=-3.521,P<0.001).Both chi-squared test and multifactorial logistic regression analysis suggested that among the clinicopathological parameters studied,only the pre-treatment gastrin level(P=0.018 and 0.005)and the type of treatment(P=0.014 and 0.017)were significantly associated with G-NET recurrence.Conclusions:Individualized treatment strategies may reduce the risk of relapse after G-NET treatment.Long-term SSA therapy may be a secure and efficacious treatment option for type 1 G-NET with more than six lesions,and it substantially decreases the incidence of post-treatment recurrence.
文摘BACKGROUND Numerous studies have indicated that the temozolomide and capecitabine regimen(TEMCAP)exhibits a certain level of efficacy in treating advanced,welldifferentiated gastroenteropancreatic neuroendocrine tumors(GEP-NET).However,published data from Peru are limited.We hypothesize that this regimen could be a viable therapeutic option for advanced GEP-NET in the Peruvian population.AIM To evaluate overall survival(OS)in patients diagnosed with advanced GEP-NET treated with TEMCAP at the Instituto Nacional de Enfermedades Neoplásicas(INEN)in Lima-Perú.METHODS A retrospective review was conducted to identify patients with GEP-NEN treated with the TEMCAP regimen between 2011 and 2021 at the INEN.A total of thirtyeight patients were included in the final analysis:Thirty-five received TEMCAP as a first-line treatment,and three as a second-line treatment.The primary objective was to evaluate OS.The efficacy and safety of TEMCAP were assessed until the occurrence of unacceptable toxicity or disease progression.Survival outcomes were estimated using the Kaplan-Meier method.RESULTS The median age of the patients was 52 years(range 24-77 years),and 53.3%were female.The most common symptoms at diagnosis were abdominal pain in 31 patients(81.6%).Primary tumors included 12 in the rectum(31.6%),11 in the pancreas(28.9%),3 in the ileum(7.9%),2 in the mesentery(5.3%),2 in the small intestine(5.3%),1 in the appendix(2.6%),1 in the stomach(2.6%)and 6 cases of liver metastasis of unknown primary(15.8%).Five were neuroendocrine tumors(NET)G1(13.2%),33 were NET G2(86.8%),five had Ki67<3%(13.2%),and 33 had Ki67 between 3%and 20%(86.8%).TEMCAP was administered to 35(92.1%)patients as first-line treatment.OS at 12,36,and 60 months was estimated in 80%,66%,and 42%,respectively,with a median OS of 49 months.CONCLUSION TEMCAP therapy is a viable first-line option regarding efficacy and tolerability in areas where standard therapy is inaccessible.
文摘AIM:To elucidate the variety of ways early-stage hepatocellular carcinoma(HCC)can appear on magnetic resonance(MR)imaging by analyzing T1-weighted,T2-weighted,and gadolinium-enhanced dynamic studies.METHODS:Seventy-three patients with well-differentiated HCC(wHCC)or dysplastic nodules were retrospectively identified from medical records,and new histological sections were prepared and reviewed.The tumor nodules were categorized into three groups:dysplastic nodule(DN),wHCC compatible with Edmondson-Steiner grade I HCC(w1-HCC),and wHCC compatible with Edmondson-Steiner gradeⅡHCC(w2-HCC).The signal intensity on pre-contrast MR imaging and the enhancing pattern for each tumor were recorded and compared between the three tumor groups.RESULTS:Among the 73 patients,14 were diagnosed as having DN,40 were diagnosed as having w1-HCC,and 19 were diagnosed as having w2-HCC.Hyperintensity measurements on T2-weighted axial images(T2WI)were statistically significant between DNs and wHCC(P=0.006)and between DN and w1-HCC(P=0.02).The other imaging features revealed no significant differences between DN and wHCC or between DN and w1-HCC.Hyperintensity on both T1W out-phase imaging(P=0.007)and arterial enhancement on dynamic study(P=0.005)showed statistically significant differences between w1-HCC and w2-HCC.The other imaging features revealed no significant differences between w1-HCC and w2-HCC.CONCLUSION:In the follow-up for a cirrhotic nodule,increased signal intensity on T2WI may be a sign of malignant transformation.Furthermore,a noted loss of hyperintensity on T1WI and the detection of arterial enhancement might indicate further progression of the histological grade.
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.
文摘Liposarcoma is the most common soft tissue sarcoma and accounts for 15%-20% of all mesenchymal malignancies. The tumor occurs most frequently in the limbs, retroperitoneum and rarely has a visceral location. We report a case of a gastric liposarcoma in a male patient. A 68 years old male patient was admitted to hospital for abdominal discomfort and fullness lasting for a month. He reported rare episodes of vomiting. The CT examination revealed a large epigastric mass （8 cm × 4 cm） involving the lesser curvature of the stomach, in contact with the pancreas and gallbladder. Fatty areas within the mass were evident. A total gastrectomy together with cholecystectomy was performed. The histopathological diagnosis was a well differentiated liposarcoma. The patient did not undergo any adjuvant treatment, he is under close follow up and two years later he is disease free. We report this case due to the rarity of this tumor in the stomach （nine cases reported in the literature）.
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.
基金the National Natural Science Foundation of China,No.81272833.
文摘BACKGROUND Well-differentiated liposarcoma is the second most common pathologic type of retroperitoneal sarcoma.It is characterized by a huge mass,but multiple organ invasions are common.Surgery is the only treatment option for potential cure.Hyper-accuracy three-dimensional(3D)reconstruction is widely used in robotic partly nephrectomy owing to its ability to visualize overlapping anatomy.CASE SUMMARY A 54-year-old man was admitted for progressive abdominal distension over the preceding 2 mo.Computed tomography revealed a 32 cm×21 cm×12 cm lipomatous mass.Hyper-accuracy 3D reconstruction was performed because of the complex relationship between the mass and nearby tissue.The patient underwent surgical resection,and the tumor did not recur for over 16 mo.CONCLUSION Hyper-accuracy 3D reconstruction is useful for operative planning owing to its intuitiveness and precise determination of anatomical structures in both tumors and nearby tissues.
基金Supported by the National Key Research and Development Program,No.2018YFC1002105.
文摘BACKGROUND Retroperitoneal lipomas are extremely rare tumors and tend to be large in size(>10 cm)when diagnosed,causing various clinical manifestations.Preoperative diagnosis of retroperitoneal lipomas is difficult.There is a lack of relevant information about the management and prognosis of these benign tumors due to limited reports.CASE SUMMARY A 53-year-old woman who complained about progressive abdominal distention and aggravating satiety was referred to the gynecological outpatient department of Peking Union Medical College Hospital.Computerized tomography(CT)revealed an immense mass with fat density,measuring 28.6 cm×16.6 cm in size.Adjacent organs,including the intestinal tract and uterus,were squeezed to the right side of the abdomen.An exploratory laparotomy was performed with suspicion of liposarcoma.Intraoperatively,a giant yellowish lobulated mass was found occupying the retroperitoneum and it was removed by tumor debulking.Postoperative histopathological results confirmed the diagnosis of retroperitoneal lipoma.CONCLUSION Retroperitoneal lipoma is a very rare condition and is difficult to differentiate from well-differentiated liposarcoma.Radiographic investigations,especially CT and magnetic resonance imaging,are important for preoperative diagnosis.Surgical resection is the fundamental treatment,which is difficult due to its size and relation to neighboring structures.
文摘Liposarcomas (LS) are the second most common type of soft tissue malignancies in adults;they arise from mesenchymal cells and account for 1% of all adult cancers and 15% of all pediatric cancers. The site of origin can be from anywhere there is fat in the human body. LS are classified based on the primary site of origin, and mediastinal LS are extremely rare. When mediastinal neoplasms are stratified based on histology, they represent less than 1% of all </span><span style="font-family:Verdana;">mediastinal tumors. To date, less than 150 cases have been reported in the</span><span style="font-family:Verdana;"> English literature. This article aims to present an unusual case of an extremely rare malignancy and perform a systematic review of the latest literature. In </span><span style="font-family:Verdana;">this report, our group is documenting the presentation, management, and</span><span style="font-family:Verdana;"> outcome of a 65-year-old male patient with a massive anterior mediastinal primary LS. The tumor was displacing the mediastinum into the right chest, occupying most of the left chest, and pushing the diaphragm into the abdomen. Mediastinal liposarcomas are extremely rare malignancies and can prove to be challenging to diagnose and treat. Aggressive surgical treatment with R0 resection is the gold standard, however, tumor biology in many cases is associated with variable growth rates and encroachment of adjacent vital structures and blood vessels. When complex anatomical structures preclude an R0 resection, there is a high incidence of local recurrence. In cases where there is a high risk of recurrence, radiotherapy is indicated and chemotherapy has a more limited role.
文摘The incidence of nodal metastasis is quite common in well-differentiated thyroid cancer.However,its clinical significance is generally quite minimal.The adverse pathological features need to be recognized.The debate continues over prophylactic central compartment dissection.However,it needs to be re-evaluated in terms of complications of elective procedure.The extent of lateral neck dissection is standardized from level II through level V.Recurrent nodal disease is more likely to be persistent nodal disease.Appropriate preoperative imaging is very crucial.Surgery for recurrent disease needs to be considered based on nodal prognostic factors and location of the disease.The approach of using active surveillance and continuous monitoring is reasonable,especially for recurrence below 1 cm.
