Gastric submucosal tumors(SMTs) are a rather frequent finding,occurring in about 0.36%of routine upper GIendoscopies.Endoscopic ultrasonography(EUS) has emerged as a reliable investigative procedure for evaluation of ...Gastric submucosal tumors(SMTs) are a rather frequent finding,occurring in about 0.36%of routine upper GIendoscopies.Endoscopic ultrasonography(EUS) has emerged as a reliable investigative procedure for evaluation of these lesions.Diagnostic EUS has the ability to differentiate intramural tumors from extraluminal compressions and can also show the layer of origin of gastric SMTs.Tumors can be further characterized by their layer of origin,echo pattern and margin.EUS-risk criteria of their malignant potential are presented,although the emergence of EUS-FNA has opened new indications for transmural tissue diagnosis and expanded the possibilities of EUS in SMTs of the stomach.Tissue diagnosis should address whether the SMT is a Gastrointestinal stromal tumour(GIST) or another tumor type and evaluate the malignant potential of a given GIST.However,there seems to be a lack of data on the optimal strategy in SMTs suspected to be GISTs with a negative EUS-FNA tissue diagnosis.The current management strategies,as well as open questions regarding their treatment are also presented.展开更多
AIM:To evaluate the effects of preoperative immunonutrition and other nutrition models on the cellular immunity parameters of patients with gastrointestinal tumors before surgical intervention.In addition,effects on p...AIM:To evaluate the effects of preoperative immunonutrition and other nutrition models on the cellular immunity parameters of patients with gastrointestinal tumors before surgical intervention.In addition,effects on postoperative complications were examined. METHODS:Patients with gastrointestinal tumors were randomized into 3 groups.The immunonutrition group received a combination of arginine,fatty acids and nucleotides.The second and third group received normal nutrition and standard enteral nutrition,respectively.Nutrition protocols were administered for 7 d prior to the operation.Nutritional parameters,in particular prealbumin levels and lymphocyte subpopulations(CD4+,CD8+,CD16+/56+,and CD69 cells)were evaluated before and after the nutrition protocols.Groups were compared in terms of postoperative complications and duration of hospital stay. RESULTS:Of the 42 patients who completed thestudy,16 received immunonutrition,13 received normal nutrition and 13 received standard enteral nutrition. prealbumin values were low in every group,but this parameter was improved after the nutritional protocol only in the immunonutrition group(13.64±8.83 vs 15.98±8.66,P=0.037).Groups were similar in terms of CD4+,CD16+/56,and CD69+prior to the nutritional protocol;whereas CD8+was higher in the standard nutrition group compared to the immunonutrition group.After nutritional protocols,none of the groups had an increase in their lymphocyte subpopulations.Also,groups did not differ in terms of postoperative complications and postoperative durations of hospital stay. CONCLUSION:Preoperative immunonutrition provided a significant increase in prealbumin levels,while it did not significantly alter T lymphocyte subpopulation counts,the rate of postoperative complications and the duration of hospital stay.展开更多
Solid pseudopapillary neoplasms are rare.This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas,including the epidemiology,cytology,molecular pathology,differential...Solid pseudopapillary neoplasms are rare.This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas,including the epidemiology,cytology,molecular pathology,differential diagnosis,treatment,and prognosis.Solid pseudopapillary neoplasms are low-grade malignant tumours of the pancreas characterized by poorly cohesive epithelial cells with solid and pseudopapillary patterns.Solid pseudopapillary neoplasms occur predominantly in young women.Although solid pseudopapillary neoplasms can occur throughout the pancreas,they arise slightly more frequently in the tail of the pancreas.The aetiology is unknown.Extremely rare cases have been reported in the setting of familial adenomatous polyposis.There are no symptoms unique to solid pseudopapillary neoplasms,however,the most common symptom is abdominal pain or discomfort.The features of solid pseudopapillary neoplasms on computed tomography imaging are indicative of the pathologic changes within the tumour.Typically,well-demarcated masses with variably solid and cystic appearances.Microscopically,these tumours are composed of epithelial cells forming solid and pseudopapillary structures,frequently undergoing haemorrhagic cystic degeneration.Typically,these tumours express nuclear and/or cytoplasmicβ-catenin.Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1,the gene encodingβ-catenin.The overall prognosis is excellent,and most patients are cured by complete surgical resection.展开更多
Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas,which may pose diagnostic difficulties.Because of its rarity,little is known...Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas,which may pose diagnostic difficulties.Because of its rarity,little is known about its clinical and pathologic features.This article reviews the clinical and pathologic features of pancreatoblastoma in adults including differential diagnosis,treatment,and follow-up.Although pancreatoblastoma commonly occurs in childhood,there have now been more than 70 adult pancreatoblastomas described in the literature.There is a slight male predominance.There are no symptoms unique to pancreatoblastomas and adult patients are frequently symptomatic.The most common presenting symptom is abdominal pain.Grossly,the tumours are often large and well-circumscribed.Microscopically,pancreatoblastomas are composed of neoplastic cells with predominantly acinar differentiation and characteristic squamoid nests.These tumours are positive for trypsin,chymotrypsin,lipase,and BCL10.Loss of heterozygosity on chromosome 11p is the most common molecular alteration in pancreatoblastomas.Adult pancreatoblastomas are aggressive tumours with frequent local invasion,recurrence,and distant metastasis.Treatment consists of surgical resection.Chemotherapy and radiotherapy may have a role in the treatment of recurrent,residual,unresectable,and metastatic disease.It is important to distinguish pancreatoblastomas from morphological mimics such as acinar cell carcinomas,solid pseudopapillary neoplasms,and pancreatic neuroendocrine neoplasms.展开更多
Bone is one of the preferred sites for the metastasis of malignant tumours,such as breast cancer,lung cancer and malignant melanoma.Tumour cells colonizing bone have the capacity to induce the expression of receptor a...Bone is one of the preferred sites for the metastasis of malignant tumours,such as breast cancer,lung cancer and malignant melanoma.Tumour cells colonizing bone have the capacity to induce the expression of receptor activator of nuclear factor-κB ligand(RANKL),which promotes osteoclast differentiation and activation.Tumour-induced osteoclastic bone resorption leads to a vicious cycle between tumours and bone cells that fuels osteolytic tumour growth,causing bone pain and hypercalcaemia.Furthermore,RANKL contributes to bone metastasis by acting as a chemoattractant to bone for tumour cells that express its receptor,RANK.Thus inhibition of the RANKL–RANK pathway is a promising treatment for bone metastasis,and a human monoclonal anti-RANKL antibody,denosumab,has been used in the clinic.However,orally available drugs targeting RANKL must be developed to increase the therapeutic benefits to patients.Here we report the efficacy of the small-molecule RANKL inhibitor AS2676293 in treating bone metastasis using mouse models.Oral administration of AS2676293 markedly inhibited bone metastasis of human breast cancer cells MDA-MB-231-5a-D-Luc2 as well as tumour-induced osteolysis.AS2676293 suppressed RANKLmediated tumour migration in the transwell assay and inhibited bone metastasis of the murine cell line B16F10,which is known not to trigger osteoclast activation.Based on the results from this study,RANKL inhibition with a small-molecule compound constitutes a promising therapeutic strategy for treating bone metastasis by inhibiting both osteoclastic bone resorption and tumour migration to bone.展开更多
Aim: To reveal the exonic and 3’UTR sequences of KRAS, TP53, APC, BRAF, PIK3CA genes in sporadic colorectal tumors and to investigate the clinical relevance of 3’UTR variations in miRNA profiles. Methods: In the stu...Aim: To reveal the exonic and 3’UTR sequences of KRAS, TP53, APC, BRAF, PIK3CA genes in sporadic colorectal tumors and to investigate the clinical relevance of 3’UTR variations in miRNA profiles. Methods: In the study, the exonic and 3’UTR sequences of five genes in 12 sporadic colorectal tumors were extracted by next generation sequencing. In tumors with variation in the 3’UTR region, the changes caused by the variation in the miRNA binding profile were detected. The expression profile of these miRNAs in colorectal and other solid tumors compared to normal tissue was determined. Pathway analysis was performed to determine which signaling pathways miRNAs affect. Results: Case-10 in our study was wild type KRAS and received cetuximab treatment and developed drug resistance. In this case, it was concluded that the expression of KRAS increased and tumorigenesis progressed due to miRNAs that do not bind to this region due to variations in the 3’UTR region. Among these miRNAs, hsa-miR-124-3p was found to have decreased expression in colorectal tumors and to be associated with the ECM-receptor interaction pathway. Conclusion: Variations in the 3’UTR regions of genes critical in the process of carsinogenesis are associated with drug resistance and the process of tumorigenesis.展开更多
Brain metastases from solid tumours are associated with poor prognosis despite aggressive treatment. Temozolomide can be used for the treatment of glioblastoma multiforme as well as melanoma. It has also been shown to...Brain metastases from solid tumours are associated with poor prognosis despite aggressive treatment. Temozolomide can be used for the treatment of glioblastoma multiforme as well as melanoma. It has also been shown to have activity in patients with brain metastases from various malignancies, since it can cross the blood-brain barrier. To better understand the efficacy of temozolomide in the treatment of brain metastases, we carried out a review of 21 published clinical trials to determine whether temozolomide would benefit patients with brain metastases from solid tumours. Information regarding complete response, partial response, stable disease, objective response and objective response rate were collected to assess clinical outcomes. A modest therapeutic effect was observed when temozolomide was used as a single agent, however, the combination of temozolomide with whole-brain radiotherapy and/or other anticancer drugs exhibited encouraging activity. Thus, future high quality studies are warranted to confirm our findings.展开更多
Pancreatic neuroendocrine tumours are uncommon neoplasms of the pancreas, accounting for around 2% of all primary pancreatic tumors. This specific group of tumours includes insulinoma, gastrinoma, glucagonoma, VIPoma,...Pancreatic neuroendocrine tumours are uncommon neoplasms of the pancreas, accounting for around 2% of all primary pancreatic tumors. This specific group of tumours includes insulinoma, gastrinoma, glucagonoma, VIPoma, somatostatinoma and others. They may cause a clinical syndrome due to hormone overproduction. These tumours tend to be less aggressive than pancreatic adenocarcinoma, however, more than 50% of them have metastasized to the liver at the time of diagnosis. Under this circumstance, surgery is impossible to resect all metastases. Thus other various medical measures have been explored in the treatment of these tumors.展开更多
Pancreatic neuroendocrine neoplasms are a rare and complex group of neoplastic lesions that develop from pancreatic islet cells.Their incidence has dramatically increased during the last two decades.Due to its complex...Pancreatic neuroendocrine neoplasms are a rare and complex group of neoplastic lesions that develop from pancreatic islet cells.Their incidence has dramatically increased during the last two decades.Due to its complex nature and pathophysiological behaviour,surgical management continues to evolve.Surgery remains the cornerstone of treatment for most non-functional and functional pancreatic neuroendocrine tumours,while lymphadenectomy remains a controversial subject.Different techniques,such as pancreas-preserving and minimally invasive approaches,continue to evolve and offer the same overall outcomes as open surgery.This comprehensive review describes in detail the current and most up-todate classification and staging of pancreatic neuroendocrine tumours,explores the rationale for nonsurgical and surgical management,and focuses on surgical treatment and more specifically,on minimally invasive approaches.展开更多
Mixed neuroendocrine non-neuroendocrine neoplasms(MiNENs)are a hetero-geneous group of malignant neoplasms that can settle in the gastroenteropan-creatic tract.They are composed of a neuroendocrine(NE)and a non-NE com...Mixed neuroendocrine non-neuroendocrine neoplasms(MiNENs)are a hetero-geneous group of malignant neoplasms that can settle in the gastroenteropan-creatic tract.They are composed of a neuroendocrine(NE)and a non-NE compo-nent in at least 30%of each tumour.The non-NE component can include different histological combinations of glandular,squamous,mucinous and sarcomatoid phenotypes,and one or both of the components can be low-or high grade malignant.Recent changes in the nomenclature of these neoplasms might lead to great deal of confusion,and the lack of specific clinical trials is the main reason why their management is difficult.The review aims to clarify the definition of MiNEN and analyze available evidence about their diagnosis and treatment options according to their location and extension through careful analysis of the available data.It would be important to reach a general consensus on their diagnosis in order to construct a classification that remains stable over time and facilitates the design of clinical trials that,due to their low incidence,will require long recruitment periods.展开更多
BACKGROUND Endoscopic submucosal dissection(ESD)and transanal endoscopic submucosal dissection(TES)are widely employed surgical techniques.However,the comparative efficacy and safety of both remain inconclusive.AIM To...BACKGROUND Endoscopic submucosal dissection(ESD)and transanal endoscopic submucosal dissection(TES)are widely employed surgical techniques.However,the comparative efficacy and safety of both remain inconclusive.AIM To comprehensively analyze and discern differences in surgical outcomes between ESD and TES.METHODS We conducted a systematic search of the electronic databases PubMed,Embase,Cochrane Central Register of Controlled Trials,Scopus,and CINAHL from inception till August 2023.We analyzed outcomes including recurrence rate,en bloc resection,R0 resection rate,perforation rate,procedure length,and hospital stay length applying a random-effects inverse-variance model.We assessed publication bias by conducting an Egger’s regression test and sensitivity analyses.RESULTS We pooled data from 11 studies involving 1013 participants.We found similar recurrence rates,with a pooled odds ratio of 0.545(95%CI:0.176-1.687).En bloc resection,R0 resection,and perforation rate values were also similar for both ESD and TES.The pooled analysis for procedure length indicated a mean difference of-4.19 min(95%CI:-22.73 to 14.35),and the hospital stay was on average shorter for ESDs by about 0.789 days(95%CI:-1.671 to 0.093).CONCLUSION Both ESD and TES displayed similar efficacy and safety profiles across multiple outcomes.Our findings show that individualized patient and surgeon preferences,alongside specific clinical contexts,can be considered when selecting between these two techniques.展开更多
Endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP)-guided ablation procedures are emerging as a minimally invasive therapeutic alternative to radiological and surgical treatments for...Endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP)-guided ablation procedures are emerging as a minimally invasive therapeutic alternative to radiological and surgical treatments for locally advanced pancreatic cancer (LAPC), pancreatic neuroendocrine tumours (PNETs), and pancreatic cystic lesions (PCLs). The advantages of treatment under endoscopic control are the real-time imaging guidance and the possibility to reach a deep target like the pancreas. Currently, radiofrequency probes specifically designed for ERCP or EUS ablation are available as well as hybrid cryotherm probe combining radiofrequency with cryotechnology. To date, many reports and case series have confirmed the safety and feasibility of that kind of ablation technique in the pancreatic setting. Moreover, EUS-guided fine-needle injection is emerging as a method to deliver ablative and anti-tumoral agents inside the tumuor. Ethanol injection has been proposed mostly for the treatment of PCLs and for symptomatic functioning PNETs, and the use of gemcitabine and paclitaxel is also interesting in this setting. EUS-guided injection of chemical or biological agents including mixed lymphocyte culture, oncolytic viruses, and immature dendritic cells has been investigated for the treatment of LAPC. Data on the long-term efficacy of these approaches, and large prospective randomized studies are needed to confirm the real clinical benefits of these techniques for the management of pancreatic lesions.展开更多
Man made electromagnetic irradiation and fields cover now the globe due to the recent extensive propagation of mobile telephony.The increased load affects animals and also plants.Especially birds have been studied.Hum...Man made electromagnetic irradiation and fields cover now the globe due to the recent extensive propagation of mobile telephony.The increased load affects animals and also plants.Especially birds have been studied.Humans are also sensitive.They are good bioindicators as epidemiological methods are available.Humans can also report symptoms which cannot be directly measured with presently available technologies.The nonionizing irradiation can as the ionizing one break the DNA,damage proteins,even increase the blood brain barrier permeability,disturb the night rest,cause fatigue and hormonal disturbances.An increase of the tumours of human head has been described in correlation with the long term mobile phone use and on that side more exposed.The regulations covering mobile telephony are already about two decades old and need re-evaluation.The multitude of irradiation and the interaction of the different wavelength exposures,i.e.,frequency sensitivity is poorly known at present.We should not forget the comparative studies of different species especially those which rely in their lives on electromagnetic orientation physiology.Some countries have issued warnings on the exposures of children.The producers of mobile technology have recently warned the users not to keep those devices in active stage in skin contact.展开更多
Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical,morphological,and immunohistochemical features.This article summarizes the clinical and pathologic...Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical,morphological,and immunohistochemical features.This article summarizes the clinical and pathological features of primary renal angiosarcoma and anastomosing haemangioma of the kidney including epidemiology,genetics,and prognosis.Renal anastomosing haemangiomas are benign neoplasms characterized by anastomosing capillary-sized vascular channels.These tumours are rare,with about 75 cases reported in the literature.Most anastomosing haemangiomas are found incidentally on ultrasound,computed tomography,or magnetic resonance imaging.Common symptoms include abdominal pain,haematuria,and abdominal mass.Renal anastomosing haemangiomas are characterized by recurrent mutations in GNAQ and GNA14 genes.The prognosis of anastomosing haemangioma is excellent.Primary renal angiosarcomas are malignant tumours showing endothelial differentiation.To date,76 cases have been described in the literature.Primary renal angiosarcomas are frequently symptomatic.The clinical features of renal angiosarcomas are similar to those of renal anastomosing haemangiomas,including abdominal pain,haematuria,and abdominal mass.Angiogenesis-related genes and vascular-specific receptor tyrosine kinases such as KDR,TIE1,SNRK,TEK,and FLT1 are upregulated in angiosarcomas.Primary renal angiosarcomas are highly aggressive neoplasms with a poor prognosis despite surgical treatment,chemotherapy,radiotherapy,or targeted therapy.展开更多
BACKGROUND The prognostic significance of lymph nodes(LNs)metastases and the optimum number of LN yield in gastroenteropancreatic neuroendocrine tumours(GEP NETs)undergoing curative resection is still debatable.Many s...BACKGROUND The prognostic significance of lymph nodes(LNs)metastases and the optimum number of LN yield in gastroenteropancreatic neuroendocrine tumours(GEP NETs)undergoing curative resection is still debatable.Many studies have demonstrated that cure rate for patients with GEP NETs can be improved by the resection of the primary tumour and regional lymphadenectomy AIM To evaluate the effect of lymph node(LN)status and yield on relapse-free survival(RFS)and overall survival(OS)in patients with resected GEP NETs.METHODS Data on patients who underwent curative resection for GEP NETs between January 2002 and March 2017 were analysed retrospectively.Grade 3 tumours(Ki67>20%)were excluded.Univariate Cox proportional hazard models were computed for RFS and OS and assessed alongside cut-point analysis to distinguish a suitable binary categorisation of total LNs retrieved associated with RFS.RESULTS A total of 217 patients were included in the study.The median age was 59 years(21-97 years)and 51%(n=111)were male.Primary tumour sites were small bowel(42%),pancreas(25%),appendix(18%),rectum(7%),colon(3%),gastric(2%),others(2%).Median follow up times for all patients were 41 mo(95%CI:36-51)and 71 mo(95%CI:63–76)for RFS and OS respectively;50 relapses and 35 deaths were reported.LNs were retrieved in 151 patients.Eight or more LNs were harvested in 106 patients and LN positivity reported in 114 patients.Three or more positive LNs were detected in 62 cases.The result of univariate analysis suggested perineural invasion(P=0.0023),LN positivity(P=0.033),LN retrieval of≥8(P=0.047)and localisation(P=0.0049)have a statistically significant association with shorter RFS,but there was no effect of LN ratio on RFS:P=0.1 or OS:P=0.75.Tumour necrosis(P=0.021)and perineural invasion(P=0.016)were the only two variables significantly associated with worse OS.In the final multivariable analysis,localisation(pancreas HR=27.33,P=0.006,small bowel HR=32.44,P=0.005),and retrieval of≥8 LNs(HR=2.7,P=0.036)were independent prognostic factors展开更多
BACKGROUND Synovial sarcoma(SS) is a rare type of soft tissue sarcoma that is usually developed from areas where synovial tissue exists, especially at the extremities.Nevertheless, several cases of retroperitoneal SS(...BACKGROUND Synovial sarcoma(SS) is a rare type of soft tissue sarcoma that is usually developed from areas where synovial tissue exists, especially at the extremities.Nevertheless, several cases of retroperitoneal SS(RSS) have been described. We herein report a case of RSS presented in our institution.CASE SUMMARY A 69-year-old female patient was admitted with a large, palpable, firm mass in the right abdominal space SS. Computerized tomography scan depicted a concentric, sharply marinated retro-peritoneal lesion which was displacing the right kidney and the lower edge of the liver. Subsequently, the patient underwent surgical excision of the mass with additional right nephrectomy and resection of the right adrenal gland and a part of the diaphragm. The final histological diagnosis of the tumour was grade II monophasic RSS.CONCLUSION RSS is encountered in the biphasic type, the monophasic fibrous, and the monophasic epithelial category as well. Relevant clinical manifestations are not always documented at early stages. Therefore, the final diagnosis is posed after complete histological examination taking into consideration the results of immunochemistry and genetic analysis. Therapeutic approach happens often late when metastases at the lungs and the liver are apparent. Thus, 5-year survival rates remain low.展开更多
文摘Gastric submucosal tumors(SMTs) are a rather frequent finding,occurring in about 0.36%of routine upper GIendoscopies.Endoscopic ultrasonography(EUS) has emerged as a reliable investigative procedure for evaluation of these lesions.Diagnostic EUS has the ability to differentiate intramural tumors from extraluminal compressions and can also show the layer of origin of gastric SMTs.Tumors can be further characterized by their layer of origin,echo pattern and margin.EUS-risk criteria of their malignant potential are presented,although the emergence of EUS-FNA has opened new indications for transmural tissue diagnosis and expanded the possibilities of EUS in SMTs of the stomach.Tissue diagnosis should address whether the SMT is a Gastrointestinal stromal tumour(GIST) or another tumor type and evaluate the malignant potential of a given GIST.However,there seems to be a lack of data on the optimal strategy in SMTs suspected to be GISTs with a negative EUS-FNA tissue diagnosis.The current management strategies,as well as open questions regarding their treatment are also presented.
文摘AIM:To evaluate the effects of preoperative immunonutrition and other nutrition models on the cellular immunity parameters of patients with gastrointestinal tumors before surgical intervention.In addition,effects on postoperative complications were examined. METHODS:Patients with gastrointestinal tumors were randomized into 3 groups.The immunonutrition group received a combination of arginine,fatty acids and nucleotides.The second and third group received normal nutrition and standard enteral nutrition,respectively.Nutrition protocols were administered for 7 d prior to the operation.Nutritional parameters,in particular prealbumin levels and lymphocyte subpopulations(CD4+,CD8+,CD16+/56+,and CD69 cells)were evaluated before and after the nutrition protocols.Groups were compared in terms of postoperative complications and duration of hospital stay. RESULTS:Of the 42 patients who completed thestudy,16 received immunonutrition,13 received normal nutrition and 13 received standard enteral nutrition. prealbumin values were low in every group,but this parameter was improved after the nutritional protocol only in the immunonutrition group(13.64±8.83 vs 15.98±8.66,P=0.037).Groups were similar in terms of CD4+,CD16+/56,and CD69+prior to the nutritional protocol;whereas CD8+was higher in the standard nutrition group compared to the immunonutrition group.After nutritional protocols,none of the groups had an increase in their lymphocyte subpopulations.Also,groups did not differ in terms of postoperative complications and postoperative durations of hospital stay. CONCLUSION:Preoperative immunonutrition provided a significant increase in prealbumin levels,while it did not significantly alter T lymphocyte subpopulation counts,the rate of postoperative complications and the duration of hospital stay.
文摘Solid pseudopapillary neoplasms are rare.This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas,including the epidemiology,cytology,molecular pathology,differential diagnosis,treatment,and prognosis.Solid pseudopapillary neoplasms are low-grade malignant tumours of the pancreas characterized by poorly cohesive epithelial cells with solid and pseudopapillary patterns.Solid pseudopapillary neoplasms occur predominantly in young women.Although solid pseudopapillary neoplasms can occur throughout the pancreas,they arise slightly more frequently in the tail of the pancreas.The aetiology is unknown.Extremely rare cases have been reported in the setting of familial adenomatous polyposis.There are no symptoms unique to solid pseudopapillary neoplasms,however,the most common symptom is abdominal pain or discomfort.The features of solid pseudopapillary neoplasms on computed tomography imaging are indicative of the pathologic changes within the tumour.Typically,well-demarcated masses with variably solid and cystic appearances.Microscopically,these tumours are composed of epithelial cells forming solid and pseudopapillary structures,frequently undergoing haemorrhagic cystic degeneration.Typically,these tumours express nuclear and/or cytoplasmicβ-catenin.Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1,the gene encodingβ-catenin.The overall prognosis is excellent,and most patients are cured by complete surgical resection.
文摘Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas,which may pose diagnostic difficulties.Because of its rarity,little is known about its clinical and pathologic features.This article reviews the clinical and pathologic features of pancreatoblastoma in adults including differential diagnosis,treatment,and follow-up.Although pancreatoblastoma commonly occurs in childhood,there have now been more than 70 adult pancreatoblastomas described in the literature.There is a slight male predominance.There are no symptoms unique to pancreatoblastomas and adult patients are frequently symptomatic.The most common presenting symptom is abdominal pain.Grossly,the tumours are often large and well-circumscribed.Microscopically,pancreatoblastomas are composed of neoplastic cells with predominantly acinar differentiation and characteristic squamoid nests.These tumours are positive for trypsin,chymotrypsin,lipase,and BCL10.Loss of heterozygosity on chromosome 11p is the most common molecular alteration in pancreatoblastomas.Adult pancreatoblastomas are aggressive tumours with frequent local invasion,recurrence,and distant metastasis.Treatment consists of surgical resection.Chemotherapy and radiotherapy may have a role in the treatment of recurrent,residual,unresectable,and metastatic disease.It is important to distinguish pancreatoblastomas from morphological mimics such as acinar cell carcinomas,solid pseudopapillary neoplasms,and pancreatic neuroendocrine neoplasms.
基金partially supported by a grant for Practical Research Project for Rare/Intractable Diseases (JP17ek0109106) from the Japan Agency for Medical Research and Development a Grant-in-Aid for Specially Promoted Research from the Japan Society for Promotion of Science (JSPS) (15H05703)+3 种基金a Grant-in-Aid for Young Scientists A from JSPS (15H05653)a Grantin-Aid for Scientific Research (B) from JSPS (18H02919)a Grant-in-Aid for Challenging Research (Pioneering) from JSPS (17K19582)grants from Mitsui Life Social Welfare Foundation and Kobayashi Foundation for Cancer Research
文摘Bone is one of the preferred sites for the metastasis of malignant tumours,such as breast cancer,lung cancer and malignant melanoma.Tumour cells colonizing bone have the capacity to induce the expression of receptor activator of nuclear factor-κB ligand(RANKL),which promotes osteoclast differentiation and activation.Tumour-induced osteoclastic bone resorption leads to a vicious cycle between tumours and bone cells that fuels osteolytic tumour growth,causing bone pain and hypercalcaemia.Furthermore,RANKL contributes to bone metastasis by acting as a chemoattractant to bone for tumour cells that express its receptor,RANK.Thus inhibition of the RANKL–RANK pathway is a promising treatment for bone metastasis,and a human monoclonal anti-RANKL antibody,denosumab,has been used in the clinic.However,orally available drugs targeting RANKL must be developed to increase the therapeutic benefits to patients.Here we report the efficacy of the small-molecule RANKL inhibitor AS2676293 in treating bone metastasis using mouse models.Oral administration of AS2676293 markedly inhibited bone metastasis of human breast cancer cells MDA-MB-231-5a-D-Luc2 as well as tumour-induced osteolysis.AS2676293 suppressed RANKLmediated tumour migration in the transwell assay and inhibited bone metastasis of the murine cell line B16F10,which is known not to trigger osteoclast activation.Based on the results from this study,RANKL inhibition with a small-molecule compound constitutes a promising therapeutic strategy for treating bone metastasis by inhibiting both osteoclastic bone resorption and tumour migration to bone.
文摘Aim: To reveal the exonic and 3’UTR sequences of KRAS, TP53, APC, BRAF, PIK3CA genes in sporadic colorectal tumors and to investigate the clinical relevance of 3’UTR variations in miRNA profiles. Methods: In the study, the exonic and 3’UTR sequences of five genes in 12 sporadic colorectal tumors were extracted by next generation sequencing. In tumors with variation in the 3’UTR region, the changes caused by the variation in the miRNA binding profile were detected. The expression profile of these miRNAs in colorectal and other solid tumors compared to normal tissue was determined. Pathway analysis was performed to determine which signaling pathways miRNAs affect. Results: Case-10 in our study was wild type KRAS and received cetuximab treatment and developed drug resistance. In this case, it was concluded that the expression of KRAS increased and tumorigenesis progressed due to miRNAs that do not bind to this region due to variations in the 3’UTR region. Among these miRNAs, hsa-miR-124-3p was found to have decreased expression in colorectal tumors and to be associated with the ECM-receptor interaction pathway. Conclusion: Variations in the 3’UTR regions of genes critical in the process of carsinogenesis are associated with drug resistance and the process of tumorigenesis.
文摘Brain metastases from solid tumours are associated with poor prognosis despite aggressive treatment. Temozolomide can be used for the treatment of glioblastoma multiforme as well as melanoma. It has also been shown to have activity in patients with brain metastases from various malignancies, since it can cross the blood-brain barrier. To better understand the efficacy of temozolomide in the treatment of brain metastases, we carried out a review of 21 published clinical trials to determine whether temozolomide would benefit patients with brain metastases from solid tumours. Information regarding complete response, partial response, stable disease, objective response and objective response rate were collected to assess clinical outcomes. A modest therapeutic effect was observed when temozolomide was used as a single agent, however, the combination of temozolomide with whole-brain radiotherapy and/or other anticancer drugs exhibited encouraging activity. Thus, future high quality studies are warranted to confirm our findings.
文摘Pancreatic neuroendocrine tumours are uncommon neoplasms of the pancreas, accounting for around 2% of all primary pancreatic tumors. This specific group of tumours includes insulinoma, gastrinoma, glucagonoma, VIPoma, somatostatinoma and others. They may cause a clinical syndrome due to hormone overproduction. These tumours tend to be less aggressive than pancreatic adenocarcinoma, however, more than 50% of them have metastasized to the liver at the time of diagnosis. Under this circumstance, surgery is impossible to resect all metastases. Thus other various medical measures have been explored in the treatment of these tumors.
文摘Pancreatic neuroendocrine neoplasms are a rare and complex group of neoplastic lesions that develop from pancreatic islet cells.Their incidence has dramatically increased during the last two decades.Due to its complex nature and pathophysiological behaviour,surgical management continues to evolve.Surgery remains the cornerstone of treatment for most non-functional and functional pancreatic neuroendocrine tumours,while lymphadenectomy remains a controversial subject.Different techniques,such as pancreas-preserving and minimally invasive approaches,continue to evolve and offer the same overall outcomes as open surgery.This comprehensive review describes in detail the current and most up-todate classification and staging of pancreatic neuroendocrine tumours,explores the rationale for nonsurgical and surgical management,and focuses on surgical treatment and more specifically,on minimally invasive approaches.
文摘Mixed neuroendocrine non-neuroendocrine neoplasms(MiNENs)are a hetero-geneous group of malignant neoplasms that can settle in the gastroenteropan-creatic tract.They are composed of a neuroendocrine(NE)and a non-NE compo-nent in at least 30%of each tumour.The non-NE component can include different histological combinations of glandular,squamous,mucinous and sarcomatoid phenotypes,and one or both of the components can be low-or high grade malignant.Recent changes in the nomenclature of these neoplasms might lead to great deal of confusion,and the lack of specific clinical trials is the main reason why their management is difficult.The review aims to clarify the definition of MiNEN and analyze available evidence about their diagnosis and treatment options according to their location and extension through careful analysis of the available data.It would be important to reach a general consensus on their diagnosis in order to construct a classification that remains stable over time and facilitates the design of clinical trials that,due to their low incidence,will require long recruitment periods.
文摘BACKGROUND Endoscopic submucosal dissection(ESD)and transanal endoscopic submucosal dissection(TES)are widely employed surgical techniques.However,the comparative efficacy and safety of both remain inconclusive.AIM To comprehensively analyze and discern differences in surgical outcomes between ESD and TES.METHODS We conducted a systematic search of the electronic databases PubMed,Embase,Cochrane Central Register of Controlled Trials,Scopus,and CINAHL from inception till August 2023.We analyzed outcomes including recurrence rate,en bloc resection,R0 resection rate,perforation rate,procedure length,and hospital stay length applying a random-effects inverse-variance model.We assessed publication bias by conducting an Egger’s regression test and sensitivity analyses.RESULTS We pooled data from 11 studies involving 1013 participants.We found similar recurrence rates,with a pooled odds ratio of 0.545(95%CI:0.176-1.687).En bloc resection,R0 resection,and perforation rate values were also similar for both ESD and TES.The pooled analysis for procedure length indicated a mean difference of-4.19 min(95%CI:-22.73 to 14.35),and the hospital stay was on average shorter for ESDs by about 0.789 days(95%CI:-1.671 to 0.093).CONCLUSION Both ESD and TES displayed similar efficacy and safety profiles across multiple outcomes.Our findings show that individualized patient and surgeon preferences,alongside specific clinical contexts,can be considered when selecting between these two techniques.
文摘Endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP)-guided ablation procedures are emerging as a minimally invasive therapeutic alternative to radiological and surgical treatments for locally advanced pancreatic cancer (LAPC), pancreatic neuroendocrine tumours (PNETs), and pancreatic cystic lesions (PCLs). The advantages of treatment under endoscopic control are the real-time imaging guidance and the possibility to reach a deep target like the pancreas. Currently, radiofrequency probes specifically designed for ERCP or EUS ablation are available as well as hybrid cryotherm probe combining radiofrequency with cryotechnology. To date, many reports and case series have confirmed the safety and feasibility of that kind of ablation technique in the pancreatic setting. Moreover, EUS-guided fine-needle injection is emerging as a method to deliver ablative and anti-tumoral agents inside the tumuor. Ethanol injection has been proposed mostly for the treatment of PCLs and for symptomatic functioning PNETs, and the use of gemcitabine and paclitaxel is also interesting in this setting. EUS-guided injection of chemical or biological agents including mixed lymphocyte culture, oncolytic viruses, and immature dendritic cells has been investigated for the treatment of LAPC. Data on the long-term efficacy of these approaches, and large prospective randomized studies are needed to confirm the real clinical benefits of these techniques for the management of pancreatic lesions.
文摘Man made electromagnetic irradiation and fields cover now the globe due to the recent extensive propagation of mobile telephony.The increased load affects animals and also plants.Especially birds have been studied.Humans are also sensitive.They are good bioindicators as epidemiological methods are available.Humans can also report symptoms which cannot be directly measured with presently available technologies.The nonionizing irradiation can as the ionizing one break the DNA,damage proteins,even increase the blood brain barrier permeability,disturb the night rest,cause fatigue and hormonal disturbances.An increase of the tumours of human head has been described in correlation with the long term mobile phone use and on that side more exposed.The regulations covering mobile telephony are already about two decades old and need re-evaluation.The multitude of irradiation and the interaction of the different wavelength exposures,i.e.,frequency sensitivity is poorly known at present.We should not forget the comparative studies of different species especially those which rely in their lives on electromagnetic orientation physiology.Some countries have issued warnings on the exposures of children.The producers of mobile technology have recently warned the users not to keep those devices in active stage in skin contact.
文摘Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical,morphological,and immunohistochemical features.This article summarizes the clinical and pathological features of primary renal angiosarcoma and anastomosing haemangioma of the kidney including epidemiology,genetics,and prognosis.Renal anastomosing haemangiomas are benign neoplasms characterized by anastomosing capillary-sized vascular channels.These tumours are rare,with about 75 cases reported in the literature.Most anastomosing haemangiomas are found incidentally on ultrasound,computed tomography,or magnetic resonance imaging.Common symptoms include abdominal pain,haematuria,and abdominal mass.Renal anastomosing haemangiomas are characterized by recurrent mutations in GNAQ and GNA14 genes.The prognosis of anastomosing haemangioma is excellent.Primary renal angiosarcomas are malignant tumours showing endothelial differentiation.To date,76 cases have been described in the literature.Primary renal angiosarcomas are frequently symptomatic.The clinical features of renal angiosarcomas are similar to those of renal anastomosing haemangiomas,including abdominal pain,haematuria,and abdominal mass.Angiogenesis-related genes and vascular-specific receptor tyrosine kinases such as KDR,TIE1,SNRK,TEK,and FLT1 are upregulated in angiosarcomas.Primary renal angiosarcomas are highly aggressive neoplasms with a poor prognosis despite surgical treatment,chemotherapy,radiotherapy,or targeted therapy.
文摘BACKGROUND The prognostic significance of lymph nodes(LNs)metastases and the optimum number of LN yield in gastroenteropancreatic neuroendocrine tumours(GEP NETs)undergoing curative resection is still debatable.Many studies have demonstrated that cure rate for patients with GEP NETs can be improved by the resection of the primary tumour and regional lymphadenectomy AIM To evaluate the effect of lymph node(LN)status and yield on relapse-free survival(RFS)and overall survival(OS)in patients with resected GEP NETs.METHODS Data on patients who underwent curative resection for GEP NETs between January 2002 and March 2017 were analysed retrospectively.Grade 3 tumours(Ki67>20%)were excluded.Univariate Cox proportional hazard models were computed for RFS and OS and assessed alongside cut-point analysis to distinguish a suitable binary categorisation of total LNs retrieved associated with RFS.RESULTS A total of 217 patients were included in the study.The median age was 59 years(21-97 years)and 51%(n=111)were male.Primary tumour sites were small bowel(42%),pancreas(25%),appendix(18%),rectum(7%),colon(3%),gastric(2%),others(2%).Median follow up times for all patients were 41 mo(95%CI:36-51)and 71 mo(95%CI:63–76)for RFS and OS respectively;50 relapses and 35 deaths were reported.LNs were retrieved in 151 patients.Eight or more LNs were harvested in 106 patients and LN positivity reported in 114 patients.Three or more positive LNs were detected in 62 cases.The result of univariate analysis suggested perineural invasion(P=0.0023),LN positivity(P=0.033),LN retrieval of≥8(P=0.047)and localisation(P=0.0049)have a statistically significant association with shorter RFS,but there was no effect of LN ratio on RFS:P=0.1 or OS:P=0.75.Tumour necrosis(P=0.021)and perineural invasion(P=0.016)were the only two variables significantly associated with worse OS.In the final multivariable analysis,localisation(pancreas HR=27.33,P=0.006,small bowel HR=32.44,P=0.005),and retrieval of≥8 LNs(HR=2.7,P=0.036)were independent prognostic factors
文摘BACKGROUND Synovial sarcoma(SS) is a rare type of soft tissue sarcoma that is usually developed from areas where synovial tissue exists, especially at the extremities.Nevertheless, several cases of retroperitoneal SS(RSS) have been described. We herein report a case of RSS presented in our institution.CASE SUMMARY A 69-year-old female patient was admitted with a large, palpable, firm mass in the right abdominal space SS. Computerized tomography scan depicted a concentric, sharply marinated retro-peritoneal lesion which was displacing the right kidney and the lower edge of the liver. Subsequently, the patient underwent surgical excision of the mass with additional right nephrectomy and resection of the right adrenal gland and a part of the diaphragm. The final histological diagnosis of the tumour was grade II monophasic RSS.CONCLUSION RSS is encountered in the biphasic type, the monophasic fibrous, and the monophasic epithelial category as well. Relevant clinical manifestations are not always documented at early stages. Therefore, the final diagnosis is posed after complete histological examination taking into consideration the results of immunochemistry and genetic analysis. Therapeutic approach happens often late when metastases at the lungs and the liver are apparent. Thus, 5-year survival rates remain low.
