Several pathohistological classification systems exist for the diagnosis of gastric cancer. Many studies have investigated the correlation between the pathohistological characteristics in gastric cancer and patient ch...Several pathohistological classification systems exist for the diagnosis of gastric cancer. Many studies have investigated the correlation between the pathohistological characteristics in gastric cancer and patient characteristics, disease specific criteria and overall outcome. It is still controversial as to which classification system imparts the most reliable information, and therefore, the choice of system may vary in clinical routine. In addition to the most common classification systems, such as the Laurén and the World Health Organization (WHO) classifications, other authors have tried to characterize and classify gastric cancer based on the microscopic morphology and in reference to the clinical outcome of the patients. In more than 50 years of systematic classification of the pathohistological characteristics of gastric cancer, there is no sole classification system that is consistently used worldwide in diagnostics and research. However, several national guidelines for the treatment of gastric cancer refer to the Laurén or the WHO classifications regarding therapeutic decision-making, which underlines the importance of a reliable classification system for gastric cancer. The latest results from gastric cancer studies indicate that it might be useful to integrate DNA- and RNA-based features of gastric cancer into the classification systems to establish prognostic relevance. This article reviews the diagnostic relevance and the prognostic value of different pathohistological classification systems in gastric cancer.展开更多
Intravascular large B-cell lymphoma(IVLBCL),a rare subtype of non-Hodgkin lymphoma,is classified as an independent subtype of extranodal diffuse large B-cell lymphoma(DLBCL)in the 2008 World Health Organization(WHO)Cl...Intravascular large B-cell lymphoma(IVLBCL),a rare subtype of non-Hodgkin lymphoma,is classified as an independent subtype of extranodal diffuse large B-cell lymphoma(DLBCL)in the 2008 World Health Organization(WHO)Classification(Turner et al.,2010).The 5th edition of the World Health Organization(WHO 2022)classification of hematolymphoid tumors retains this subtype(Alaggio et al.,2022).IVLBCL,which is characterized by neoplastic lymphocyte proliferation within the lumen of small blood vessels,tends to invade organs,such as the nervous system,skin,bone marrow(BM),and lung(D'Angelo et al.,2019;Satoh et al.,2019;Vasquez et al.,2019;Fukami et al.,2020).展开更多
AIMTo describe magnetic resonance(MR)imaging features of pancreatic neuroendocrine neoplasms(PanNENs)according to their grade and tumor-nodes-metastases stage by comparing them to histopathology and to determine the a...AIMTo describe magnetic resonance(MR)imaging features of pancreatic neuroendocrine neoplasms(PanNENs)according to their grade and tumor-nodes-metastases stage by comparing them to histopathology and to determine the accuracy of MR imaging features in predicting their biological behavior.METHODSThis study was approved by our institutional review board;requirement for informed patient consent was waived due to the retrospective nature of the study.Preoperative MR examinations of 55 PanNEN patients(29 men,26 women;mean age of 57.6 years,range 21-83 years)performed between June 2013 and December 2015 were reviewed.Qualitative and quantitative features were compared between tumor grades and stages determined by histopathological analysis.RESULTSIll defined margins were more common in G2-3 and stage III-IV PanNENs than in G1 and low-stage tumors(P<0.001);this feature had high specificity in the identification of G2-3 and stage III-IV tumors(90.3%and 96%,95%CI:73.1-97.5 and 77.7-99.8).The mean apparent diffusion coefficient value was significantly lower in G2-3 and stage III-IV lesions compared to well differentiated and low-stage tumors(1.09×10<sup>-3</sup>mm<sup>2</sup>/s vs 1.45×10<sup>-3</sup>mm<sup>2</sup>/s and 1.10×10<sup>-3</sup>mm<sup>2</sup>/s vs 1.53×10<sup>-3</sup>mm<sup>2</sup>/s,P=0.003 and 0.001).Receiving operator characteristic analysis determined optimal cut-offs of 1.21 and 1.28×10<sup>-3</sup>mm<sup>2</sup>/s for the identification of G2-3 and stage III-IV tumors,with sensitivity and specificity values of 70.8/80.7%and 64.5/64%(95%CI:48.7-86.6/60-92.7 and 45.4-80.2/42.6-81.3).CONCLUSIONMR features of PanNENs vary according to their grade of differentiation and their stage at diagnosis and could predict the biological behavior of these tumors.展开更多
Gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs)are rare epithelial neoplasms derived from pluripotent endocrine cells along the gastrointestinal tract and pancreas.GEP-NENs are classified into well-different...Gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs)are rare epithelial neoplasms derived from pluripotent endocrine cells along the gastrointestinal tract and pancreas.GEP-NENs are classified into well-differentiated neuroendocrine tumors and poorly differentiated neuroendocrine carcinomas.Despite overlapping morphological features,GEP-NENs vary in molecular biology,epigenetic,clinical behavior,treatment response,and prognosis features and remain an unmet clinical challenge.In this review,we introduce recent updates on the histopathologic classification,including the tumor grading and staging system,molecular genetics,and systemic evaluation of the diagnosis and treatment of GEP-NENs at different anatomic sites,together with some insights into the diagnosis of challenging and unusual cases.We also discuss the application of novel therapeutic approaches for GEP-NENs,including peptide receptor radionuclide therapy,targeted therapy,and immunotherapy with immune checkpoint inhibitors.These findings will help improve patient care with precise diagnosis and individualized treatment of patients with GEP-NENs.展开更多
Colorectal neuroendocrine tumors (NETs) originate from neuroendocrine cells in the intestinal tract, and represent a small area within oncology, but one which has provided increasing new data during the past years. Al...Colorectal neuroendocrine tumors (NETs) originate from neuroendocrine cells in the intestinal tract, and represent a small area within oncology, but one which has provided increasing new data during the past years. Although the World Health Organization has determined clinical and histological features to predict prognosis for such tumors, they may not be valid on an individual basis. We aim to give an overview of the recent findings with regard to pathology, molecular genetics and diagnosis of NETs.展开更多
Chronic eosinophilic leukemia (CEL) is a rare disorder that is characterized by hypereosinophilia with increased number of blood or marrow blasts (>5% and <20%). CEL is distinguished from hypereosinophilic syndr...Chronic eosinophilic leukemia (CEL) is a rare disorder that is characterized by hypereosinophilia with increased number of blood or marrow blasts (>5% and <20%). CEL is distinguished from hypereosinophilic syndrome (HES) by the presence of eosinophilic clonality. Chronic eosinophilic leukemia not otherwise specified (CEL-NOS) diagnosis is made when no fusion genes are detected by most modern molecular testing, particularly the most common fusion gene FIP1L-1/PDGFRA (Factor Interacting with PAP like-1/Platelet-Derived Growth Factor Receptor Alpha). This disease is very rare, and its description in the literature is not well characterized. We report a fetal case of severe CEL-NOS in a 19-year-old male who presented with a plethora of clinical features consists of constitutional symptoms, pancytopenia, intravascular thrombosis, acute stroke and endomyocardial infiltrates. The course of his disease was aggressive and resistant to conventional treatment. After a short period of improvement, an acute transformation into blast crisis (BC) had occurred. The diagnosis was confirmed by morphology and immunophenotyping of bone marrow biopsy. The patient eventually died of heart failure and sepsis. To our knowledge this is the first case report of fatal CEL-NOS transforming into severe blast crisis.展开更多
1.INTRODUCTION Hematological malignancies with NUP98 rearrangement(NUP98r)were included as recurrent genetic abnormali-ties due to their distinctive clinicopathologic characteristics in the 2022 European LeukmiaNet(EL...1.INTRODUCTION Hematological malignancies with NUP98 rearrangement(NUP98r)were included as recurrent genetic abnormali-ties due to their distinctive clinicopathologic characteristics in the 2022 European LeukmiaNet(ELN)classification,the 2022 World Health Organization(WHO)classification,and the International Consensus Classification.展开更多
Background:To describe the epidemiological characteristics of central nervous system(CNS)tumors in children,based on the neurosurgery department of Beijing Tiantan Hospital.Methods:From January 2015 to December 2019,3...Background:To describe the epidemiological characteristics of central nervous system(CNS)tumors in children,based on the neurosurgery department of Beijing Tiantan Hospital.Methods:From January 2015 to December 2019,3180 children were histopathologically diagnosed with CNS tumors based on the 2016 World Health Organization(WHO)classification of tumors.Patients were 0 to 15 years old.We analyzed age-related gender preferences,tumor locations,and the histological grades of the tumors.In addition,the epidemiological characteristics of the five most common intracranial tumors were compared to the previous studies.Results:In this study,intracranial and spinal tumors account for 96.4%(3066)and 3.6%(114)of all tumors,with a preponderance of supratentorial tumors(57.9%).Among all pediatric patients,low-grade tumors comprise 67.1%(2135).The integral gender ratio of males to females is 1.47:1 and the average age of patients is 7.59 years old.The five most common intracranial tumors are craniopharyngioma(15.4%),medulloblastoma(14.3%),pilocytic astrocytoma(11.8%),diffuse astrocytoma(9.8%),and anaplastic ependymoma(4.8%).Conclusions:Due to the lack of national data on childhood brain tumors,we used a large nationally representative population sample based on the largest pediatric neurosurgery center in China.We analyzed the data of the past 5 years,reflecting the incidence of CNS tumors in Chinese children to a certain extent,and laying a data foundation for subsequent clinical studies.展开更多
文摘Several pathohistological classification systems exist for the diagnosis of gastric cancer. Many studies have investigated the correlation between the pathohistological characteristics in gastric cancer and patient characteristics, disease specific criteria and overall outcome. It is still controversial as to which classification system imparts the most reliable information, and therefore, the choice of system may vary in clinical routine. In addition to the most common classification systems, such as the Laurén and the World Health Organization (WHO) classifications, other authors have tried to characterize and classify gastric cancer based on the microscopic morphology and in reference to the clinical outcome of the patients. In more than 50 years of systematic classification of the pathohistological characteristics of gastric cancer, there is no sole classification system that is consistently used worldwide in diagnostics and research. However, several national guidelines for the treatment of gastric cancer refer to the Laurén or the WHO classifications regarding therapeutic decision-making, which underlines the importance of a reliable classification system for gastric cancer. The latest results from gastric cancer studies indicate that it might be useful to integrate DNA- and RNA-based features of gastric cancer into the classification systems to establish prognostic relevance. This article reviews the diagnostic relevance and the prognostic value of different pathohistological classification systems in gastric cancer.
文摘Intravascular large B-cell lymphoma(IVLBCL),a rare subtype of non-Hodgkin lymphoma,is classified as an independent subtype of extranodal diffuse large B-cell lymphoma(DLBCL)in the 2008 World Health Organization(WHO)Classification(Turner et al.,2010).The 5th edition of the World Health Organization(WHO 2022)classification of hematolymphoid tumors retains this subtype(Alaggio et al.,2022).IVLBCL,which is characterized by neoplastic lymphocyte proliferation within the lumen of small blood vessels,tends to invade organs,such as the nervous system,skin,bone marrow(BM),and lung(D'Angelo et al.,2019;Satoh et al.,2019;Vasquez et al.,2019;Fukami et al.,2020).
文摘AIMTo describe magnetic resonance(MR)imaging features of pancreatic neuroendocrine neoplasms(PanNENs)according to their grade and tumor-nodes-metastases stage by comparing them to histopathology and to determine the accuracy of MR imaging features in predicting their biological behavior.METHODSThis study was approved by our institutional review board;requirement for informed patient consent was waived due to the retrospective nature of the study.Preoperative MR examinations of 55 PanNEN patients(29 men,26 women;mean age of 57.6 years,range 21-83 years)performed between June 2013 and December 2015 were reviewed.Qualitative and quantitative features were compared between tumor grades and stages determined by histopathological analysis.RESULTSIll defined margins were more common in G2-3 and stage III-IV PanNENs than in G1 and low-stage tumors(P<0.001);this feature had high specificity in the identification of G2-3 and stage III-IV tumors(90.3%and 96%,95%CI:73.1-97.5 and 77.7-99.8).The mean apparent diffusion coefficient value was significantly lower in G2-3 and stage III-IV lesions compared to well differentiated and low-stage tumors(1.09×10<sup>-3</sup>mm<sup>2</sup>/s vs 1.45×10<sup>-3</sup>mm<sup>2</sup>/s and 1.10×10<sup>-3</sup>mm<sup>2</sup>/s vs 1.53×10<sup>-3</sup>mm<sup>2</sup>/s,P=0.003 and 0.001).Receiving operator characteristic analysis determined optimal cut-offs of 1.21 and 1.28×10<sup>-3</sup>mm<sup>2</sup>/s for the identification of G2-3 and stage III-IV tumors,with sensitivity and specificity values of 70.8/80.7%and 64.5/64%(95%CI:48.7-86.6/60-92.7 and 45.4-80.2/42.6-81.3).CONCLUSIONMR features of PanNENs vary according to their grade of differentiation and their stage at diagnosis and could predict the biological behavior of these tumors.
文摘Gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs)are rare epithelial neoplasms derived from pluripotent endocrine cells along the gastrointestinal tract and pancreas.GEP-NENs are classified into well-differentiated neuroendocrine tumors and poorly differentiated neuroendocrine carcinomas.Despite overlapping morphological features,GEP-NENs vary in molecular biology,epigenetic,clinical behavior,treatment response,and prognosis features and remain an unmet clinical challenge.In this review,we introduce recent updates on the histopathologic classification,including the tumor grading and staging system,molecular genetics,and systemic evaluation of the diagnosis and treatment of GEP-NENs at different anatomic sites,together with some insights into the diagnosis of challenging and unusual cases.We also discuss the application of novel therapeutic approaches for GEP-NENs,including peptide receptor radionuclide therapy,targeted therapy,and immunotherapy with immune checkpoint inhibitors.These findings will help improve patient care with precise diagnosis and individualized treatment of patients with GEP-NENs.
基金Supported by The Science and Technology Commission of Shanghai Municipality
文摘Colorectal neuroendocrine tumors (NETs) originate from neuroendocrine cells in the intestinal tract, and represent a small area within oncology, but one which has provided increasing new data during the past years. Although the World Health Organization has determined clinical and histological features to predict prognosis for such tumors, they may not be valid on an individual basis. We aim to give an overview of the recent findings with regard to pathology, molecular genetics and diagnosis of NETs.
文摘Chronic eosinophilic leukemia (CEL) is a rare disorder that is characterized by hypereosinophilia with increased number of blood or marrow blasts (>5% and <20%). CEL is distinguished from hypereosinophilic syndrome (HES) by the presence of eosinophilic clonality. Chronic eosinophilic leukemia not otherwise specified (CEL-NOS) diagnosis is made when no fusion genes are detected by most modern molecular testing, particularly the most common fusion gene FIP1L-1/PDGFRA (Factor Interacting with PAP like-1/Platelet-Derived Growth Factor Receptor Alpha). This disease is very rare, and its description in the literature is not well characterized. We report a fetal case of severe CEL-NOS in a 19-year-old male who presented with a plethora of clinical features consists of constitutional symptoms, pancytopenia, intravascular thrombosis, acute stroke and endomyocardial infiltrates. The course of his disease was aggressive and resistant to conventional treatment. After a short period of improvement, an acute transformation into blast crisis (BC) had occurred. The diagnosis was confirmed by morphology and immunophenotyping of bone marrow biopsy. The patient eventually died of heart failure and sepsis. To our knowledge this is the first case report of fatal CEL-NOS transforming into severe blast crisis.
基金supported by grant from the National Key R&D Program of China(2019YFA0111000,2022YFC2502700)the National Natural Science Foundation of China(82170158,82470166,82200149,82100175,82200149,82100170)+1 种基金the priority academic program development of Jiangsu Higher Education Institution,the Natural Science Foundation of Jiangsu Province(BK20231195,BK20210087)the Open Project of Jiangsu Biobank of Clinical Resources(SBK202003001,SBK202003003).
文摘1.INTRODUCTION Hematological malignancies with NUP98 rearrangement(NUP98r)were included as recurrent genetic abnormali-ties due to their distinctive clinicopathologic characteristics in the 2022 European LeukmiaNet(ELN)classification,the 2022 World Health Organization(WHO)classification,and the International Consensus Classification.
文摘Background:To describe the epidemiological characteristics of central nervous system(CNS)tumors in children,based on the neurosurgery department of Beijing Tiantan Hospital.Methods:From January 2015 to December 2019,3180 children were histopathologically diagnosed with CNS tumors based on the 2016 World Health Organization(WHO)classification of tumors.Patients were 0 to 15 years old.We analyzed age-related gender preferences,tumor locations,and the histological grades of the tumors.In addition,the epidemiological characteristics of the five most common intracranial tumors were compared to the previous studies.Results:In this study,intracranial and spinal tumors account for 96.4%(3066)and 3.6%(114)of all tumors,with a preponderance of supratentorial tumors(57.9%).Among all pediatric patients,low-grade tumors comprise 67.1%(2135).The integral gender ratio of males to females is 1.47:1 and the average age of patients is 7.59 years old.The five most common intracranial tumors are craniopharyngioma(15.4%),medulloblastoma(14.3%),pilocytic astrocytoma(11.8%),diffuse astrocytoma(9.8%),and anaplastic ependymoma(4.8%).Conclusions:Due to the lack of national data on childhood brain tumors,we used a large nationally representative population sample based on the largest pediatric neurosurgery center in China.We analyzed the data of the past 5 years,reflecting the incidence of CNS tumors in Chinese children to a certain extent,and laying a data foundation for subsequent clinical studies.
