目的探讨胰腺导管内管状乳头状肿瘤(intraductal tubulopapillary neoplasm of the pancreas,ITPN)伴相关浸润性癌的病理形态、免疫表型、基因特征及鉴别诊断。方法回顾性分析江苏省人民医院病理学部2015~2022年确诊的5例ITPN伴有相关...目的探讨胰腺导管内管状乳头状肿瘤(intraductal tubulopapillary neoplasm of the pancreas,ITPN)伴相关浸润性癌的病理形态、免疫表型、基因特征及鉴别诊断。方法回顾性分析江苏省人民医院病理学部2015~2022年确诊的5例ITPN伴有相关浸润性癌,总结其临床及影像学资料、组织病理学、特殊染色、免疫组化特征及分子遗传学改变。结果5例患者中男性3例,女性2例;平均年龄63.2岁;临床症状表现为不同程度的上腹部疼痛、黄疸等。影像提示肿瘤位于胰头部(3例)、壶腹部周围(1例)及全胰(1例),均显示管内实性生长,伴有胰管不同程度扩张;组织学显示肿瘤呈管状生长模式,表现为背靠背密集的腺管结构,缺乏或少见乳头状结构,某些区域呈实性片状生长,巢团中央常见坏死;瘤细胞立方形,异型性明显,核分裂象易见,罕见黏液分化;本组5例均伴有相关浸润性癌,4例为导管腺癌,1例伴有罕见的肉瘤样癌成分。弹力纤维染色示明确浸润区域周围弹力纤维完全缺失;轮廓尚清区域周围弹力纤维部分存在。免疫组织化学显示肿瘤细胞不同程度表达CK7、CK19、MUC1、MUC6,不表达MUC5AC、MUC2、CgA、Syn、胰蛋白酶、糜蛋白酶、脂肪酶、BCL10。我们选取其中3例(1例T1a期,1例T1b期,1例T2期)行KRAS、TP53、FGFR2、HER2及PIK3CA基因检测,结果显示其中1例TP53基因8号外显子框内插入突变,随后对该病例行NGS检测,显示其还存在BRAF、BAP1、FOXO1、PIK3CG等多个基因突变。随访2例死亡,3例尚生存。结论ITPN是一种相对少见的胰腺导管内肿瘤亚型,可伴有相关浸润性癌成分。其具有独特的影像学表现及病理特征,特征性分子遗传学的改变可有望为肿瘤的治疗提供潜在靶点。展开更多
Radiofrequency ablation(RFA),particularly endoscopic ultrasound-guided RFA(EUS-RFA),has emerged as a promising minimally invasive approach for the treatment of pancreatic cancer,especially in patients with locally adv...Radiofrequency ablation(RFA),particularly endoscopic ultrasound-guided RFA(EUS-RFA),has emerged as a promising minimally invasive approach for the treatment of pancreatic cancer,especially in patients with locally advanced or unresectable disease.This review outlines recent technological developments in EUS-RFA,including innovations in energy delivery systems,probe design,and real-time thermal monitoring,which have improved the precision and safety of the procedure.Clinical studies combining EUS-RFA with chemotherapy have demonstrated encouraging outcomes,with improvements in overall survival,progression-free survival,tumor necrosis,and symptom control compared to chemotherapy alone.Additionally,RFA-induced tumor antigen release and modulation of the tumor microenvironment suggest a potential synergistic role with immunotherapy.Despite its promise,the widespread adoption of EUS-RFA is limited by a lack of large-scale randomized controlled trials and standardized treatment protocols.展开更多
BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor,and spontaneous splenic rupture(SSR)as its presenting manifestation is rarely reported among pancreatic mal...BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor,and spontaneous splenic rupture(SSR)as its presenting manifestation is rarely reported among pancreatic malignancies.CASE SUMMARY We herein report a rare case of a 59-year-old female who presented with acute left upper quadrant abdominal pain without any history of trauma.Abdominal imaging demonstrated a heterogeneous splenic lesion with hemoperitoneum,raising clinical suspicion of SSR.Emergency laparotomy revealed a pancreatic tumor invading the spleen and left kidney,with associated splenic rupture and dense adhesions,necessitating en bloc resection of the distal pancreas,spleen,and left kidney.Histopathology revealed a biphasic malignancy composed of moderately differentiated pancreatic ductal adenocarcinoma and an undifferentiated carcinoma with rhabdoid morphology and loss of SMARCB1 expression.Immunohistochemical analysis confirmed complete loss of SMARCB1/INI1 in the undifferentiated component,along with a high Ki-67 index(approximately 80%)and CD10 positivity.The ductal adenocarcinoma component retained SMARCB1/INI1 expression and was positive for CK7 and CK-pan.Transitional zones between the two tumor components suggested progressive dedifferentiation and underlying genomic instability.The patient received adjuvant chemotherapy with gemcitabine and nab-paclitaxel and maintained a satisfactory quality of life at the 6-month follow-up.CONCLUSION This study reports a rare case of SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma of the pancreas combined with ductal adenocarcinoma,presenting as SSR-an exceptionally uncommon initial manifestation of pancreatic malignancy.展开更多
目的 基于国际癌症研究机构发布的GLOBOCAN 2018、2020及2022版本,系统分析2018–2022年间全球及中国胰腺癌的发病和死亡情况,并总结其主要影响因素,为我国胰腺癌的防控策略制定与临床诊疗实践提供参考。方法 收集并整理GLOBOCAN数据库...目的 基于国际癌症研究机构发布的GLOBOCAN 2018、2020及2022版本,系统分析2018–2022年间全球及中国胰腺癌的发病和死亡情况,并总结其主要影响因素,为我国胰腺癌的防控策略制定与临床诊疗实践提供参考。方法 收集并整理GLOBOCAN数据库中胰腺癌的发病例数、死亡例数,以及粗发病率、粗死亡率、世界年龄标准化发病率(age-standardized incidence rate by world standard population,ASIRW)、世界年龄标准化死亡率(age-standardized mortality rate by world standard population,ASMRW)等指标;同时结合人类发展指数(human development index,HDI)、国家收入水平等社会经济学参数,对全球与中国胰腺癌在不同地区、不同年龄与不同性别中的分布特点进行比较分析。结果 从2018年至2022年,全球胰腺癌新发病例数由45.8万增至51.1万,粗发病率从5.4/10万上升至6.5/10万;死亡例数从43.2万上升至46.7万,粗死亡率从5.7/10万增至5.9/10万,ASMRW从4.4/10万下降至4.3/10万。中国胰腺癌新发病例数从11.6万增至11.9万,占全球的23.3%,粗发病率维持在(8~9)/10万;死亡例数从11.0万下降至10.6万,粗死亡率从7.8/10万降至7.5/10万,ASMRW从4.9/10万降至3.9/10万。2022年非常高HDI国家的胰腺癌ASIRW为7.9/10万,ASMRW为6.9/10万,远高于低HDI国家的1.4/10万、1.3/10万。胰腺癌发病呈明显年龄相关性,≥75岁组全球新发191 157例(粗发病率为63.3/10万),中国新发37 722例(粗发病率为51.2/10万);全球和中国的男性发病率和死亡率均高于女性。结论 胰腺癌正成为全球和中国重要的公共卫生挑战,其发病与死亡在未来仍可能继续攀升。应进一步加强控烟、肥胖管理、糖尿病监测等综合防控措施;针对高危人群开展早期筛查及规范化诊治对于提升胰腺癌生存率至关重要。完善全国肿瘤登记体系,并结合多学科协作模式,可为精准防治胰腺癌奠定坚实基础。展开更多
文摘目的探讨胰腺导管内管状乳头状肿瘤(intraductal tubulopapillary neoplasm of the pancreas,ITPN)伴相关浸润性癌的病理形态、免疫表型、基因特征及鉴别诊断。方法回顾性分析江苏省人民医院病理学部2015~2022年确诊的5例ITPN伴有相关浸润性癌,总结其临床及影像学资料、组织病理学、特殊染色、免疫组化特征及分子遗传学改变。结果5例患者中男性3例,女性2例;平均年龄63.2岁;临床症状表现为不同程度的上腹部疼痛、黄疸等。影像提示肿瘤位于胰头部(3例)、壶腹部周围(1例)及全胰(1例),均显示管内实性生长,伴有胰管不同程度扩张;组织学显示肿瘤呈管状生长模式,表现为背靠背密集的腺管结构,缺乏或少见乳头状结构,某些区域呈实性片状生长,巢团中央常见坏死;瘤细胞立方形,异型性明显,核分裂象易见,罕见黏液分化;本组5例均伴有相关浸润性癌,4例为导管腺癌,1例伴有罕见的肉瘤样癌成分。弹力纤维染色示明确浸润区域周围弹力纤维完全缺失;轮廓尚清区域周围弹力纤维部分存在。免疫组织化学显示肿瘤细胞不同程度表达CK7、CK19、MUC1、MUC6,不表达MUC5AC、MUC2、CgA、Syn、胰蛋白酶、糜蛋白酶、脂肪酶、BCL10。我们选取其中3例(1例T1a期,1例T1b期,1例T2期)行KRAS、TP53、FGFR2、HER2及PIK3CA基因检测,结果显示其中1例TP53基因8号外显子框内插入突变,随后对该病例行NGS检测,显示其还存在BRAF、BAP1、FOXO1、PIK3CG等多个基因突变。随访2例死亡,3例尚生存。结论ITPN是一种相对少见的胰腺导管内肿瘤亚型,可伴有相关浸润性癌成分。其具有独特的影像学表现及病理特征,特征性分子遗传学的改变可有望为肿瘤的治疗提供潜在靶点。
文摘Radiofrequency ablation(RFA),particularly endoscopic ultrasound-guided RFA(EUS-RFA),has emerged as a promising minimally invasive approach for the treatment of pancreatic cancer,especially in patients with locally advanced or unresectable disease.This review outlines recent technological developments in EUS-RFA,including innovations in energy delivery systems,probe design,and real-time thermal monitoring,which have improved the precision and safety of the procedure.Clinical studies combining EUS-RFA with chemotherapy have demonstrated encouraging outcomes,with improvements in overall survival,progression-free survival,tumor necrosis,and symptom control compared to chemotherapy alone.Additionally,RFA-induced tumor antigen release and modulation of the tumor microenvironment suggest a potential synergistic role with immunotherapy.Despite its promise,the widespread adoption of EUS-RFA is limited by a lack of large-scale randomized controlled trials and standardized treatment protocols.
文摘BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a highly aggressive tumor,and spontaneous splenic rupture(SSR)as its presenting manifestation is rarely reported among pancreatic malignancies.CASE SUMMARY We herein report a rare case of a 59-year-old female who presented with acute left upper quadrant abdominal pain without any history of trauma.Abdominal imaging demonstrated a heterogeneous splenic lesion with hemoperitoneum,raising clinical suspicion of SSR.Emergency laparotomy revealed a pancreatic tumor invading the spleen and left kidney,with associated splenic rupture and dense adhesions,necessitating en bloc resection of the distal pancreas,spleen,and left kidney.Histopathology revealed a biphasic malignancy composed of moderately differentiated pancreatic ductal adenocarcinoma and an undifferentiated carcinoma with rhabdoid morphology and loss of SMARCB1 expression.Immunohistochemical analysis confirmed complete loss of SMARCB1/INI1 in the undifferentiated component,along with a high Ki-67 index(approximately 80%)and CD10 positivity.The ductal adenocarcinoma component retained SMARCB1/INI1 expression and was positive for CK7 and CK-pan.Transitional zones between the two tumor components suggested progressive dedifferentiation and underlying genomic instability.The patient received adjuvant chemotherapy with gemcitabine and nab-paclitaxel and maintained a satisfactory quality of life at the 6-month follow-up.CONCLUSION This study reports a rare case of SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma of the pancreas combined with ductal adenocarcinoma,presenting as SSR-an exceptionally uncommon initial manifestation of pancreatic malignancy.
文摘目的 基于国际癌症研究机构发布的GLOBOCAN 2018、2020及2022版本,系统分析2018–2022年间全球及中国胰腺癌的发病和死亡情况,并总结其主要影响因素,为我国胰腺癌的防控策略制定与临床诊疗实践提供参考。方法 收集并整理GLOBOCAN数据库中胰腺癌的发病例数、死亡例数,以及粗发病率、粗死亡率、世界年龄标准化发病率(age-standardized incidence rate by world standard population,ASIRW)、世界年龄标准化死亡率(age-standardized mortality rate by world standard population,ASMRW)等指标;同时结合人类发展指数(human development index,HDI)、国家收入水平等社会经济学参数,对全球与中国胰腺癌在不同地区、不同年龄与不同性别中的分布特点进行比较分析。结果 从2018年至2022年,全球胰腺癌新发病例数由45.8万增至51.1万,粗发病率从5.4/10万上升至6.5/10万;死亡例数从43.2万上升至46.7万,粗死亡率从5.7/10万增至5.9/10万,ASMRW从4.4/10万下降至4.3/10万。中国胰腺癌新发病例数从11.6万增至11.9万,占全球的23.3%,粗发病率维持在(8~9)/10万;死亡例数从11.0万下降至10.6万,粗死亡率从7.8/10万降至7.5/10万,ASMRW从4.9/10万降至3.9/10万。2022年非常高HDI国家的胰腺癌ASIRW为7.9/10万,ASMRW为6.9/10万,远高于低HDI国家的1.4/10万、1.3/10万。胰腺癌发病呈明显年龄相关性,≥75岁组全球新发191 157例(粗发病率为63.3/10万),中国新发37 722例(粗发病率为51.2/10万);全球和中国的男性发病率和死亡率均高于女性。结论 胰腺癌正成为全球和中国重要的公共卫生挑战,其发病与死亡在未来仍可能继续攀升。应进一步加强控烟、肥胖管理、糖尿病监测等综合防控措施;针对高危人群开展早期筛查及规范化诊治对于提升胰腺癌生存率至关重要。完善全国肿瘤登记体系,并结合多学科协作模式,可为精准防治胰腺癌奠定坚实基础。
