AIM To investigate the feasibility and safety of secondary endoscopic submucosal dissection(ESD) for residual or locally recurrent gastric tumors. METHODS Between 2010 and 2017, 1623 consecutive patients underwent ESD...AIM To investigate the feasibility and safety of secondary endoscopic submucosal dissection(ESD) for residual or locally recurrent gastric tumors. METHODS Between 2010 and 2017, 1623 consecutive patients underwent ESD for gastric neoplasms at a single tertiary referral center. Among these, 28 patients underwent secondary ESD for a residual or locally recurrent tumor. Our analysis compared clinicopathologic factors between primary ESD and secondary ESD groups. RESULTS The en bloc resection and curative rate of resection of secondary ESD were 92.9% and 89.3%, respectively. The average procedure time of secondary ESD was significantly longer than primary ESD(78.2 min vs 55.1 min, P = 0.004), and the adverse events rate was not significantly different but trended slightly higher in the secondary ESD group compared to the primary ESD group(10.7% vs 3.8%, P = 0.095). Patients who received secondary ESD had favorable outcomes without severe adverse events. During a mean follow-up period, no local recurrence occurred in patients who received secondary ESD. CONCLUSION Secondary ESD of residual or locally recurrent gastric tumors appears to be a feasible and curative treatment though it requires greater technical efficiency and longer procedure time.展开更多
Over the last two decades multiple studies have demonstrated an increased incidence of additional malignancies in patients with intraductal papillary mucinous neoplasms(IPMNs).Additional malignancies have been identif...Over the last two decades multiple studies have demonstrated an increased incidence of additional malignancies in patients with intraductal papillary mucinous neoplasms(IPMNs).Additional malignancies have been identified in 10%-52% of patients with IPMNs.The majority of these additional cancers occur before or concurrent with the diagnosis of IPMN.The gastrointestinal tract is most commonly involved in secondary malignancies,with benign colon polyps and colon cancer commonly seen in western countries and gastric cancer commonly seen in Asian countries.Other extrapancreatic malignancies associated with IPMNs include benign and malignant esophageal neoplasms,gastrointestinal stromal tumors,carcinoid tumors,hepatobiliary cancers,breast cancers,prostate cancers,and lung cancers.There is no clear etiology for the development of secondary malignancies in patients with IPMN.Although population-based studies have shown different results from single institution studies regarding the exact incidence of additional primary cancers in IPMN patients,both have reached the same conclusion:there is a higher incidence of extrapancreatic malignancies in patients with IPMNs than in the general population.This f inding has signif icant clinical implications for both the initial evaluation and the subsequent long-term followup of patients with IPMNs.If a patient has not had recent colonoscopy,this should be performed during the evaluation of a newly diagnosed IPMN.Upper endoscopy should be performed in patients from Asian countries or for those who present with symptoms suggestive of upper gastrointestinal disease.Routine screening studies(breast and prostate) should be carried out as currently recommended for patient's age both before and after the diagnosis of IPMN.展开更多
The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML fro...The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML from the myeloproliferative disorders essential thrombocythemia(ET),polycythemia vera(PV)and chronic megakaryocytic granulocytic myeloproliferation(CMGM).The 2006-2008 European Clinical Molecular and Pathological(ECMP)criteria discovered 3variants of thrombocythemia:ET with features of PV(prodromal PV),"true"ET and ET associated with CMGM.The 2008 World Health Organization(WHO)-ECMP and 2014 WHO-CMP classifications defined three phenotypes of JAK2^(V617F)mutated ET:normocellular ET(WHO-ET),hypercelluar ET due to increased erythropoiesis(prodromal PV)and ET with hypercellular megakaryocytic-granulocytic myeloproliferation.The JAK2^(V617F)mutation load in heterozygous WHO-ET is low and associated with normal life expectance.The hetero/homozygous JAK2^(V617F)mutation load in PV and myelofibrosis is related to myeloproliferative neoplasm(MPN)disease burden in terms of symptomaticsplenomegaly,constitutional symptoms,bone marrow hypercellularity and myelofibrosis.JAK2 exon 12mutated MPN presents as idiopathic eryhrocythemia and early stage PV.According to 2014 WHO-CMP criteria JAK2 wild type MPL^(515)mutated ET is the second distinct thrombocythemia featured by clustered giant megakaryocytes with hyperlobulated stag-horn-like nuclei,in a normocellular bone marrow consistent with the diagnosis of"true"ET.JAK2/MPL wild type,calreticulin mutated hypercellular ET appears to be the third distinct thrombocythemia characterized by clustered larged immature dysmorphic megakaryocytes and bulky(bulbous)hyperchromatic nuclei consistent with CMGM or primary megakaryocytic granulocytic myeloproliferation.展开更多
The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced p...The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced primary myelofibrosis(MF). Essential thrombocythemia(ET) according to PVSG and 2007/2008 WHO criteria comprises three variants of JAK2V617 F mutated ET when the ECMP criteria are applied. These include normocellular ET,hypercellular ET with features of early PV(prodromal PV),and hypercellular ET due to megakaryocytic,granulocytic myeloprolifera-tion(ET.MGM). Evolution of prodromal PV into overt PV is common. Development of MF is rare in normocellular ET(WHO-ET) but rather common in hypercellular ET.MGM. The JAK2V617 F mutation burden in heterozygous mutated normocellular ET and in heterozygous/homozygous or homozygous mutated PV and ET.MGM is of major prognostic significance. JAK2/MPL wild type ET associated with prefibrotic primary megakaryocytic and granulocytic myeloproliferation(PMGM) is characterized by densely clustered immature dysmorphic megakaryocytes with bulky(bulbous) hyperchromatic nuclei,which are never seen in JAK2V617 F mutated ET,and PV and also not in MPL515 mutated normocellular ET(WHO-ET). JAK2V617 mutation burden,spleen size,LDH,circulating CD34+ cells,and pre-treatment bone marrow histopathology are mandatory to stage the myeloproliferative neoplasms ET,PV,PMGM for proper prognosis assessment and therapeutic implications. MF itself is not a disease because reticulin fibrosis and reticulin/collagen fibrosis are secondary responses of activated polyclonal fibroblasts to cytokines released from the clonal myeloproliferative granulocytic and megakaryocytic progenitor cells in ET.MGM,PV and PMGM.展开更多
Angiosarcoma of bone is an exceedingly rare primary bone malignancy that can present as an aggressive osteolytic lesion. This subset can radiologically mimic non-vascular neoplasms and impose serious challenges in rea...Angiosarcoma of bone is an exceedingly rare primary bone malignancy that can present as an aggressive osteolytic lesion. This subset can radiologically mimic non-vascular neoplasms and impose serious challenges in reaching the correct diagnosis. Meanwhile histological diagnosis can be extremely challenging too, as the pathological features often resemble that of aneurysmal bone cysts. We present an unusual case of a 22-year-old woman who presented with a rapidly growing humeral tumor of 8 months’ duration. The case of intraosseous angiosarcoma presented as a diagnostic dilemma and the relevant radiological and pathologic findings were discussed. We describe the clinical, radiological and pathological features of this unique case, and review the literature concerning Angiosarcoma of bone. Our case highlights the diagnostic difficulties for such very rare tumours and clinico-pathological correlation is of paramount importance to differential diagnosis.展开更多
<strong>Introduction:</strong> Conjunctival-corneal or choroidal calcifications are frequent in SHPT, blindness is however exceptional. We report a case of blindness secondary to compressive ischemic optic...<strong>Introduction:</strong> Conjunctival-corneal or choroidal calcifications are frequent in SHPT, blindness is however exceptional. We report a case of blindness secondary to compressive ischemic optic neuropathy. <strong>Case Report:</strong> Mr. B.E.K., 49 years old, has a chronic renal failure secondary to unlabeled glomerular nephropathy for 17 years. He has been on chronic hemodialysis for 12 years and has had SHPT for nine years. He secondarily developed disabling segmental osteoarticular deformities associated with kyphoscoliosis, “drumstick” fingers and facial dysmorphism. Five months before admission he developed eye pain and reduced visual acuity progressing within one month to blindness. Biology noted: serum creatinine at 726 umol/l (60 - 120 umol/L), azotemia at 14.3 mmol/l (2.5 - 7.5 mmol/L), serum calcium at 2.25 (2.25 - 2.55 mmol/L), phosphatemia at 1.13 (0.8 - 1.35 mmol/L), alkaline phosphatases at 2196 (5 - 270 IU/L) and parathyroid hormone level at 2257 (10 - 60 pg/mL). Retinal angiography revealed lesions suggestive of ischemic neuropathy. The orbit CT scan with 3D coronal reconstruction revealed narrowing of the caliber of the optical channels with dystrophic thickening of the skull base and cranial vault. Cranioencephalic and orbital MRI revealed diffuse brown tumors and pre-chiasmatic optic atrophy. <strong>Discussion:</strong> The most frequent ocular complications of SHPT are conjunctival-corneal or sclero-choroidal calcifications, asymptomatic, associated with hypercalcemia. Compressive manifestations are rarer, represented mainly by an amputation of the visual field, diplopia, ptosis or blindness, as described in our patient. The main cause is osteodystrophy and brown tumors of the skull base (1% - 2%). <strong>Conclusion:</strong> This case report underlines the importance of early detection of SHPT, in order to avoid its major complications, such as blindness, especially since current preventive and curative measures have proven their effectiveness.展开更多
目的了解骨质疏松性椎体压缩骨折二级预防失败患者骨代谢指标及骨密度的特点。方法选择2015年1月至2020年1月在我院骨科因骨质疏松性椎体压缩骨折行2次或2次以上手术治疗的患者为观察组研究对象,共65例,同时选择同时期行手术治疗的非多...目的了解骨质疏松性椎体压缩骨折二级预防失败患者骨代谢指标及骨密度的特点。方法选择2015年1月至2020年1月在我院骨科因骨质疏松性椎体压缩骨折行2次或2次以上手术治疗的患者为观察组研究对象,共65例,同时选择同时期行手术治疗的非多次脊柱压缩骨折患者219例作为配对资料对照组,回顾性分析这284例患者临床资料,记录其术前骨代谢标志物水平及骨密度(bone mineral density,BMD)结果并进行两组患者间比较。结果观察组患者骨代谢标志物中人血清和血浆中的总I型前胶原氨基端肽(tP1NP)、N⁃MID骨钙素(OCN)以及血清25⁃羟维生素D(25⁃hydroxy vitamin D,25OHD)水平与对照组相比差异无统计学意义(P>0.05),骨代谢标志物I型胶原羧基端肽β特殊序列(β⁃CTX)水平明显高于对照组(P<0.05)。两组患者髋部BMD值、股骨颈BMD值差异无统计学意义(P>0.05),观察组腰椎BMD值明显低于对照组(P<0.05)。结论骨质疏松性椎体压缩骨折二级预防失败患者具有更为活跃的骨吸收状态及更低的腰椎骨密度。展开更多
基金Supported by Basic Science Research Program through the National Research Foundation of Korea(NRF)funded by the Ministry of Science and ICT(NRF-2015R1C1A1A01054352)
文摘AIM To investigate the feasibility and safety of secondary endoscopic submucosal dissection(ESD) for residual or locally recurrent gastric tumors. METHODS Between 2010 and 2017, 1623 consecutive patients underwent ESD for gastric neoplasms at a single tertiary referral center. Among these, 28 patients underwent secondary ESD for a residual or locally recurrent tumor. Our analysis compared clinicopathologic factors between primary ESD and secondary ESD groups. RESULTS The en bloc resection and curative rate of resection of secondary ESD were 92.9% and 89.3%, respectively. The average procedure time of secondary ESD was significantly longer than primary ESD(78.2 min vs 55.1 min, P = 0.004), and the adverse events rate was not significantly different but trended slightly higher in the secondary ESD group compared to the primary ESD group(10.7% vs 3.8%, P = 0.095). Patients who received secondary ESD had favorable outcomes without severe adverse events. During a mean follow-up period, no local recurrence occurred in patients who received secondary ESD. CONCLUSION Secondary ESD of residual or locally recurrent gastric tumors appears to be a feasible and curative treatment though it requires greater technical efficiency and longer procedure time.
文摘Over the last two decades multiple studies have demonstrated an increased incidence of additional malignancies in patients with intraductal papillary mucinous neoplasms(IPMNs).Additional malignancies have been identified in 10%-52% of patients with IPMNs.The majority of these additional cancers occur before or concurrent with the diagnosis of IPMN.The gastrointestinal tract is most commonly involved in secondary malignancies,with benign colon polyps and colon cancer commonly seen in western countries and gastric cancer commonly seen in Asian countries.Other extrapancreatic malignancies associated with IPMNs include benign and malignant esophageal neoplasms,gastrointestinal stromal tumors,carcinoid tumors,hepatobiliary cancers,breast cancers,prostate cancers,and lung cancers.There is no clear etiology for the development of secondary malignancies in patients with IPMN.Although population-based studies have shown different results from single institution studies regarding the exact incidence of additional primary cancers in IPMN patients,both have reached the same conclusion:there is a higher incidence of extrapancreatic malignancies in patients with IPMNs than in the general population.This f inding has signif icant clinical implications for both the initial evaluation and the subsequent long-term followup of patients with IPMNs.If a patient has not had recent colonoscopy,this should be performed during the evaluation of a newly diagnosed IPMN.Upper endoscopy should be performed in patients from Asian countries or for those who present with symptoms suggestive of upper gastrointestinal disease.Routine screening studies(breast and prostate) should be carried out as currently recommended for patient's age both before and after the diagnosis of IPMN.
文摘The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML from the myeloproliferative disorders essential thrombocythemia(ET),polycythemia vera(PV)and chronic megakaryocytic granulocytic myeloproliferation(CMGM).The 2006-2008 European Clinical Molecular and Pathological(ECMP)criteria discovered 3variants of thrombocythemia:ET with features of PV(prodromal PV),"true"ET and ET associated with CMGM.The 2008 World Health Organization(WHO)-ECMP and 2014 WHO-CMP classifications defined three phenotypes of JAK2^(V617F)mutated ET:normocellular ET(WHO-ET),hypercelluar ET due to increased erythropoiesis(prodromal PV)and ET with hypercellular megakaryocytic-granulocytic myeloproliferation.The JAK2^(V617F)mutation load in heterozygous WHO-ET is low and associated with normal life expectance.The hetero/homozygous JAK2^(V617F)mutation load in PV and myelofibrosis is related to myeloproliferative neoplasm(MPN)disease burden in terms of symptomaticsplenomegaly,constitutional symptoms,bone marrow hypercellularity and myelofibrosis.JAK2 exon 12mutated MPN presents as idiopathic eryhrocythemia and early stage PV.According to 2014 WHO-CMP criteria JAK2 wild type MPL^(515)mutated ET is the second distinct thrombocythemia featured by clustered giant megakaryocytes with hyperlobulated stag-horn-like nuclei,in a normocellular bone marrow consistent with the diagnosis of"true"ET.JAK2/MPL wild type,calreticulin mutated hypercellular ET appears to be the third distinct thrombocythemia characterized by clustered larged immature dysmorphic megakaryocytes and bulky(bulbous)hyperchromatic nuclei consistent with CMGM or primary megakaryocytic granulocytic myeloproliferation.
文摘The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced primary myelofibrosis(MF). Essential thrombocythemia(ET) according to PVSG and 2007/2008 WHO criteria comprises three variants of JAK2V617 F mutated ET when the ECMP criteria are applied. These include normocellular ET,hypercellular ET with features of early PV(prodromal PV),and hypercellular ET due to megakaryocytic,granulocytic myeloprolifera-tion(ET.MGM). Evolution of prodromal PV into overt PV is common. Development of MF is rare in normocellular ET(WHO-ET) but rather common in hypercellular ET.MGM. The JAK2V617 F mutation burden in heterozygous mutated normocellular ET and in heterozygous/homozygous or homozygous mutated PV and ET.MGM is of major prognostic significance. JAK2/MPL wild type ET associated with prefibrotic primary megakaryocytic and granulocytic myeloproliferation(PMGM) is characterized by densely clustered immature dysmorphic megakaryocytes with bulky(bulbous) hyperchromatic nuclei,which are never seen in JAK2V617 F mutated ET,and PV and also not in MPL515 mutated normocellular ET(WHO-ET). JAK2V617 mutation burden,spleen size,LDH,circulating CD34+ cells,and pre-treatment bone marrow histopathology are mandatory to stage the myeloproliferative neoplasms ET,PV,PMGM for proper prognosis assessment and therapeutic implications. MF itself is not a disease because reticulin fibrosis and reticulin/collagen fibrosis are secondary responses of activated polyclonal fibroblasts to cytokines released from the clonal myeloproliferative granulocytic and megakaryocytic progenitor cells in ET.MGM,PV and PMGM.
文摘Angiosarcoma of bone is an exceedingly rare primary bone malignancy that can present as an aggressive osteolytic lesion. This subset can radiologically mimic non-vascular neoplasms and impose serious challenges in reaching the correct diagnosis. Meanwhile histological diagnosis can be extremely challenging too, as the pathological features often resemble that of aneurysmal bone cysts. We present an unusual case of a 22-year-old woman who presented with a rapidly growing humeral tumor of 8 months’ duration. The case of intraosseous angiosarcoma presented as a diagnostic dilemma and the relevant radiological and pathologic findings were discussed. We describe the clinical, radiological and pathological features of this unique case, and review the literature concerning Angiosarcoma of bone. Our case highlights the diagnostic difficulties for such very rare tumours and clinico-pathological correlation is of paramount importance to differential diagnosis.
文摘<strong>Introduction:</strong> Conjunctival-corneal or choroidal calcifications are frequent in SHPT, blindness is however exceptional. We report a case of blindness secondary to compressive ischemic optic neuropathy. <strong>Case Report:</strong> Mr. B.E.K., 49 years old, has a chronic renal failure secondary to unlabeled glomerular nephropathy for 17 years. He has been on chronic hemodialysis for 12 years and has had SHPT for nine years. He secondarily developed disabling segmental osteoarticular deformities associated with kyphoscoliosis, “drumstick” fingers and facial dysmorphism. Five months before admission he developed eye pain and reduced visual acuity progressing within one month to blindness. Biology noted: serum creatinine at 726 umol/l (60 - 120 umol/L), azotemia at 14.3 mmol/l (2.5 - 7.5 mmol/L), serum calcium at 2.25 (2.25 - 2.55 mmol/L), phosphatemia at 1.13 (0.8 - 1.35 mmol/L), alkaline phosphatases at 2196 (5 - 270 IU/L) and parathyroid hormone level at 2257 (10 - 60 pg/mL). Retinal angiography revealed lesions suggestive of ischemic neuropathy. The orbit CT scan with 3D coronal reconstruction revealed narrowing of the caliber of the optical channels with dystrophic thickening of the skull base and cranial vault. Cranioencephalic and orbital MRI revealed diffuse brown tumors and pre-chiasmatic optic atrophy. <strong>Discussion:</strong> The most frequent ocular complications of SHPT are conjunctival-corneal or sclero-choroidal calcifications, asymptomatic, associated with hypercalcemia. Compressive manifestations are rarer, represented mainly by an amputation of the visual field, diplopia, ptosis or blindness, as described in our patient. The main cause is osteodystrophy and brown tumors of the skull base (1% - 2%). <strong>Conclusion:</strong> This case report underlines the importance of early detection of SHPT, in order to avoid its major complications, such as blindness, especially since current preventive and curative measures have proven their effectiveness.
文摘目的了解骨质疏松性椎体压缩骨折二级预防失败患者骨代谢指标及骨密度的特点。方法选择2015年1月至2020年1月在我院骨科因骨质疏松性椎体压缩骨折行2次或2次以上手术治疗的患者为观察组研究对象,共65例,同时选择同时期行手术治疗的非多次脊柱压缩骨折患者219例作为配对资料对照组,回顾性分析这284例患者临床资料,记录其术前骨代谢标志物水平及骨密度(bone mineral density,BMD)结果并进行两组患者间比较。结果观察组患者骨代谢标志物中人血清和血浆中的总I型前胶原氨基端肽(tP1NP)、N⁃MID骨钙素(OCN)以及血清25⁃羟维生素D(25⁃hydroxy vitamin D,25OHD)水平与对照组相比差异无统计学意义(P>0.05),骨代谢标志物I型胶原羧基端肽β特殊序列(β⁃CTX)水平明显高于对照组(P<0.05)。两组患者髋部BMD值、股骨颈BMD值差异无统计学意义(P>0.05),观察组腰椎BMD值明显低于对照组(P<0.05)。结论骨质疏松性椎体压缩骨折二级预防失败患者具有更为活跃的骨吸收状态及更低的腰椎骨密度。
