肺癌神经内分泌分化与术后生存关系探讨(英文)

目的探讨非小细胞肺癌神经内分泌(NSCLC-NE)分化与患者手术后生存关系。方法收集1997年4月～1999年4月98例肺癌手术切除病理标本,采用免疫组化标记特异性烯醇化酶(NSE)及突触素(SY),并按强弱区分为“+、++、+++”。对同一手术病例标本采用电镜观察特异性 NE 颗粒。术后病例随访36例,最长60月。采用Cox 多因素风险模型分析 NSCLC-NE 分化与患者术后生存的关系。结果 91例为非小细胞肺癌。非小细胞肺癌 NE 阳性表达率为63.7%(58/91),其中 NSE 阳性表达54例(59.3%),SY 阳性表达22例(24.1%),电镜观察 NE 特异性颗粒30例(33.0%)。结合免疫组化和电镜观察 NSCLC-NE 分化44例(48.4%)。Cox 模型多因素分析结果表明 NSCLC-NE 分化者术后生存时间明显缩短(P=0.048)。术后生存与肺癌细胞分化程度(P=0.006)、病理分期(P=0.001)、NE 表达强弱(P=0.054)有密切关系。结论 NSCLC-NE 分化与肿瘤细胞分化和患者术后生存有关。采用 NE 标志特标记肿瘤,并观察其强弱改变,对术后评估具有较重要的参考意义,可作为临床判断患者预后指标之一。     

Intrathoracic caudate lobe of the liver:A case report and literature review

Heterotopic supradiaphragmatic livers are rare.A total of 23 cases of primary supradiaphragmatic livers have been reported in the literature.The clinical presentations of heterotopic supradiaphragmatic liver are variable.The simultaneous detection of intrathoracic accessory liver and pulmonary sequestration is extremely rare,and only one case has previously been reported.It is difficult to make a correct diagnosis preoperatively.We presented a 53-year-old woman with complaints of an intermittent,productive cough and dyspnea for two months that was refractory to medical treatment.She had no previous history of trauma or surgery.A chest radiograph only showed a widening of the mediastinum.Contrast-enhanced computed tomography of the chest revealed a well-circumscribed homogenous soft-tissue mass,approximately 4.35 cm×2.5 cm×6.14 cm in size,protruding through the right diaphragmatic crura to the right pleural cavity,attached to the inferior vena cava,esophagus and liver.There was no conclusive diagnosis before surgery.After the operation,we discovered that this patient was the first case of a supradiaphragmatic heterotopic liver,which passed through the inferior vena cava foramen and was coincidentally combined with an intralobar pulmonary sequestration that was found intraoperatively.We discussed its successful management with surgical resection via a thoracic approach and reviewed the published literature.     

Allogeneic stem cell transplantation in AML with t(6;9)(p23;q34);DEK-NUP214 shows a favourable outcome when performed in first complete remission

Acute myeloid leukaemia (AML) with t(6;9)(p23;q34) is a poor-risk entity, commonly associated with FLT3-ITD (internal tandem duplication). Allogeneic stem-cell tranplantation (allo-SCT) is recommended, although studies analysing the outcome of allo-SCT in this setting are lacking. We selected 195 patients with t(6;9) AML, who received a first allo-SCT between 2000 and 2016 from the EBMT (European Society for Blood and Marrow Transplantation) registry. Disease status at time of allo-SCT was the strongest independent prognostic factor, with a two-year leukaemia-free survival and relapse incidence of 57% and 19% in patients in CR1 (first complete remission), 34% and 33% in CR2 (second complete remission), and 24% and 49% in patients not in remission, respectively (P < 0·001). This study, which represents the largest one available in t(6;9) AML, supports the recommendation to submit these patients to allo-SCT in CR1.     

Little girl who conquered the "ALPPS’’

An insufficient future liver remnant(FLR)is associated with post-hepatectomy liver failure.Associating liver partition and portal vein ligation for stage hepatectomy(ALPPS)has been shown to be effective for the induction of rapid FLR hypertrophy so as to improve the resectability in patients with insufficient FLR.We hereby report our experience of this novel approach for a 6-year-old patient with hepatoblastoma.Computed tomography showed a hepatoblastoma measuring12.5 cm×9.9 cm×11.7 cm in the right liver(Couinaud segmentⅣ,ⅤandⅧ).Volumetric assessment of the FLR i.e.,left lateral section was 112.6 mL i.e.,21.2%of the estimated total liver volume.In view of the small-for-size FLR,ALPPS was contemplated.An anterior approach was adopted for the in-situ parenchymal split without mobilisation of the right liver.FLR volumetry on the seventh postoperative day was 160.7 mL,which represented a 46.1%gain in volume,and a FLR/ESLV ratio of 30.2%.A right trisectionectomy was performed on the eighth postoperative day.Postoperative recovery was uneventful.Patient was discharged on day 16 after the first operation.To our knowledge,this was the first report that showed the applicability of ALPPS to a paediatric patient.     

Long-term outcome of allogeneic PBSC transplantation in pediatric patients with hematological malignancies: a report of the Spanish Working Party for Blood and Marrow Transplantation in Children (GETMON) and the Spanish Group for Allogeneic Peripheral Blood Transplantation (GETH)

We analyzed the clinical outcome in 90 children undergoing allogeneic PBSC transplantation from HLA-identical relative for leukemia. GvHD prophylaxis was CsA+ methotrexate in 50 and CsA+/-steroids in 40. Median CD34+ cells infused were 6 x 10(6)/kg (range, 1.4-32). Median follow-up was 60 months (range, 6-115). CI of transplant-related mortality (TRM) was 18.4+/-4%. On multivariate analysis, high Lansky score (>80) at transplantation was associated with lower TRM (HR, 0.9; P<0.0002). Relapse incidence (RI) was 33.6+/-6%. On multivariate analysis, high Lansky score at transplantation and cGvHD were associated with lower RI (HR, 0.04; P<0.0005 and HR, 0.23; P<0.03, respectively). Disease-free survival (DFS) was 57.8+/-5%. Disease status at transplantation (HR, 0.33; P<0.02), steroid treatment at day +90 (HR, 5.61; P<0.005) and cGvHD (HR, 0.23; P<0.005) had a significant impact on DFS in multivariate analysis. CI of cGvHD was 63.7+/-7%. Patients with cGvHD had better DFS (65+/-5%) because of lower RI (15.7+/-6%) and similar TRM (27.4+/-4%). These data suggest acceptable long-term outcomes after allogeneic PBSC transplantation in children despite the high incidence of cGvHD. These patients had a lower risk of relapse and a better DFS.     

Risk assessment and outcome of chronic graft-versus-host disease after allogeneic peripheral blood progenitor cell transplantation in pediatric patients

We retrospectively evaluated the incidence, risk factors for chronic graft-versus-host disease (cGvHD) and outcome in 80 pediatric patients (36 male) (median age 13 years) who underwent allogeneic peripheral blood progenitor cell transplantation. Patients were grafted from an HLA-identical sibling after myeloablative conditioning (total body irradiation (TBI) based 52; non-TBI 28). GvHD prophylaxis used were: cyclosporin A (CsA)+ short methotrexate (MTX) in 52 and CsA+/-prednisone in 28. The median number of CD34+ cells infused were 5.8 x 10(6)/kg (range: 1.4-32.8). The median follow-up was 24 months (range: 3-94). In all, 28 patients had cGvHD (confidence interval (CI): 54.2+/-10%). Factors that were significant on univariate analysis were diagnosis (P=0.03) and GvHD prophylaxis administered (P=0.04). On multivariate analysis, only GvHD prophylaxis used was associated with a significant risk of cGvHD (hazard ratio (HR): 3.94; 95% CI: 1.41-10.91, P=0.009). The CI of cGvHD for patients receiving CsA+MTX was 40.9+/-12 vs 76.5+/-18% for patients who did not (P=0.03). The probability of relapse was 36+/-6% for all patients (12.5+/-8% for patients with cGvHD vs 47.9+/-8% without cGvHD). The probability of disease-free survival was better for patients with cGvHD (69.9+/-10 vs 37.9+/-7%; HR: 3.59, 95% CI: 1.47-5.56; P=0.001). Our data suggest that the GvHD prophylaxis used is the most relevant predictor of cGvHD. Patients with cGvHD had a lower risk of relapse and a better survival.