Abetalipoproteinemia (ABL; OMIM 200100) and homozygous hypobetalipoproteinemia (HHBL; OMIM 107730) are rare diseases characterized by hypocholesterolemia and malabsorption of lipid-soluble vitamins leading to retinal degeneration, neuropathy and coagulopathy. Hepatic steatosis is also common. The root cause of both disorders is improper packaging and secretion of apolipoprotein (apo) B-containing lipoprotein particles due to mutations either in both alleles of the MTP (alias MTTP) gene encoding microsomal triglyceride transfer protein (MTP) or both alleles of the APOB gene itself in the case of ABL and HHBL, respectively. Clinical diagnosis is based on signs and symptoms, acanthocytosis on blood smear, and virtually absent apo B-containing lipoproteins, including chylomicrons, very low density lipoprotein and low density lipoprotein. Obligate heterozygote parents of ABL patients usually have normal lipids consistent with autosomal recessive inheritance, while heterozygous parents of HHBL patients typically have half normal levels of apo B-containing lipoproteins consistent with autosomal co-dominant inheritance. Definitive diagnosis involves sequencing the MTP and APOB genes, for which >30 and >60 mutations have been described for ABL and HHBL, respectively. Follow-up includes monitoring for ophthalmologic, neurologic, hematologic, and hepatic complications, as well as compliance with treatment. Investigations include lipid profile, serum transaminases, markers for lipid-soluble vitamins, and periodic instrumental assessment of ocular and neurological function. Mainstays of treatment include adherence to a low-fat diet, and supplementation with essential fatty acids and high oral doses of fat soluble vitamins. Prognosis is variable, but early diagnosis and strict adherence to treatment can recover normal neurological function and halt disease progression.

PURPOSE It is unclear whether laparoscopic distal gastrectomy for locally advanced gastric cancer is oncologically equivalent to open distal gastrectomy. The noninferiority of laparoscopic subtotal gastrectomy with D2 lymphadenectomy for locally advanced gastric cancer compared with open surgery in terms of 3-year relapse-free survival rate was evaluated. PATIENTS AND METHODS A phase III, open-label, randomized controlled trial was conducted for patients with histologically proven locally advanced gastric adenocarcinoma suitable for distal subtotal gastrectomy. The primary end point was the 3-year relapse-free survival rate; the upper limit of the hazard ratio (HR) for noninferiority was 1.43 between the laparoscopic and open distal gastrectomy groups. RESULTS From November 2011 to April 2015, 1,050 patients were randomly assigned to laparoscopy (n = 524) or open surgery (n = 526). After exclusions, 492 patients underwent laparoscopic surgery and 482 underwent open surgery and were included in the analysis. The laparoscopy group, compared with the open surgery group, suffered fewer early complications (15.7% v 23.4%, respectively; P = .0027) and late complications (4.7% v 9.5%, respectively; P = .0038), particularly intestinal obstruction (2.0% v 4.4%, respectively; P = .0447). The 3-year relapse-free survival rate was 80.3% (95% CI, 76.0% to 85.0%) for the laparoscopy group and 81.3% (95% CI, 77.0% to 85.0%; log-rank P = .726) for the open group. Cox regression analysis after stratification by the surgeon revealed an HR of 1.035 (95% CI, 0.762 to 1.406; log-rank P = .827; P for noninferiority = .039). When stratified by pathologic stage, the HR was 1.020 (95% CI, 0.751 to 1.385; log-rank P = .900; P for noninferiority = .030). CONCLUSION Laparoscopic distal gastrectomy with D2 lymphadenectomy was comparable to open surgery in terms of relapse-free survival for patients with locally advanced gastric cancer. Laparoscopic distal gastrectomy with D2 lymphadenectomy could be a potential standard treatment option for locally advanced gastric cancer.

Abstract:Background. Although the results of gastric cancer treatment have markedly improved, this disease remains the most common cause of cancer death in Korea. Methods. Clinicopathologic characteristics were analyzed for 10 783 consecutive patients who underwent operation for gastric cancer at the Department of Surgery, Seoul National University Hospital, from 1970 to 1996. We also evaluated survival and prognostic factors for 9262 consecutive patients operated from 1981 to 1996. The clinicopathologic variables for evaluating prognostic values were classified as patient-, tumor-, and treatment-related factors. The prognostic significance of treatment modality [surgery alone, surgery ؉ chemotherapy, surgery ؉ immunotherapy ؉ chemotherapy (immunochemosurgery)] was evaluated in patients with stage III gastric cancer (according to the International Union Against Cancer TNM classification of 1987). For the assessment of lymph node metastasis, both the number of involved lymph nodes and the ratio of involved to resected lymph nodes were analyzed, as a quantitative system. Results. The mean age of the 10 783 patients was 53.5 years and the male-to-female ratio was 2.07 : 1. Resection was performed in 9058 patients (84.0% resection rate). The 5-year survival rates were 55.9% for all patients and 64.8% for patients who received curative resection. Age, sex, preoperative hemoglobin and albumin levels, type of operation, curability of operation, tumor location, Borrmann type, tumor size, histologic differentiation, Lauren's classification, perineural invasion, lymphatic invasion, vascular invasion, depth of invasion, number of involved lymph nodes, ratio of involved to resected lymph nodes, and distant metastasis had prognostic significance on univariate analysis. Radical lymph node dissection, with more than 25 resected lymph nodes improved survival in patients with stage II and IIIa disease. As postoperative adjuvant therapy, immunochemotherapy was most effective in patients with stage III disease. Patients with identical numbers of lymph nodes -either the number of involved lymph nodes or the number of resected lymph nodes-were divided according to their ratios of involved-to-resected lymph nodes. In each numeric group, there were significant survival differences according to the ratio of involved-toresected lymph nodes. However, patients who had the same involved-to-resected lymph node ratio did not show significant differences in survival rate according to either the number of involved or the number of resected lymph nodes. On multivariate analysis, curability of operation, depth of invasion, and ratio of involved to resected lymph nodes were independent significant prognostic factors. Conclusions. Curative resection, depth of invasion, and lymph node metastasis were the most significant prognostic factors in gastric cancer. With regard to the status of lymph node metastasis, the ratio of involved to resected lymph nodes had a more precise and comprehensive prognostic value than only the number of involved or resec...

Our data suggest that LADG for EGC is feasible and safe. We expect the results of the present study to be confirmed by prospective randomized analysis.

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