大宮 直木

BACKGROUND: Primary small intestinal lymphoma (PSIL) is a rare disease, and its clinical characteristics and prognostic factors remain insufficiently defined because of limited large-scale data. METHODS: We conducted a nationwide multicenter retrospective study of 686 patients diagnosed with PSIL between January 2008 and December 2017 at 44 institutions in Japan. Clinical characteristics, histological subtypes, and overall survival were analyzed. RESULTS: B-cell lymphoma was the predominant immunophenotype (n = 623, 91%), whereas T-cell lymphoma was uncommon (n = 50, 7%). Diffuse large B-cell lymphoma (DLBCL) was the most frequent histological subtype (n = 330, 48%), followed by follicular lymphoma (FL) (n = 224, 33%). Compared with other B-cell lymphomas, FL was more common in women and more frequently involved the jejunum (P < 0.001). The 5-year overall survival rate was significantly higher in patients with B-cell lymphoma than in those with T-cell lymphoma (81% vs. 23%, P < 0.001). Multivariate Cox regression analysis identified age > 60 years and T-cell lymphoma as independent adverse prognostic factors, whereas FL was an independent predictor of favorable overall survival. CONCLUSIONS: This nationwide multicenter analysis delineates the clinicopathological features and survival outcomes of PSIL in Japan. FL represents the second most common subtype after DLBCL and is associated with a favorable prognosis among PSIL subtypes.

BACKGROUND/AIM: Atezolizumab plus bevacizumab (Ate+Bev) is widely used as first-line therapy for unresectable hepatocellular carcinoma (HCC). However, a subset of patients experience early disease progression, often detected at the first radiologic assessment around 6 weeks. Evidence guiding second-line therapy in this subgroup is limited, and the clinical value of lenvatinib after early progressive disease (PD) remains unclear. PATIENTS AND METHODS: We retrospectively analyzed 36 patients with unresectable HCC who received lenvatinib after failure of first-line Ate+Bev. Patients were stratified by early PD, defined as radiologic progression at the scheduled 6-week assessment after starting Ate+Bev. Outcomes included antitumor response, progression-free survival (PFS), and overall survival (OS). RESULTS: Objective response rate (ORR) and disease control rate (DCR) assessed by RECIST 1.1 were comparable between patients with and without early PD (ORR: 28.6% vs. 13.8%; DCR: 85.7% vs. 86.2%; p=0.342). Median PFS was also similar between groups [5.2 months (95% confidence interval=1.9-NA) vs. 6.1 months (3.7-7.5); p=0.307]. In multivariate analyses adjusting for Child-Pugh class, Barcelona Clinic Liver Cancer (BCLC) stage, and reduced starting dose, early PD was not significantly associated with either PFS or OS, whereas Child-Pugh class A was independently associated with improved OS. Correlation between first- and second-line PFS was weak and non-significant (r=0.077, p=0.682). CONCLUSION: Lenvatinib demonstrated comparable antitumor activity and survival outcomes even in patients with early PD on first-line Ate+Bev, indicating that early radiologic progression does not necessarily signify refractoriness to subsequent systemic therapy. These findings support lenvatinib as a viable second-line option regardless of early Ate+Bev response, particularly in patients with preserved liver function. Larger prospective studies are needed to confirm these observations.

BACKGROUND: Owing to the rarity of metastatic tumors in the small bowel, their clinicopathological features, and prognostic factors remain poorly understood. This study aimed to clarify the clinicopathological features and factors associated with the prognosis of patients with small bowel metastasis from other organs in Japan. METHODS: We retrospectively examined 253 patients who were histopathologically diagnosed with small bowel metastases between January 2008 and December 2017 at multiple institutions in Japan. We identified the clinicopathological features of the condition and determined the factors associated with the prognosis of these patients. RESULTS: Obstructive symptoms were the most frequent clinical presentations (39% abdominal pain and 18% vomiting), while gastrointestinal bleeding was observed in 27% of patients. The diagnostic modalities included enteroscopy (33%), balloon-assisted enteroscopy (30%), and capsule endoscopy (13%). The most common primary tumor was lung cancer (38%), followed by colorectal cancer (18%), gastric cancer (9%), and malignant melanoma (6%). Surgical intervention, including tumor resection or bypass surgery, was performed in 79% of patients. The cumulative survival rates of patients at 12, 24, and 60 months were 49%, 36%, and 22%, respectively. Multivariate analysis identified surgery as a significant factor for improving overall survival (HR = 0.56, 95% CI 0.35-0.89, p = 0.01). CONCLUSIONS: The lung cancer is the most frequent primary tumor of metastatic tumors in the small bowel. Surgical intervention was associated with improved survival outcomes.

BACKGROUND: Peutz-Jeghers syndrome (PJS), a rare genetic disorder characterized by hamartomatous gastrointestinal polyps, poses increased risks of various cancers. Despite the importance of early intervention, the optimal timing for jejunal-ileal polypectomy remains unclear owing to the limited number of comparative studies. METHODS: Herein, we conducted a nationwide survey in Japan and analyzed data from 184 patients with PJS identified through a two-stage sampling process. The initial screening of 2912 medical institutions yielded 1748 facilities, of which 1077 responded to the survey. Time-dependent Cox proportional hazards models and logistic regression analyses were used to examine the association between the timing of jejunal-ileal polypectomy and the risk of surgery for intussusception. RESULTS: Among 184 patients (47.0% women; mean age, 33.5 years), intussusception was the most common complication (67.7%). In the Cox proportional hazards analysis excluding surgeries within 1 year of diagnosis, early jejunal-ileal polypectomy was associated with a reduced risk of surgery for intussusception (adjusted hazard ratio, 0.17; 95% confidence interval [CI] 0.04-0.74, p = 0.018). Logistic regression analysis showed higher odds of surgery in the late treatment group compared with the early treatment group (adjusted odds ratio, 4.26; 95% CI 1.38-13.16, p = 0.012). CONCLUSIONS: Early jejunal-ileal polypectomy may reduce the risk of intussusception in patients with PJS. However, the need for frequent endoscopic procedures must be balanced considering patient burden. These findings support the importance of early intervention and highlight the need for optimized surveillance strategies that consider clinical effectiveness and patients' quality of life.