Time Trend Analysis of Splenectomy for Splenic Marginal Zone Lymphoma: Declining Surgery, Promising Survival.

BACKGROUND: The purpose of current study was to examine the incidence, characteristics, treatment, and survival of splenic marginal zone lymphoma (SMZL). METHODS: Using SEER-18 database, patients diagnosed with SMZL between 2000 and 2018 were included. Effect of splenectomy on survival was evaluated after balancing the confounding factors by propensity score matching. Rates of splenectomy and 1-year relative survival were calculated for each year. A logistic regression model identified factors related to splenectomy, and a Cox regression model assessed factors linked to overall survival (OS). RESULTS: A total of 2790 patients with SMZL were analyzed. The majority were older than 60 years, female, and white. The age-adjusted incidence of SMZL was 0.17/100,000 person-years, with higher incidence in males. Incidence increased by 0.68%/year and peaked at 80-84 years for both genders. The SMZL-specific survival rates at 3 and 5 years were 89.6% and 85.3%, respectively. Meanwhile, the relative survival rates for the same periods were 88.6% and 85.9%, respectively. Splenectomy patients were more likely to be younger, male, and diagnosed with early-stage disease. Despite the decreasing utilization rate of splenectomy from 59.4% in 2000 to 16.2% in 2018, the 1-year relative survival rate remained relatively stable with minor fluctuations over time. Whether or not the patient underwent splenectomy was not found to be a significant prognostic indicator for OS. CONCLUSIONS: Our study demonstrated a decreasing use of splenectomy but a relatively stable survival in patients with SMZL, highlighting the urgency to better understand the role of splenectomy and its associated outcomes.

Risk of second primary malignancies in survivors of pancreatic neuroendocrine neoplasms from 2000 to 2018.

BACKGROUND AND AIM: As a result of improved survival, cancer survivors continue to remain at risk of developing second primary malignancies (SPMs). However, the association between first primary pancreatic neuroendocrine neoplasms (PanNENs) and SPMs has not been thoroughly investigated. METHODS: Using the Surveillance, Epidemiology, and End Results-18 database, patients histologically diagnosed with PanNENs as their first malignancy between 2000 and 2018 were identified. Standardized incidence ratios (SIRs) with 95% confidence intervals (CIs) and excess absolute risks per 10 000 person-years of SPMs were calculated to estimate the risk of being diagnosed with subsequent cancers compared with the general population. RESULTS: A total of 489 (5.7%) PanNENs survivors developed an SPM during the follow up, with a median latency between first and second cancer diagnoses of 32.0 months. The overall SIR of SPMs was 1.30 (95% CI: 1.19, 1.42) and the excess absolute risk was 35.67 cases per 10 000 person-years in comparison with the general population. Age 25-64 years at PanNENs diagnosis was associated with statistically higher risks for SPMs of all cancers combined. Latency stratification was significant for elevated SPMs risk between 2-23 and 84+ months after diagnosis. White patients were found to have a significantly increased incidence of SPMs (SIR: 1.23, 95% CI: 1.11, 1.35), mainly owing to the higher risk of stomach, small intestine, pancreas, kidney and renal pelvis, and thyroid cancers. CONCLUSION: Pancreatic neuroendocrine neoplasms survivors experience a significant increase in the burden of SPMs compared with the reference population. The heightened relative risk calls for careful long-term scrutiny as part of survivorship care plans.

Early-onset pancreatic neuroendocrine neoplasms: A distinct disease with improved survival compared with old individuals.

Background: The incidence, clinicopathologic characteristics, treatment patterns, and survival of early-onset pancreatic neuroendocrine neoplasms (EOPanNENs) have not been well explored. Methods: Patients diagnosed with PanNENs were identified from the SEER database between 2000 and 2018. EOPanNENs were defined as diagnosis in patients aged less than 50 years, while the remaining were defined as later-onset pancreatic neuroendocrine neoplasms (LOPanNENs). Incidence, clinical features, management, and prognosis were analyzed in our study. Multivariable analyses were performed to identify factors associated with overall survival (OS) in EOPanNENs and LOPanNENs, respectively. Results: A total of 5172 patients with PanNENs were included: 1267 (24.5%) in the EOPanNENs cohort and 3905 (75.5%) in the LOPanNENs cohort. The age-adjusted incidence rate significantly increased among later-onset cases, while it remained relatively stable in early-onset cases. EOPanNENs were more frequently to be female, unmarried, and with better tumor differentiation compared with LOPanNENs. Of note, early-onset patients presented with a higher rate of lymph node involvement, and they were more likely to receive surgical treatment. For local-regional disease at presentation, surgery alone was the most frequently used regimen over the last two decades. With regard to distant stage, a combination of surgery and chemotherapy was more often utilized. Risk factors for PanNENs survival were more correlated with LOPanNENs compared with EOPanNENs. The OS and cancer-specific survival (CSS) were significantly better in the EOPanNENs group. Further analyses showed that EOPanNENs ≤ 2cm were associated with more favorable survival outcomes than EOPanNENs>2cm. Conclusion: EOPanNENs are a clinically rare and distinct entity from LOPanNENs. The advantages in survival for the EOPanNENs cohort over time were largely driven by the indolent clinical courses including better tumor differentiation and intensified surgical treatment. Further investigations are warranted to better understand the characteristics of this disease subgroup.