The Risk of Second Primary Colorectal Adenocarcinomas Is Not Increased among Patients with Gastroenteropancreatic Neuroendocrine Neoplasms: A Nationwide Population-Based Study

2018 ◽  
Vol 107 (3) ◽  
pp. 280-283 ◽  
Author(s):  
Tobias Stemann Lau ◽  
Gitte Dam ◽  
Peter Jepsen ◽  
Henning Grønbæk ◽  
Klaus Krogh ◽  
...  

Background: Second primary colorectal adenocarcinomas (SPCA) may occur with a higher frequency in patients with gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs). In a nationwide population-based study, we investigated the risk of SPCA in GEP-NEN patients and compared it to the general population. Methods: Using the nationwide Danish registries, we identified 2,831 GEP-NEN patients (median age 63 years [IQR 50–73 years], 53% women) diagnosed in 1995–2010. We used Cox regression to compare the incidence of SPCA in GEP-NEN patients relative to a gender- and age-matched general population sample of 56,044 persons. Results: We observed 20 SPCAs among the 2,831 GEP-NEN patients with a total time at risk of 14,003 years (incidence = 143 per 100,000 person-years) and 770 colorectal adenocarcinomas in the general population of 56,044 persons with a total time at risk of 466,801 years (incidence = 165 per 100,000 person-years). The hazard ratio (HR) of SPCA from GEP-NEN diagnosis to the end of follow-up was 1.22 (95% CI: 0.78–1.92) in GEP-NEN patients compared to the general population. This nonsignificant association was the result of a strong positive association in the first 6 months after diagnosis of GEP-NEN (HR = 9.43 [95% CI: 4.98–17.86]) followed by a negative association in the remainder of the follow-up period (HR = 0.50 [95% CI: 0.20–1.21]). Conclusion: In this population-based study, there was no increased risk of SPCA among GEP-NEN patients. The clinical workup in newly diagnosed GEP-NEN patients likely explains the positive short-term association followed by a negative association.

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Christina Bergqvist ◽  
François Hemery ◽  
Arnaud Jannic ◽  
Salah Ferkal ◽  
Pierre Wolkenstein

AbstractNeurofibromatosis 1 (NF1) is an inherited, autosomal-dominant, tumor predisposition syndrome with a birth incidence as high as 1:2000. A patient with NF1 is four to five times more likely to develop a malignancy as compared to the general population. The number of epidemiologic studies on lymphoproliferative malignancies in patients with NF1 is limited. The aim of this study was to determine the incidence rate of lymphoproliferative malignancies (lymphoma and leukemia) in NF1 patients followed in our referral center for neurofibromatoses. We used the Informatics for Integrated Biology and the Bedside (i2b2) platform to extract information from the hospital’s electronic health records. We performed a keyword search on clinical notes generated between Jan/01/2014 and May/11/2020 for patients aged 18 years or older. A total of 1507 patients with confirmed NF1 patients aged 18 years and above were identified (mean age 39.2 years; 57% women). The total number of person-years in follow-up was 57,736 (men, 24,327 years; women, 33,409 years). Mean length of follow-up was 38.3 years (median, 36 years). A total of 13 patients had a medical history of either lymphoma or leukemia, yielding an overall incidence rate of 22.5 per 100,000 (0.000225, 95% confidence interval (CI) 0.000223–0.000227). This incidence is similar to that of the general population in France (standardized incidence ratio 1.07, 95% CI 0.60–1.79). Four patients had a medical history leukemia and 9 patients had a medical history of lymphoma of which 7 had non-Hodgkin lymphoma, and 2 had Hodgkin lymphoma. Our results show that adults with NF1 do not have an increased tendency to develop lymphoproliferative malignancies, in contrast to the general increased risk of malignancy. While our results are consistent with the recent population-based study in Finland, they are in contrast with the larger population-based study in England whereby NF1 individuals were found to be 3 times more likely to develop both non-Hodgkin lymphoma and lymphocytic leukemia. Large-scale epidemiological studies based on nationwide data sets are thus needed to confirm our findings.


2013 ◽  
Vol 169 (5) ◽  
pp. 577-585 ◽  
Author(s):  
Chang-Hsien Lu ◽  
Kuan-Der Lee ◽  
Ping-Tsung Chen ◽  
Chih-Cheng Chen ◽  
Feng-Che Kuan ◽  
...  

ObjectiveMost studies on second primary malignancies (SPMs) after primary thyroid cancer were conducted in USA or Europe. The discrepancy between SPMs in these studies could be attributed to geographical and ethnic heterogeneity. Thus, there is a clear need for another large-scale epidemiological study, particularly in Asian countries, to define the incidence and risk of SPMs in thyroid cancer survivors.DesignA population-based study was conducted using the nation-wide database from Taiwan Cancer Registry between 1979 and 2006.MethodsWe quantified standardized incidence ratios (SIRs) and cumulative incidence of SPMs among 19 068 individuals (4205 males and 14 863 females) with primary thyroid cancer.ResultsA total of 644 cases (3.38%) developed at least a SPM during 134 678 person-years of follow-up. The risk for subsequent SPMs was significantly greater than that of the general population (SIR=1.33, 95% CI 1.23–1.44). There was a greater risk of developing major salivary glands, nasopharyngeal, lung, thymus, breast (females), bladder, and brain cancers, and leukemia and lymphoma. We observed that the risk was highest within the first 5 years of diagnosis of thyroid cancer (SIR=5.29, 1.68, and 0.68 for ≦5, 5–10, and >10 respectively) and in the younger patients (SIR=1.81 vs 1.61 for <50 and ≧50 respectively). The median overall survival for primary thyroid cancer patients was 23.28 years, but it was only 4.73 years for those who developed SPMs.ConclusionThyroid cancer is associated with a 33% risk increment of SPMs, which had a negative impact on survival. There are sites of SPMs in the Asian population that are distinctive from those in the Western population, suggesting that other genetic predisposition or environmental factors may play a role.


Author(s):  
Arimatias Raitio ◽  
Johanna Syvänen ◽  
Asta Tauriainen ◽  
Anna Hyvärinen ◽  
Ulla Sankilampi ◽  
...  

Abstract Purpose Several studies have reported high prevalence of undescended testis (UDT) among boys with congenital abdominal wall defects (AWD). Due to rarity of AWDs, however, true prevalence of testicular maldescent among these boys is not known. We conducted a national register study to determine the prevalence of UDT among Finnish males with an AWD. Methods All male infants with either gastroschisis or omphalocele born between Jan 1, 1998 and Dec 31, 2015 were identified in the Register of Congenital Malformations. The data on all performed operations were acquired from the Care Register for Health Care. The register data were examined for relevant UDT diagnosis and operation codes. Results We identified 99 males with gastroschisis and 89 with omphalocele. UDT was diagnosed in 10 (10.1%) infants with gastroschisis and 22 (24.7%) with omphalocele. Majority of these required an operation; 8/99 (8.1%) gastroschisis and 19/89 (21.3%) omphalocele patients. UDT is more common among AWD patients than general population with the highest prevalence in omphalocele. Conclusions Cryptorchidism is more common among boys with an AWD than general population. Furthermore, omphalocele carries significantly higher risk of UDT and need for orchidopexy than gastroschisis. Due to high prevalence testicular maldescent, careful follow-up for UDT is recommended.


Author(s):  
Chieh-Sen Chuang ◽  
Kai-Wei Yang ◽  
Chia-Ming Yen ◽  
Cheng-Li Lin ◽  
Chia-Hung Kao

Objective: Previous research has demonstrated that patients with a history of organophosphate poisoning tend to have a higher risk of neurological disorder. However, research on the rate of seizure development in patients after organophosphate poisoning is lacking. This study examined whether individuals with organophosphate poisoning have an increased risk of seizures through several years of follow-up. Patients and Methods: We conducted a retrospective study on a cohort of 45,060 individuals (9012 patients with a history of organophosphate poisoning and 36,048 controls) selected from the Taiwan National Health Insurance Research Database. The individuals were observed for a maximum of 12 years to determine the rate of new-onset seizure disorder. We selected a comparison cohort from the general population that was randomly frequency-matched by age, sex, and index year and further analyzed the risk of seizures using a Cox regression model adjusted for sex, age, and comorbidities. Results: During the study period, the risk of seizure development was 3.57 times greater in patients with organophosphate poisoning compared with individuals without, after adjustments for age, sex, and comorbidities. The absolute incidence of seizures was highest in individuals aged 20 to 34 years in both cohorts (adjusted hazard ratio = 13.0, 95% confidence interval = 5.40−31.4). A significantly higher seizure risk was also observed in patients with organophosphate poisoning and comorbidities other than cirrhosis. Conclusions: This nationwide retrospective cohort study demonstrates that seizure risk is significantly increased in patients with organophosphate poisoning compared with the general population.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 15-17
Author(s):  
Renata Abrahao ◽  
Ann M Brunson ◽  
Justine M. Kahn ◽  
Qian Li ◽  
Aaron S Rosenberg ◽  
...  

Introduction Second primary malignancy (SPM) is one of the most devastating late complications following Hodgkin lymphoma (HL) treatment. Historically, the most common SPMs in patients treated for HL are solid tumors, which are largely related to radiation exposure during initial therapy. For the last three decades, efforts to address the risk of SPM after HL therapy have focused on reducing exposure to radiation, as well as refining the approach for patients where radiation is indicated. To date, few population-based studies in the United States have quantified the burden of SPMs and evaluated the potential effect of changes in therapeutic management over time. Additionally, to our knowledge, no study has compared SPM risk between human immunodeficiency virus (HIV)-infected and HIV-uninfected HL survivors. Methods We used data from the California Cancer Registry on 21,043 patients diagnosed with primary HL between 1988 and 2015 with follow-up through 2017. We calculated standardized incidence ratios (SIRs) with corresponding 95% confidence intervals (CIs) and absolute excess risks (AERs) to compare SPM incidence in our HL cohort with the expected number of first primary cancer incidence in the general California population, based on patient's age at diagnosis (5-year categories), sex, calendar year (3-year intervals), cancer site, and race/ethnicity. SIRs are presented by HIV status, SPM latency, treatment era, and cancer type. P-values for trends were used to examine whether SPM risk changed over time. Findings Among 20,303 HIV-uninfected patients (median follow-up of 14.1 years), overall SPM risk was increased 1.95-fold compared with the general population (SIR=1.95, 95% CI 1.86-2.04). In 740 HIV-infected patients (median follow-up of 11.7 years), overall risk was increased 2.68-fold compared with the general population (SIR=2.68, 95% CI 2.0-3.40), translating to an 37% higher incidence of SPM in HIV-infected vs. HIV-uninfected patients. The AER (per 10,000 person-years) of SPM was 43.1 in HIV-uninfected and 76.5 in HIV-infected patients, resulting in a 33.4 excess SPM per 10,000 person-years in HL survivors with HIV. Malignancies that contributed the most to overall AER were non-Hodgkin lymphoma (NHL), female breast and lung cancers in HIV-uninfected patients; and Kaposi sarcoma, NHL, anorectal and head & neck (HNC) cancers in HIV-infected patients. Notably, among HIV-uninfected patients, the highest overall risk of SPM occurred ≥20 years after diagnosis (SIR= 2.27, 95% CI 1.99-2.58) (Figure). In contrast, the highest overall risk in HIV-infected patients was observed &lt;2 years after diagnosis (SIR=4.42, 95% CI 2.53-7.19). Radiation used decreased from 46.9% in 1988-1996 to 29.5% in 2007-2015. Among HIV-uninfected patients, there was a trend towards decreased risk over time of overall and selected solid SPMs (lung, female breast, and gastrointestinal cancers) (Table). In an analysis restricted to HIV-uninfected patients who received radiation irrespective of chemotherapy, findings also suggested a declined risk of overall and selected solid SPMs over time: any solid (SIR=2.15 in 1988-1996 and SIR=1.30 in 2007-2015, p&lt;0.0001), lung (SIR=3.69 in 1988-1996 and SIR=1.81 in 2007-2015, p=0.0031), and female breast (SIR=2.95 in 1988-1996 and SIR=0.63 in 2007-2015, p&lt;0.0001). Conclusion Compared with the general population, the risk of developing a SPM following HL treatment was significantly higher among both HIV-uninfected and HIV-infected patients, with the absolute excess risk greater for those with HIV infection. There were different temporal patterns and types of SPM between HIV-uninfected and HIV-infected patients. These findings prompt the question on whether earlier and/or more intensive cancer screening should be pursued for HIV-infected survivors. The trend towards decreased risk for selected solid SPMs among HIV-uninfected patients, especially lung and female breast cancers, suggest that strategies to reduce radiation in HL survivors may be working. Despite promising trends in this group, the observation that SPM risk was highest ≥20 years after initial therapy further highlights the need for long-term surveillance and survivorship care in this at-risk population. Disclosures Rosenberg: Takeda: Speakers Bureau; Janssen: Speakers Bureau; Amgen: Membership on an entity's Board of Directors or advisory committees; Seattle Genetics: Membership on an entity's Board of Directors or advisory committees. Wun:Glycomimetics, Inc.: Consultancy.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 632-632 ◽  
Author(s):  
Malin Hultcrantz ◽  
Therese M-L Andersson ◽  
Ola Landgren ◽  
Asa Rangert Derolf ◽  
Paul W Dickman ◽  
...  

Abstract Background Patients with myeloproliferative neoplasms (MPNs) are reported to have a higher risk of thrombosis compared to the general population. However, the magnitude of the increase in risk in MPNs patients is not known since there is a lack of studies including control subjects. Therefore, we conducted a large population-based study to assess the risk of arterial and venous thrombosis in patients with MPNs in relation to matched controls. Patients and Methods All patients with MPNs reported to the Swedish Cancer Register and/or registered in the Inpatient Register from 1980 to 2009 were included. For each patient, four controls matched for age, sex, and county of residence, were randomly identified from the Register of Total Population. End of follow-up was December 31st 2010. Events of arterial and venous thrombosis, including deaths, were identified from the Inpatient and Outpatient Registers and the Cause of Death Register. Arterial thrombosis was defined as myocardial infarction, ischemic stroke, or peripheral arterial thrombus/embolus. Venous thrombosis was defined as pulmonary embolism, deep venous thrombosis (DVT), liver or splanchnic vein thrombosis, cerebral venous sinus thrombosis, or other venous thrombosis/embolus. Odd ratios (ORs) of arterial and venous thrombosis at diagnosis +/-30 days were calculated using logistic regression. Cox regression and flexible parametric models were used to estimate proportional and non-proportional hazard ratios (HRs) with 95% confidence intervals (CIs). Follow-up in all regression models started 30 days after diagnosis to avoid detection bias at time of diagnosis. Results A total of 11,155 patients and 44,620 matched controls were identified. Forty-six percent (n=5,161) were men and median age at MPN diagnosis was 69 years. The OR for arterial and venous thrombosis at time of MPN diagnosis +/- 30 days was 55.0 (95% CI 51.1-59.2 p<0.001) and 64.3 (42.2-98.1 p<0.001) respectively in MPN patients compared to controls. In MPN patients, the risk of arterial thrombosis was significantly 4.9-fold (4.8-5.0 p<0.001) increased compared to matched controls. The HR of myocardial infarction was 3.9 (3.7-4.1 p<0.001) and stroke 4.9 (4.8-5.0 p<0.001) in MPN patients. The HR of arterial thrombosis in MPN patients decreased shortly after diagnosis but thereafter increased with follow-up time in relation to controls (Figure 1a). There was a similar risk of arterial thrombosis in patients of different MPN subtypes compared to controls, the HR in patients with polycythemia vera (PV) was 5.0 (4.8-5.2), essential thrombocythemia (ET) 4.7 (4.6-5.0), primary myelofibrosis (PMF) 5.0 (4.7-5.3), and MPN-unclassifiable (MPN-U) 5.1 (4.8-5.5), respectively. The HR of venous thrombosis in MPN patients was 6.7 (6.2-7.2 p<0.001), where the HRs of pulmonary embolism was 7.5 (6.6-8.5 p<0.001) and DVT was 5.3 (4.8-5.9 p<0.001) compared to matched controls. MPN patients had a substantially increased risk of liver and splanchnic vein thrombosis, HR=41.4 (26.4-64.9 p<0.001). The risk of venous thrombosis in MPN patients decreased shortly after diagnosis and thereafter remained stable in relation to controls during follow-up time (Figure 1b). Compared to controls, patients with PV had a larger increase in risk of venous thrombosis than the other subtypes (HR=9.0; 8.0-10.1), while the HR of venous thrombosis in patients with ET was 5.4 (4.7-6.2), PMF 6.0 (4.9-7.5), and MPN-U 4.6 (3.7-5.7), respectively. Conclusions Patients with MPNs have an overall five- to sevenfold elevated risk of thrombosis compared to the general population. The highest HRs were seen for venous thrombosis, especially abdominal thrombosis. The odds of having a thrombosis at time of MPN diagnosis were high, indicating that thrombosis is an important first symptom of MPN. The elevated risk of arterial thrombosis increased while the elevated risk of venous thrombosis remained stable during follow-up time. This large population-based study is, to our knowledge, the first to quantify the excess risk of thrombosis in MPN patients compared to the general population. Our results indicate that we need to consider time after diagnosis in risk score models and rethink the strategies for thromboprophylaxis in patients with MPN in order to decrease the risk of thrombotic events. Figure 1A Risk of arterial (a) and venous (b) thrombosis in MPN patients compared to matched controls during follow-up time. Figure 1A. Risk of arterial (a) and venous (b) thrombosis in MPN patients compared to matched controls during follow-up time. Figure 1B Figure 1B. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1830-1830
Author(s):  
Amir Bista ◽  
Dipesh Uprety ◽  
Yazhini Vallatharasu ◽  
Lubina Arjyal ◽  
Subash Ghimire ◽  
...  

Abstract Introduction: Systemic mastocytosis (SM) is a rare hematological disorder characterized by clonal proliferation and activation of abnormal mast cells. It can vary from an indolent form to an aggressive form including progression to leukemia. There is limited data on epidemiology, clinical characteristics and outcome of this disease in population based setting. Till date, a retrospective study by Lim, Ken-Hong, et.al which included 342 patients diagnosed with systemic mastocytosis in Mayo clinic, is the largest series of patient published so far. This study reports the clinical presentation and outcome of patients with SM but there are no population based study in United States so far. We therefore conducted this population based study to determine epidemiology, survival pattern and incidence of second primary malignancy among patients with SM. Methods: We used SEER 18 database (2000-2014) to select all adult patients with age 20 or above with SM. Patient population was divided into various cohorts based on age (20-59, 60-79 and 80+ years), sex, race (Caucasians, African American and Others), area of residence (rural, urban and metropolitan) and annual household income (<$25000, $25000-<$50000 and ≥$50000). Age adjusted incidence rate was calculated using 2000 US standard population using SEER stat rate session. 5-year relative survival (RS) rate was calculated using SEER stat and compared using Z test. Cox proportional hazard model was used for multivariate analysis of factors associated with relative survival using Cansurv software. MP-SIR session in SEER stat was used to calculate the risk of second primary malignancy. Result: The incidence was found to be 0.046 per 10000 among general population. Incidence was found to be higher among Caucasians compared to African American (0.056 vs. 0.018 per 100000). Median age at diagnosis was 55 years. Of the total 425 patients, majority were Caucasians (92.5%), age <60 years (59.3%) and from metropolitan area (84.7%) but there was equal distribution among male and females. 10 year overall survival was found to be 61.5±3.1% by KM curve. 5-year RS was found to be 74.0±2.7% for the whole population. Females had significantly better survival compared to males, 5-year RS of 84.7±3.1% vs. 62.3±4.3%, P <0.0001. Survival trended to be better for Caucasians compared to African American but didn't reach clinical significance, 74.6±2.8 vs. 50±14.8, p 0.08. Patients <60 years had significant better survival compared to 60 to 79 and 80+ years ( 5-year RS of 88.6±2.4 vs. 58.4±5.2 and 16.0±11.2 respectively with P <0.0001 for both comparison). In multivariate analysis, younger age group, female sex and Caucasian race were found to be independent predictor of better 5 year relative survival with P<0.0001 compared to their counterparts. Patients with systemic mastocytosis were found to have higher risk of developing both solid organ as well as hematological malignancy within 5 years of diagnosis (as shown in table 1). After that the risk decreased and was comparable to general population. Conclusion: Our study shows that systemic mastocytosis is rare disease in general population and survival is better among specific subgroup of patients including females, younger patient and Caucasians. As the majority deaths occurred in first 5 years after diagnosis and as incidence of second primary malignancy is higher in the first 5 years, we recommend close follow up for first five years after diagnosis. Disclosures No relevant conflicts of interest to declare.


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