Cancer Institute's Surveillance Research Articles

Uptake in sentinel lymph node biopsy for endometrial cancer with T3 classification

ObjectiveThe current clinical practice guidelines for endometrial cancer specify sentinel lymph node (SLN) biopsy to be performed in apparent uterine-confined disease. However, a recent population-based analysis found that the utilization of SLN biopsy is increasing in extra-uterine disease such as T2 classification. The objective of this study was to examine trends and outcomes related to SLN biopsy for endometrial cancer with T3 classification, another extra-uterine disease. MethodsA population-based retrospective cohort study was conducted to examine 7004 women with T3 endometrial cancer who underwent primary surgery between 2010 and 2018, identified in the National Cancer Institute's Surveillance, Epidemiology, and End Results Program. Trends and characteristics related to SLN biopsy were assessed by multinomial regression analysis, and inverse probability of treatment weighting propensity score was used to assess overall survival related to SLN biopsy. ResultsNodal evaluation type included lymphadenectomy (n = 5276, 75.3%), SLN biopsy (n = 287, 4.1%), and none (n = 1441, 20.6%). The utilization of SLN biopsy increased from 0.4% to 12.9% between 2010 and 2018 (P < 0.001) that this association remained independent in multivariable analysis (adjusted-odds ratio compared to 2010–2012, 2.63 [95% confidence interval 1.57-4.42] for 2013–2015 and 10.1 [95% confidence interval 6.30-16.2] for 2016–2018). When compared to the lymphadenectomy group, the SLN biopsy group was less likely to have T3b disease (adjusted-odds ratio 0.69, 95% confidence interval 0.51–0.94) but had similar postoperative chemotherapy and radiotherapy (both, P > 0.05). In a weighted model, the 3-year overall survival rate was 66.3% for the SLN biopsy group and 64.7% for the lymphadenectomy group (hazard ratio 0.85, 95% confidence interval 0.69–1.05). Similar association was observed in subcohorts for young, old, endometrioid, non-endometrioid, T3a, T3b, and N0 cases. ConclusionUtilization of SLN biopsy in T3 endometrial cancer is increasing in the United States.

Characterizing isolated tumor cells in regional lymph nodes of early endometrial cancer

ObjectiveTo examine the characteristics of isolated tumor cells (ITCs) in regional lymph nodes of early-stage endometrial cancer. MethodsThis is a retrospective cohort study examining the National Cancer Institute's Surveillance, Epidemiology, and End Result Program. The study population was 6472 women with non-metastatic, node-negative T1 endometrial cancer who underwent primary hysterectomy and surgical nodal evaluation. Multivariable binary logistic regression model was used to identify the independent characteristics for ITCs. Postoperative therapy according to ITCs status was also assessed with propensity score weighting. ResultsITCs were seen in 111 (1.7%) cases. In a multivariable analysis, ITCs were largely associated with tumor factors including deep myometrial invasion (T1b versus T1a, 4.0% versus 1.0%, adjusted-odds ratio [aOR] 3.42, P < 0.001) and larger tumor size (>4 versus ≤4 cm, 3.0% versus 1.6%, aOR 1.55, P = 0.037). Moreover, women undergoing sentinel lymph node (SLN) biopsy had a higher likelihood of identifying ITCs compared to those undergoing lymphadenectomy (LND): 2.7% for SLN alone, 3.7% for SLN/LND, and 1.2% for LND alone (aOR ranged 2.60–2.99, P < 0.001). Women who had ITCs identified were more likely to receive postoperative therapy (81.8% versus 31.7%, P < 0.001), including external beam radiotherapy (EBT) alone (25.1% versus 3.2%) and chemotherapy/EBT (16.3% versus 1.9%). Similar associations were observed in the low-risk group (stage IA, grade 1–2 endometrioid, 78.4% versus 9.2%, P < 0.001), including EBT alone (35.3% versus 0.6%). ConclusionThis study suggests that a SLN protocol can identify more ITCs in the regional lymph nodes of early endometrial cancer. Deep myometrial invasion and large tumor size were associated with increased risk of ITCs. Postoperative therapy is offered more frequently in the setting of ITCs with variable treatment patterns, warranting further outcome studies and practice guidelines.

Pediatric neurological cancer incidence and trends in the United States, 2000-2018.

To characterize the epidemiological trends and sociodemographic variation of pediatric and adolescent neurological cancers by histological subtypes over time in the USA. A total of 16,511 patients aged 0-19years diagnosed with neurological cancers between 2000 and 2018, including 13,024 with central nervous system (CNS) neoplasms and 3,487 with neuroblastomas, were identified from 18 registries of the National Cancer Institute's Surveillance, Epidemiology, and End Results Program. Incidence trends over time and incidence rate ratios by race/ethnicity, sex, and age were calculated for histological subtype. Age-standardized incidence rates (ASIR) by year of diagnosis and average annual percent changes (AAPC) were calculated to measure incidence rates. ASIR by race/ethnicity, sex, and age were calculated to examine the incidence variation by these factors. Overall, age-standardized annual incidence per 100,000 person-years increased from 2.20 in 2000 to 3.21 in 2018 with an AAPC of 1.4% (95% confidence interval or CI: 0.5% to 2.4%); however, that of Hispanic decreased from 2.93 in 2000 to 2.59 in 2018 with an AAPC of -0.8% (95% CI: -1.2% to -0.3%). Non-Hispanic Black children and adolescents had a statistically significantly lower incidence than non-Hispanic White peers both for CNS neoplasms (incidence rate ratio or IRR: 0.67; 95% CI: 0.63 to 0.71) and neuroblastomas (IRR: 0.75; 95% CI: 0.68 to 0.83). Females generally had a lower incidence than males, especially among those with intracranial and intraspinal embryonal tumors (IRR: 0.69; 95% CI: 0.64 to 0.75). The highest incidence rate of neuroblastoma was among newborns aged less than 1year, and the highest incidence rate of CNS neoplasms was among children aged 1-4years. The incidence of neurological cancers has increased among children and adolescents from 2000 to 2018, with wide variation across demographic groups.

Incorporation of sentinel lymph node biopsy in cervical cancer surgery: Recent U.S. trends

ObjectiveTo examine the trends, characteristics, and outcomes related to sentinel lymph node (SLN) biopsy for cervical cancer surgery. MethodsThis retrospective cohort study queried the National Cancer Institute's Surveillance, Epidemiology, and End Results Program. Study population included patients with invasive cervical cancer (squamous cell carcinoma, adenocarcinoma, and adenosquamous carcinoma) who underwent both hysterectomy and lymphadenectomy for T1 classification from 2003 to 2018. Exposure allocation was per surgical nodal evaluation type (SLN biopsy or lymphadenectomy). Main outcome measures were (i) trend of utilization and patient characteristics related to SLN biopsy assessed with multivariable analysis and (ii) overall survival associated with SLN biopsy assessed with propensity score inverse probability of treatment weighting. Sensitivity cohorts included uterine-preserving conservative surgeries. ResultsA total of 12,966 patients met the inclusion criteria. Of those, 430 (3.3%) patients underwent SLN biopsy. The utilization of SLN biopsy increased significantly from 0.8% to 15.2% during the study period (P < 0.001). This association remained independent in multivariable analysis: 2011–2014 versus 2003–2010 adjusted-odds ratio 4.87, 95% confidence interval (CI) 3.29–7.23, and 2015–2018 versus 2003–2010 adjusted-odds ratio 20.6, 95%CI 14.6–29.2. In a propensity score weighted model, patients who had SLN biopsy had similar overall survival compared to those without SLN biopsy (3-year rates, 94.8% versus 94.2%, hazard ratio 0.95, 95%CI 0.64–1.41, P = 0.799). In sensitivity analysis, the increase in SLN biopsy was also observed in uterine-preserving surgeries (3.5% to 9.6% for trachelectomy, P = 0.043; and 2.5% to 19.5% in cervical excision, P < 0.001). ConclusionLandscape of surgical nodal evaluation is gradually shifting from lymphadenectomy to SLN biopsy in cervical cancer surgery.

"The past is a different country, they do things differently there": using the SEER data-base to assess prognosis in neuroendocrine tumours.

Neuroendocrine neoplasms (NENs) are still considered to be rare neoplasms, and their epidemiology has been classically studied in large population-based cancer registries. Besides the benefits and the limitations that a cancer registry may have for all the registered cancers, the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Programme, has a number of drawbacks for the study of NENs. The change in management of NENs, either from diagnostic or from therapeutic points of view, the role of the Ki-67 labelling index and introduction of sensitive functional imaging, along with the misclassification of the more benign types of NENs, are the main limitations of the prognostic ability of the SEER data-base, particularly when including older data.

Population-level trends and outcomes of sentinel lymph node biopsy in vulvar cancer surgery in the United States

ObjectiveTo examine population-level trends, characteristics, and outcomes related to nodal assessment for vulvar cancer surgery in the United States. MethodsThis is a retrospective cohort study querying the National Cancer Institute's Surveillance, Epidemiology, and End Results Program. The study population was 5604 women with T1b or T2-smaller(≤4 cm) squamous cell carcinoma of the vulva who underwent primary vulvectomy from 2003 to 2018. The exposure allocation was based on nodal evaluation type: lymphadenectomy (LND; n = 3319, 59.2%), sentinel lymph node (SLN) biopsy (n = 751, 13.4%), or no surgical nodal evaluation (n = 1534, 27.4%). The main outcomes were (i) trends and characteristics related to SLN biopsy assessed by multinomial regression model, and (ii) vulvar cancer-specific survival assessed by competing risk analysis and inverse probability of treatment weighting propensity score. Sensitivity analysis included evaluation of external cohort with T1a disease (n = 1291). ResultsThe utilization of SLN biopsy increased from 5.7% to 23.3% in 2006–2018, while the proportion of LND decreased from 64.1% to 48.8% in 2010–2018, and these associations remained independent in multivariable analysis (adjusted-P < 0.05). In the propensity score weighted model, 5-year cumulative rate for vulvar cancer-specific mortality was 15.2% (interquartile range 12.1–18.9) for the SLN biopsy group and 16.9% (interquartile range 15.6–18.3) for the LND group (subdistribution-hazard ratio 0.90, 95% confidence interval 0.76–1.06, P = 0.217). The increasing SLN biopsy use was also observed in T1a disease from 1.3% to 7.3% during the study period (P < 0.001). ConclusionThe landscape of surgical nodal evaluation is shifting from lymphadenectomy to SLN biopsy in vulvar cancer surgery in the United States. SLN biopsy-incorporated treatment approach was not associated with worse survival compared to LND.

Rural-Urban Differences in Breast Cancer Stage at Diagnosis.

Purpose: To analyze the extent to which rural-urban differences in breast cancer stage at diagnosis are explained by factors including age, race, tumor grade, receptor status, and insurance status.Methods: Using the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) 18 database, analysis was performed using data from women aged 50–74 diagnosed with breast cancer between the years 2013 and 2016. Patient rurality of residence was coded according to SEER's Rural-Urban Continuum Code 2013: Large Urban (RUCC 1), Small Urban (RUCC 2,3), and Rural (RUCC 4,5,6,7,8,9). Stage at diagnosis was coded according to SEER's Combined Summary Stage 2000 (2004+) criteria: Localized (0,1), Regional (2,3,4,5), and Distant (7). Descriptive statistics were analyzed, and variations were tested for across rural-urban categories using Kruskall–Wallis and Kendall's tau-b tests. Additionally, odds ratios (ORs) and 95% confidence intervals for the three ordinal levels of rural-urban residence were calculated while adjusting for other independent variables using ordinal logistic regression.Results: The rural residence category showed the largest proportion of women diagnosed with distant stage breast cancer. Additionally, we determined that patients with residence in both large and small urban areas had statistically significantly lower odds of higher stage diagnosis compared to rural patients even after controlling for age, race, tumor grade, receptor status, and insurance status.Conclusions: Rural women with breast cancer show small but statistically significant disparities in stage-at-diagnosis. Further research is needed to understand local area variation in these disparities across a wide range of rural communities, and to identify the most effective interventions to eliminate these disparities.

Clinicopathologic features of breast cancers diagnosed in women treated with prior radiation therapy for Hodgkin lymphoma: Results from a population-based cohort.

Childhood and young adult survivors of Hodgkin lymphoma (HL) are at elevated risk of developing breast cancer, yet little data exist on the tumor characteristics that develop in this high-risk patient population. The National Cancer Institute's Surveillance, Epidemiology, and End Results database was used to identify breast cancers diagnosed between 1990 and 2016 in women who had received prior radiation therapy for HL at age 30years or younger. Clinicopathologic features of subsequent breast cancers (breast cancer after radiation therapy for HL [BC-HL]) were examined and compared with breast cancers diagnosed in women who had no prior malignancy (breast cancer with no prior malignancy [BC-NPM]). In total, 321 breast cancers were identified in 257 women who had a history of radiation therapy for HL. The median age at HL diagnosis was 22years (interquartile range, 18-26years), and nearly all patients in the BC-HL group (97.9%) were diagnosed ≥8years after radiation therapy. Overall, 56 patients in the BC-HL group (21.8%) developed bilateral breast cancer. Compared with women who had BC-NPM, those who had BC-HL were younger (43 vs 60years; P<.001) and were less likely to present with ductal carcinoma in situ (8.4% vs 14.9%; P=.001). On multivariable analysis that included adjustment for age, invasive BC-HL was associated with smaller (≤2cm) tumor size (odds ratio, 1.64; 95% CI, 1.25-2.15) and upper outer quadrant tumors (odds ratio, 1.37; 95% CI, 1.04-1.81) compared with BC-NPM. In a subset analysis of 102 women who had HER2/neu status available, the distribution of biologic subtype was not significantly different between BC-HL and BC-NPM (P=.16). Breast cancers in women who previously received radiation therapy for HL are characterized by earlier onset disease, although most remain estrogen receptor-positive and have early stage disease at presentation. Women who have had radiation therapy for Hodgkin lymphoma at a young age are at increased risk of developing early onset breast cancer; however, most of these breast cancers are sensitive to hormones (estrogen receptor-positive) and are diagnosed at early stages. Because these breast tumors are estrogen receptor-positive, medications that prevent breast cancer by blocking the effect of or lowering hormone levels (also termed endocrine prevention) may be useful in this group of high-risk women.

Effect of postmastectomy radiotherapy on survival outcomes of patients with metaplastic breast cancer

To evaluate the effect of postmastectomy radiotherapy (PMRT) on the overall survival (OS) and breast cancer-specific survival (BCSS) of patients with metaplastic breast cancer (MpBC) in comparison with those of patients with invasive ductal breast carcinoma (IDC). We selected the patients with pathologically confirmed MpBC and IDC who either received PMRT or not from the archived cases (from January, 1998 to December, 2016) in the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database. In total, 31 982 patients were selected, including 308 patients with MpBC and PMRT, 629 with MpBC and PMRT, and 31 045 with IDC and PMRT. All the included patients were above 18 years of age without distant metastases or a second primary cancer and underwent radical surgery. Baseline characteristics of the patients were compared among the 3 subgroups, and multivariate Cox regression and Kaplan-Meier analyses were performed for analyzing the prognostic factors of MpBC, OS, and BCSS. The majority (81.2%) of patients with MpBC were older than 50 years, had pathological grade III (68%), and were negative for ER (75.9%) and PR (79.8%) and in stage T2-3 (71.3%) and N0-1 (85.6%). Multivariate Cox regression analysis showed that age, T stage, N stage, PMRT, and chemotherapy were significantly associated with the prognosis of patients with MpBC (P < 0.05), while pathological grade, ER status, or PR status did not significantly affect the prognosis (P>0.05). Kaplan-Meier analysis showed that the patients with MpBC and PMRT had better OS (HR=1.394, 95% CI: 1.125-1.727; P < 0.05) and BCSS (HR=1.390, 95% CI: 1.074-1.800; P < 0.05) than those with MpBC who did not receive PMRT; but after PMRT, the patients with MpBC had worse OS (HR=1.626, 95%CI: 1.386-1.908; P < 0.001) and BCSS (HR=1.710, 95% CI: 1.418-2.062; P < 0.001) as compared with those with IDC. MpBC has unique clinicopathological features. In patients with MpBC, age, T stage, N stage, radiotherapy and chemotherapy are all the prognostic factors affecting the survival outcomes, and PMRT can improve the OS and BCSS of the patients.

With Equal-Access, Service Members with Lymphoma Have Better Outcomes Than Their Civilian Counterparts

Introduction: The Military Health System (MHS) is a single-payer, equal-access healthcare system that provides healthcare to 1.4 million active-duty service members (ADSMs) from diverse socioeconomic backgrounds. ADSMs with cancer receive prompt care without copayments, deductibles, prior authorizations, or other insurance barriers. Lymphoma is the most common hematologic malignancy diagnosed in ADSMs. We sought to compare overall survival (OS) of ADSMs with lymphoma with matched civilian lymphoma patients. We also sought to identify variables among ADSMs that predicted OS.Methods: The Department of Defense Automated Central Tumor Registry (ACTUR) was retrospectively accessed to identify ADSMs with Hodgkin Lymphoma (HL), diffuse large B-cell lymphoma (DLBCL), and indolent lymphomas over a 20-year span from 1997-2017. A comparator arm was created from the National Cancer Institute's Surveillance, Epidemiology and End-Result (SEER)-18 database and was matched in a 4:1 ratio by age, sex, race, ethnicity, year of diagnosis, and stage. OS was assessed with Cox proportional hazards models. Kaplan Meier curves were constructed and compared using log rank tests. Among ADSMs, univariate and multivariable analyses were performed to assess OS based on race, ethnicity, sex, age at diagnosis, stage, year of diagnosis, rank, branch of service, and geographic region at diagnosis. All data analyses were conducted using STATA/SE 15.1 for Windows; the alpha level for statistical significance was set to 0.05.Results: There were 1,170 ADSMs with HL, 443 with DLBCL, and 284 with indolent lymphomas identified from ACTUR. Most ADSMs were between ages 20-40, white/non-Hispanic, enlisted, and in the Army. All three groups of ADSMs had superior OS when compared with their matched civilian counterparts. There were 49%, 48%, and 35% reductions in the risk of death among ADSMs with DLBCL, indolent lymphoma and Hodgkin lymphoma, respectively (DLBCL hazard ratio [HR] 0.51, 95% CI 0.40-0.65, p<0.0001; indolent HR 0.52, 95% CI 0.33-0.81, p <0.002; HL HR 0.65, 95% CI 0.52-0.82, p <0.002). Among the ADSMs, variables that reached statistical significance in the univariate and/or the multivariable analyses for OS include increasing age (DLBCL, indolent), advanced stage (DLBCL, HL), and geographic residence at diagnosis (DLBCL, HL). Notably, there were no statistically significant differences in OS based on race, ethnicity, sex, rank, or branch of service.Discussion: ADSMs with lymphoma treated within the MHS have significantly better survival than their matched civilian counterparts. Further, disparities based on race/ethnicity and sex are not present in the MHS, despite being pervasive in the civilian sector. It is possible that better access to care within the equal-access MHS might explain our results. Alternatively, regular fitness tests and periodic health assessments may render ADSMs are more fit and less comorbid than their civilian counterparts, translating to a better chance of long-term survival. Although this study is limited by its retrospective design and possible coding inaccuracies, the finding of advanced age and stage impacting OS serves as a good internal control and confirms the quality of ACTUR data. Findings from this study reflect favorably on ADSMs with cancer. The absence of racial/ethnic or gender disparities reflects favorably on cancer care received within the equal-access MHS. [Display omitted] DisclosuresThornton: Undisclosed Companies: Current equity holder in publicly-traded company.

Spectrum of Second Primary Malignancies and Cause-Specific Mortality Among Patients with Hairy Cell Leukemia in the United States: A Population-Based Study

BACKGROUNDHairy cell leukemia (HCL) is a rare lymphoid neoplasm with an incidence of 1,000 new cases per year and accounting for 2% of all leukemias in the United States. There is a lack of data on the spectrum of second primary malignancies (SPMs) and cause-specific mortality in the era of targeted therapies (anti-CD20, anti-CD22, and BRAF-inhibitors). We undertook this study to address the gaps in knowledge using a national registry from the United States. METHODSWe estimated the incidence of SPMs and cause-specific mortality among two-month survivors of hairy cell leukemia (HCL) using the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER)-18 registries. We included patients from 2003-2018 with ICD-O-3 code 9940/3 from the registry. We calculated the risk of SPMs using standardized incidence ratios (SIRs), and cause-specific mortality by standardized mortality ratios (SMRs) and absolute excess risk (AER)/10,000 population. We also reported survival analysis using relative survival rates (RSRs). RSR was calculated as ratio of observed to expected survival compared to the 2000 US standard population and adjusted for age, sex, and race. Overall survival (OS) analysis was reported using Kaplan-Meier (KM) method. RESULTSWe included a total of 4,261 patients with Hairy cell leukemia in this study. The median age at diagnosis was 59 yrs (range 21-85). The median follow-up duration for entire cohort was 68 months (range 0-191 mo). A total of 538 (12.6%) patients developed SPMs at last follow-up, of which 75% were solid organ malignancies, and 25% were hematologic neoplasms. The SPM risk for cohort was significantly elevated (SIR 1.33, 95% confidence interval [CI] 1.22 - 1.45) compared with the general population. The median latency period for SPM development was 3 yrs (range 1-15 yrs). The SPM risk was statistically higher than general population among Whites with HCL (SIR 1.33, 95% CI 1.22 - 1.45), but not significant among Blacks (SIR 1.31, 95% CI 0.76 - 2.09). The SIR was slightly higher in females (SIR 1.39, 95% CI 1.12 - 1.71) when compared to males (SIR 1.32, 95% CI 1.20 - 1.45). The SIR according to age groups were as follows: age < 65 years (SIR 1.36, 95% CI 1.21 - 1.52), age > 65 years (SIR 1.31, 95% CI 1.15 - 1.48). The risk for hematopoietic SPMs was higher (SIR 2.82, 95% CI 2.31 - 3.41) compared with solid organ tumors (SIR 1.16, 95% CI 1.05 - 1.28). Specific SPMs with highest risk included non-epithelial skin cancers (SIR 5.20, 95% CI 2.59 - 9.30), Hodgkin lymphoma (SIR 4.68, 95% CI 1.52 - 10.91), non-Hodgkin lymphoma [NHL] (SIR 3.91, 95% CI 3.04 - 4.95), myeloid and monocytic leukemia (SIR 2.23, 95% CI 1.15 - 3.90), melanoma (SIR 1.60, 95% CI 1.12 - 2.20), and prostate cancer (SIR 1.45, 95% CI 1.21 - 1.72). Although the risk of developing secondary NHL was significantly high throughout the follow-up period, risk for Hodgkin lymphoma was only significant >10 yr of follow-up (SIR 9.71, 95% CI 1.18 - 35). 5-year RSR for the cohort was 94.6%. On KM analysis, the mean OS for entire cohort was 11.8 yr, but the median OS was not reached (Figure 1). At last follow-up, 770 (18%) patients had died, of which 49% were due to malignant neoplasms. There was statistically significant risk of mortality due to all malignant cancers combined (SMR 2.41, 95% CI 2.17 - 2.66), largely due to mortality from leukemia (SMR 32.62, 95% CI 28.41 - 37.25, AER 70.2) and NHL (SMR 5.49, 95% CI 3.78 - 7.71, AER 9.01) (Table 1). Interestingly, risk of mortality from non-neoplastic causes was lower than general population: cardiovascular disease (SMR 0.70, 95% CI 0.58 - 0.83), pulmonary diseases (SMR 0.44, 95% CI 0.26 to 0.70), and infectious diseases (SMR 1.27, 95% CI 0.55 - 2.51). The risk of death from leukemia was highest within first year of diagnosis of HCL and continued to slowly decline thereafter, although remained statistically significantly elevated more than other causes of death (SMR 1yr 112, AER 214; SMR 1-5 yrs 36, AER 72; SMR 5-10 yrs 20, AER 43; SMR >10 yrs 17, AER 43). CONCLUSIONOur study reveals that despite therapeutic advances in HCL, hematologic neoplasms (including leukemia) remain the leading cause of death in last two decades. Additionally, the risk of developing SPMs remains elevated in patients with HCL, and is significantly high in Whites compared with Blacks. Further studies are needed to better define the healthcare needs of HCL patients beyond initial treatment to reduce the burden of mortality from HCL and other SPMs. [Display omitted] DisclosuresNarkhede: Gilead: Research Funding; Genentech/Roche: Research Funding; Genmab: Other: Medical writing support, Research Funding; TG Therapeautics: Research Funding.

Normal Life Expectancy for Polycythemia Vera Patients Is Possible

▪Introduction: Polycythemia vera (PV), a chronic myeloproliferative neoplasm, is characterized by shortened overall survival (OS) due to potentially fatal thrombosis, progression to myelofibrosis (MF), and acute leukemia. Treatment with aspirin, attentive phlebotomy (PHL) and cytoreductive agents can prevent thrombosis; but it is unknown whether available treatments can achieve a normal life-span for PV patients (pts). Our objective was to assess the extent by which PV alters OS of pts receiving available treatment at our center and in the community.Methods: We obtained demographics and survival data of adult PV pts from our Weill Cornell Medicine (WCM) Research Database Repository (PV-WCM) diagnosed from 1974-2020 as previously described (Abu-Zeinah et al. Leukemia 2021); and from the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) Program, 2001-2017. PV-WCM thrombosis and MF progression data were available. WCM institutional review board approval and SEER data use agreement were in place. OS of PV pts was estimated using Kaplan-Meier (KM) analysis. Propensity Score Matching was used to identify a subgroup of SEER pts (PV-SEER) with similar age, sex, and race demographics to PV-WCM for comparison of OS. Cox proportional hazards was used for multivariable analysis (MVA) of survival. Actuarial survival of the US population matched by age, sex, race, and year-of-birth was obtained from US Census. Observed OS was compared to actuarial OS using one-sided log-rank test.Results: We identified 470 PV-WCM and 16,492 PV pts from SEER. The median OS of the PV-WCM cohort and the SEER population was 26.6 and 12.8 years, respectively, but the groups differed by age and sex (Fig 1A). PV-WCM and matched PV-SEER, however, were well balanced by age, sex, and race (standardized mean difference <0.1). The median OS of PV-WCM was 10.8 years longer than PV-SEER (p<0.001, Fig 1B). MVA confirmed 65% lower mortality in the PV-WCM group relative to the PV-SEER population (p<0.001, Fig 1C). PV-SEER OS was shorter than actuarial OS of the US population (mortality HR 2.47, p<0.001), whereas PV-WCM OS was not significantly different than expected (mortality HR 1.15, p=0.136). Excess late mortality was observed in PV-WCM after 17 years, potentially due to increased incidence of MF progression, affecting almost 50% of PV pts after 25 years (Fig 1D).Discussion: These data point to the success of attentive management with aspirin, targeted PHL, and cytoreductive therapy at a specialty center in reducing the mortality of PV, owing in part, to ELN and NCCN treatment recommendations for preventing fatal thrombosis. Although excess thrombosis was not eliminated in PV-WCM pts, the incidence rate was low after the first two years (Fig 1D). In contrast, the incidence of MF progression increased, potentially contributing to excess late mortality. Current PV risk stratification and treatment recommendations neither predict nor aim to mitigate progression to MF, and do not satisfy the needs of younger, low-risk pts who are often symptomatic and harbor a lifetime threat of progression (Fig 1E). The recent Low-PV study showed that low-risk pts may also benefit from cytoreductive therapy with an agent such as interferon-alpha (rIFNα) over PHL alone (Barbui et al. Lancet Hematol 2021). PV-WCM pts treated with rIFNα (n=137), previously shown to have improved MF-free survival and OS (Abu-Zeinah et al. Leukemia 2021), had an OS similar to the matched US population (p=0.33, Fig 1F); whereas pts not treated with rIFNα had modestly shortened OS (HR 1.26, p=0.03). Because PV progression is among the leading causes of late morbidity and mortality, and available therapy can mitigate this, it may be time to reconsider and improve upon PV risk stratification and treatment.Conclusion: This large population-based and single center study shows that although PV survival remains shortened, normal life expectancy for PV pts is possible with available care. The discrepancy between survival in the community and at a specialized center highlights the need for greater community outreach, health equity, and education regarding treatment standards for PV. Hopefully, this study motivates development of new PV risk stratification and treatment recommendations that focus not only on hematologic control and thrombosis prevention, but also on preventing MF progression, improving OS, and restoring normal life expectancy. [Display omitted] DisclosuresAbu-Zeinah: PharmaEssentia: Membership on an entity's Board of Directors or advisory committees. Silver: Abbvie: Consultancy; PharamEssentia: Consultancy, Speakers Bureau. Scandura: CR&T (Foudation): Research Funding; European Leukemia net: Honoraria, Other: travel fees ; MPN-RF (Foundation): Research Funding; Constellation: Research Funding; Abbvie: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. OffLabel Disclosure:Interferon-alpha is used off label in the treatment of polycythemia vera.

A Temporal and Multinational Assessment of Acute Myeloid Leukemia (AML) Cancer Incidence, Survival, and Disease Burden

Introduction: As US and global populations continue to age, a corresponding rise in incidence of age-associated cancers including acute myeloid leukemia (AML) remains persistent (Kraywinkel & Spix, 2017; Reedijk et al., 2019; Siegel, Miller, & Jemal, 2020; Yi et al., 2020). Characterizing the current and future epidemiologic impact of AML is critical for assessing therapeutic demand and informing allocation of health care resources. However, very few recent publications have reported international estimates of AML diagnoses and none have projected future cases. In this study, we estimate current and future incidence of AML (2021-2040) in 29 countries from 4 continents. Next, we describe survival trends in the US over the last 4 decades and by age at diagnosis. Finally, we assess the societal burden of AML in 29 countries by calculating average years of life lost (AYLL) due to AML-related death in 2021.Methods: Incidence rates were age-standardized to the World Health Organization (WHO) New World Population. For these calculations, age-specific rates were estimated using data from the International Agency for Research on Cancer's (IARC's) Cancer Incidence in Five Continents Volume XI (CI5-XI) database for 28 countries outside of the US (11 in Asia, 9 in Europe, 6 in the Americas, and 2 in Oceania). For US rates, data from National Cancer Institute's (NCI's) Surveillance, Epidemiology, and End Results (SEER) 21 were assessed. To project crude (unstandardized) incidence rates and the number of incident cases forward to 2040, age- and sex-specific incidence rates were applied to each country's age- and sex-specific population projections using data from the United Nation's World Population Prospects. Five-year Kaplan-Meier survival curves reflecting observed survival by year of diagnosis and age at diagnosis were derived using SEER 9 and SEER 18 databases, respectively. AYLL calculations utilized US age- and sex-specific mortality rates from the National Center for Healthcare Statistics (NCHS); non-US mortality rates were estimated by applying the incidence to mortality ratio from the US. The WHO's Global Health Observatory provided life expectancy data to estimate AYLL for each of the 29 countries.Results: Age-standardized incidence rates ranged from 0.70 cases per 100,000 persons (Costa Rica) to 3.23 cases per 100,000 persons (United States) with a median of 2.28 cases per 100,000 persons (interquartile range (IQR): 1.61-2.71). Projected growth in incident AML cases varied from +3% (Russia) to +51% (Colombia) over the next 20 years; median growth in incident AML cases was +32% (IQR: 27%-43%). AML survival after diagnosis more than doubled in the US over the last 40 years with the following median survival times observed: 4 months (1978-1987) vs. 11 months (2008-2017). Five-year survival in the US was likewise extended during this period: 8% (1978-1987) to 28% (2008-2017). Disparate survival was observed when patients were stratified by age. During the period 2008 to 2017, US cases diagnosed 60 years and older (which represented 67% of all new AML cases) had a median survival of 3 months with a five-year observed survival (OS) of 10%; this was far lower than their younger counterparts (< 60 years) who experienced a median survival of 60 months and a 5-year OS of 51%. The multinational assessment of societal disease burden due to premature AML death estimated a median AYLL of 19.6 years (IQR: 17.3-23.3 years).Conclusion: This epidemiologic assessment of AML disease burden projected significant growth in new AML diagnoses over the next 20 years. In fact, we estimated the growth of AML cases to outpace population growth in most countries - a phenomenon that can be attributed in part to the disproportionate increase in the elderly population where AML is most persistent. Our analysis additionally confirmed differences in survival by age with younger patients exhibiting more favorable survival compared to older adults. This stark contrast in prognosis may be partly explained by the current treatment approach using stem cell transplant for younger, fit patients and the limited therapeutic options among older patients. Despite the noted improvements in survival over the last 4 decades, median survival among all AML patients remains poor highlighting the unmet medical need for novel therapeutic approaches. [Display omitted] DisclosuresAnderson: Amgen: Current Employment, Current equity holder in publicly-traded company. Girguis: Amgen: Current Employment, Current equity holder in publicly-traded company. Kim: Amgen: Current Employment, Current equity holder in publicly-traded company. Shewale: Amgen: Current Employment, Current equity holder in publicly-traded company. Braunlin: Amgen: Current Employment, Current equity holder in publicly-traded company. Werther: Amgen: Current Employment, Current equity holder in publicly-traded company. Hidalgo-Lopez: Amgen Inc.: Current Employment, Current holder of stock options in a privately-held company. Zaman: Amgen: Current Employment, Current equity holder in publicly-traded company. Kim: Amgen: Current Employment, Current equity holder in publicly-traded company.

Secondary Malignancies in Diffuse Large B-cell Lymphoma Survivors With 40 Years of Follow-Up: Influence of Chemotherapy and Radiotherapy

Survivors of non-Hodgkin lymphoma (NHL) are more likely to develop secondary malignancies (SM). We quantified this risk in survivors of diffuse large B-cell lymphoma (DLBCL), the most common subtype of NHL, and evaluated differences in risk by treatment modality.Standardized incidence ratios (SIR, observed-to-expected [O/E] ratio), which compare SM risk in patients to the endemic population, and absolute excess risk of SM were assessed in 45,946 patients diagnosed with DLBCL as a first malignancy between 1975 and 2016 in the National Cancer Institute's Surveillance, Epidemiology, and End Results Program. Non-melanoma skin cancers were not counted as SM. We subsequently stratified these risks by treatment modality.In all, 3,214 patients received radiotherapy alone (RT), 26,761 received chemotherapy alone (CT), 10,081 received chemoradiation (CRT), and 6,233 received no RT or CT. In total, 4,247 patients (9%) developed at least one SM, and a total of 4,862 SM developed amongst these patients (O/E 1.23, 95% Confidence Interval [CI] 1.19-1.26 P < .05). This increased risk of SM persisted regardless of treatment modality, including RT alone (O/E 1.15, 95% CI 1.03-1.27 P < .05), CT alone (O/E 1.24, 95% CI 1.2-1.29 P < .05), and CRT (O/E 1.30, 95% CI 1.23-1.38 P < .05). Overall, DLBCL survivors were at increased risk of developing leukemia, Hodgkin lymphoma, Kaposi sarcoma, head and neck, breast, thyroid, renal, bladder, bone, soft tissue, lung, liver/biliary, colon, and anal cancers compared to the endemic population (P < .05). Patients treated with any RT, including RT alone and CRT, had a similar SM risk when compared to those who did not receive any RT [O/E 1.26 (95% CI 1.2-1.33) vs. O/E 1.21 (95% CI 1.17-1.25) respectively P < .05]. Patients treated with RT had a higher risk of developing breast cancer than unirradiated patients [O/E 1.20 (95% CI 1.02-1.39) vs. O/E .86 (95% CI .76-.97) respectively P < .05]. Patients treated with any CT, including CT and CRT, had increased SM rates compared with those who did not receive CT [O/E 1.26 (95% CI 1.22-1.30) vs O/E 1.11 (95% CI 1.04-1.18) respectively P < .05]. Patients treated with CT had a higher risk of developing leukemia than those who did not receive chemotherapy [O/E 2.33 (95% CI 2.03-2.67) vs. O/E 1.4 (95% CI .98-1.93) respectively P < .05].This is the largest study to examine SM risk in patients with DLBCL based on treatment modality. The risk of SM was increased for DLBCL survivors regardless of treatment modality. Different treatment modalities were associated with unique SM risks, however, the magnitude of SM risk was similar with RT and CT. These data may help guide screening protocols for patients following treatment of DLBCL.

Risk of Secondary Breast Cancer in Female Non-Hodgkin Lymphoma Survivors: 40 Years of Follow-Up Assessed by Treatment Modality

Survivors of non-Hodgkin lymphoma (NHL) are at increased risk of treatment associated secondary malignancies. We quantified the risk of developing a secondary breast cancer (SBC) in female NHL survivors with over 40 years of follow-up, and evaluated differences in risk by treatment modality.Standardized incidence ratios (SIR, observed-to-expected [O/E] ratio), which accounts for patient years at risk, and absolute excess risk of SBC were assessed in 65,123 female patients diagnosed with NHL as a first malignancy between 1975 and 2016 in the National Cancer Institute's Surveillance, Epidemiology, and End Results Program. Only invasive disease was considered SBC, ductal carcinoma in situ was excluded. Follow up was available through 2016. SIRs were also evaluated for patients stratified by age at and latency from diagnosis.In all, 7,010 (10.8%) patients received radiotherapy alone (RT), 30,393 (46.7%) received chemotherapy alone (CT), and 7,845 (12.0%) received chemotherapy and radiation (CRT). In total, there were 1,480 female SBCs with NHL survivors having a lower incidence of SBC than the endemic rate (O/E 0.91, 95 % CI 0.87-0.96, P < 0.05. Patients treated with RT were at a higher risk of SBC than those who did not receive RT (O/E 1.02, 95% CI 0.93-1.12 vs O/E 0.87, 95% CI 0.82-0.93 respectively; P < 0.05). When stratified by treatment groups (No therapy, RT alone, CT alone, and CRT) there was a significantly higher risk of SBC in the CRT group than any other treatment group (O/E 1.18, 95% CI 1.03-1.34, P < 0.05). When patients were stratified by age at diagnosis, there was a significantly increased risk of SBC in patients who were diagnosed at age < 25 years irrespective of RT status with O/E ratios of 3.07 and 3.97 in the RT and no RT groups, respectively. However, there was no significant difference between these two treatment groups. This effect decreased with increasing age at diagnosis of NHL. The risk of developing SBC was significantly higher at > 10 years from NHL diagnosis (O/E 1.12) compared to < 10 years (O/E 0.82).This is the largest study to examine SBC risk in patients with NHL. These results demonstrate that survivors of NHL have a lower incidence of invasive breast cancer compared to the general population. Patients treated with RT did have an increased risk over those with no RT; however, this did not exceed endemic breast cancer rate. The use of CRT and time > 10 years from NHL diagnosis were associated with higher risk of developing an SBC. Importantly, women diagnosed with NHL < 25 yrs of age had a higher rate of SBCs regardless of RT use. These results may help inform breast cancer screening protocols for women with a history of NHL.

Risk of Secondary Malignancies in Ovarian Cancer Survivors: 54,305 Patients Analyzed With 40 Years of Follow-up

Survivors of ovarian cancer are at risk of developing a secondary malignancy (SM). We sought to evaluate the risk of developing SM, stratified by treatment modality and site of SM.Standardized incidence ratios (SIR, observed-to-expected [O/E] ratio) and absolute excess risk of SM, were assessed in 54,305 patients diagnosed with ovarian cancer as a first malignancy between 1975 and 2016 in the National Cancer Institute's Surveillance, Epidemiology, and End Results Program. Follow-up was available through 2016. Importantly, the SIRs take into account age at diagnosis, race, year of diagnosis and patient-years at risk. Non-melanoma skin cancers were not counted as SM. SIRs were also evaluated for patients according to latency from their initial diagnosis.Of 54,305 patients diagnosed with ovarian cancer, 1,010 received radiation therapy (RT) alone, 35,842 received chemotherapy (CT) alone, and 1,035 were treated with chemotherapy and radiation (CRT). 16,418 patients received neither CT or RT. Overall, 3,981 patients (7.3%) developed SM, which was more than the endemic rate [O/E 1.13 (95% CI 1.09- 1.16); P < 0.05]. Patients who received any RT (RT+CRT) had an increased risk of SM compared to those who didn't [O/E 1.42 (95% CI 1.24- 1.61) vs O/E 1.11 (95% CI 1.08- 1.15), respectively; P < 0.05] which included an excess risk of 4.82 bladder cancers per 10,000 patient-years at risk. Patients who received any CT (CT+CRT) had a similar risk of developing SM compared to those not treated with CT [O/E 1.11 (95% CI 1.07- 1.16) vs O/E 1.14 (95% CI 1.09- 1.12), respectively; P < 0.05]. Those who received any CT had an excess risk of 4.1 cases of leukemia per 10,000 patient-years at risk compared to those who did not receive CT. When stratified into treatment groups (No CT/RT, RT alone, CT alone, CRT), the CRT group had higher rates of SM than the no CT/RT and CT groups [O/E 1.56 (95% CI 1.25- 1.93)]. There was no statistically significant difference in the SM rate between the RT group and no CT/RT group [O/E 1.35 (95% CI 1.14- 1.57) vs O/E 1.13 (95% CI 1.07- 1.18), respectively]. The CT and CRT groups had an increased risk of leukemia compared to the no CT/RT group (P < 0.05). The CT only group had a lower risk of developing lung cancer than the no CT/RT group (P < 0.05). The excess risk of developing a solid tumor was greatest at latencies of 10-20 years. The excess risk of developing leukemia was greatest at latencies up to 10 years.This is the largest study to examine the risk of SM in patients with ovarian cancer and has the longest follow-up. The risk of SM was increased after having ovarian cancer and varied with treatment modality. Patients treated with any RT had higher rates of SM than those not treated with RT. Importantly, CT did not result in an increased excess risk of solid tumor SM, only in an increased excess risk of leukemia. These results may help inform SM screening protocols for women with ovarian cancer.A.C. Casper: None. M.W. Parsons: None. J. Chipman: None. L.M. Burt: ARRO executive committee. G. Suneja: Research Grant; National Institutes of Health. Honoraria; National Comprehensive Cancer Network. Travel Expenses; National Comprehensive Cancer Network, Radiation Oncology Institute, American Board of Radiology; Radiation Oncology Institute, National Comprehensive Cancer Network, ASTRO.K.A. Maurer: None. D.K. Gaffney: Research Grant; NCI. Consultant; NCI. run meetings; NCI.

Medical Service Use and Charges for Cancer Care in 2018 for Privately Insured Patients Younger Than 65 Years in the US

Currently, there are limited published data regarding resource use and spending on cancer care in the US. To characterize the most frequent medical services provided and the associated spending for privately insured patients with cancer in the US. This cohort study used data from the MarketScan database for the calendar year 2018 from a sample of 27.1 million privately insured individuals, including patients with a diagnosis of the 15 most prevalent cancers, predominantly from large insurers and self-insured employers. Overall societal health care spending was estimated for each cancer type by multiplying the mean total spending per patient (estimated from MarketScan) by the number of privately insured patients living with that cancer in 2018, as reported by the National Cancer Institute's Surveillance, Epidemiology, and End Results program. Analyses were performed from February 1, 2018, to July 8, 2021. Evaluation and management as prescribed by treating care team. Current Procedural Terminology and Healthcare Common Procedure Coding System codes based on cancer diagnosis code. The estimated cost of cancer care in 2018 for 402 115 patients with the 15 most prevalent cancer types was approximately $156.2 billion for privately insured adults younger than 65 years in the US. There were a total of 38.4 million documented procedure codes for 15 cancers in the MarketScan database, totaling $10.8 billion. Patients with breast cancer contributed the greatest total number of services (10.9 million [28.4%]), followed by those with colorectal cancer (3.9 million [10.2%]) and prostate cancer (3.6 million [9.4%]). Pathology and laboratory tests contributed the highest number of services performed (11.7 million [30.5%]), followed by medical services (6.3 million [16.4%]) and medical supplies and nonphysician services (6.1 million [15.9%]). The costliest cancers were those of the breast ($3.4 billion [31.5%]), followed by lung ($1.1 billion [10.2%]) and colorectum ($1.1 billion [10.2%]). Medical supplies and nonphysician services contributed the highest total spent ($4.0 billion [37.0%]), followed by radiology ($2.1 billion [19.4%]) and surgery ($1.8 billion [16.7%]). This analysis suggests that patients with breast, colorectal, and prostate cancers had the greatest number of services performed, particularly for pathology and laboratory tests, whereas patients with breast, lung, lymphoma, and colorectal cancer incurred the greatest costs, particularly for medical supplies and nonphysician services. The cost of cancer care in 2018 for the 15 most prevalent cancer types was estimated to be approximately $156.2 billion for privately insured adults younger than 65 years in the US.

Survival outcomes and prognostic factors of papillary serous adenocarcinoma and papillary squamous cell carcinoma of the uterine cervix

The aim of this retrospective population-based study was to investigate the survival outcomes and prognostic factors of patients with the two cervical carcinomas. A cohort of patients diagnosed with papillary serous adenocarcinoma of the uterine cervix (PSAC) and papillary squamous cell carcinoma (PSCC) between 1973 and 2015 were drawn from the National Cancer Institute's Surveillance Epidemiology and End Results (SEER) database. Overall survival (OS) and cancer-specific survival (CSS) were estimated using the Kaplan–Meier method, and prognostic factors were assessed using Cox proportional hazards survival regression analysis. The 5-year and 10-year OS rates were 38.4 and 33.1% for PSAC and 64.6 and 50.8% for PSCC, respectively. The 2-year and 5-year CSS rates were 60.6 and 45.9% for PSAC and 79.6 and 69.0% for PSCC, respectively. Patients with PSCC survive longer than PSAC patients and have other well-described prognostic factors for improved survival rates, including an early cancer stage, a younger patient age and standardised surgery. Impact statement What is already known on this subject? Papillary serous adenocarcinoma of the uterine cervix (PSAC) and papillary squamous cell carcinoma (PSCC)are both very rare subtypes of cervical carcinomas. What do the results of this study add? This retrospective population-based analysis has evaluated the survival outcomes and prognostic indicators of patients with PSAC and PSCC. What the implications are of these findings for clinical practice and/or further research? Knowing the survival outcomes and prognostic indicators of PSAC and PSCC patients, we can better follow up patients.

Evaluation of Gender Inequity in Thyroid Cancer Diagnosis

Thyroid cancer is more common in women than in men, but the associated causes of these differences are not fully understood. To compare sex-specific thyroid cancer rates in the US to the prevalence of subclinical thyroid cancer at autopsy. Data on thyroid cancer incidence and mortality by sex among US adults (≥18 years) were extracted from the National Cancer Institute's Surveillance, Epidemiology, and End Results Program (SEER) data for 1975 to 2017. Embase, PubMed, and Web of Science databases were searched for studies on the prevalence of subclinical thyroid cancer at autopsy of men and women, from inception to May 31, 2021. The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline was used to perform a systematic search for articles reporting the prevalence of subclinical thyroid cancer in autopsy results of both women and men. Of 101 studies identified, 8 studies containing 12 data sets met inclusion criteria; ie, they examined the whole thyroid gland, stated the number of thyroids examined, and reported results by sex. Excluded studies reported thyroid cancer in Japan after the atomic bombs or Chernobyl after the nuclear disaster; did not examine the whole thyroid gland or had incomplete information on thyroid examination methods; or did not report rates by sex. Thyroid cancer incidence and mortality data by sex, histologic type, and tumor size were extracted from SEER. The inverse variance heterogeneity model was used to meta-analyze the prevalence and the odds ratio of subclinical thyroid cancer by sex from 8 studies (12 data sets) on thyroid cancer prevalence in autopsy results. Incidence and mortality of thyroid cancer, by histologic type and tumor size; prevalence of thyroid cancer in autopsy results. In 2017, 90% of thyroid cancers diagnosed were papillary thyroid cancer (PTC) and in 2013 to 2017, the women to men incidence ratio for small (≤2 cm) PTC was 4.39:1. The incidence ratio approached 1:1 as cancer type lethality increased. The ratio of thyroid cancer mortality by gender was 1.02:1 and remained stable from 1992 to 2017. Results of the meta-analysis showed that the pooled autopsy prevalence of subclinical PTC was 14% in women (95% CI, 8%-20%) and 11% in men (95% CI, 5%-18%). The pooled odds ratio of subclinical PTC in women compared with men was 1.07 (95% CI, 0.80-1.42). This cohort study and meta-analysis found that the belief that women get thyroid cancer more often than men is an oversimplification. The gender disparity is mostly confined to the detection of small subclinical PTCs, which are equally common in both sexes at autopsy but identified during life much more often in women than men. As the lethality of the cancer type increases, the ratio of detection by gender approaches 1:1. This phenomenon may be associated with gender differences in health care utilization and patterns of clinical thinking and can harm both women, who are subject to overdetection, and men, who may be at risk of underdetection.

Prognostic factors, disparity, and equity variables impacting prognosis in bone sarcomas of the hand: SEER database review.

Primary sarcomas originating from the bones of hand and wrist are rare but carry a significant burden of morbidity. National Cancer Institute's Surveillance, Epidemiology and End Result database from 1975 to 2017 was queried to report incidence and survival data in 237 patients in the United States. Kaplan-Meier and Cox regression were used to determine the prognostic factors affecting survival. χ2 test was used to assess the correlation. Incidence of hand and wrist sarcoma was 0.017 per 100 000 persons in 2017 and has not significantly changed since 1975 (p > 0.05). Disease-specific 5-year and 10-year survival for the entire cohort was 90% and 84%, respectively. On multivariate analysis race "others," histology other than "osteosarcoma," "undifferentiated" grade, and size "≥6 cm" were predictors of worse disease-specific survival. Cross-tabulation of race with other significant prognostic factors on univariate analysis revealed a significant correlation of race with every other significant prognostic factor except for grade. The current study is an analysis of a population-based registry reporting incidence and survival data for patients with sarcoma of hand and wrist. Independent prognostic factors include race, histology, grade, and size. There is a lack of improvement in survival over the last four decades.