One Hundred Years After “Carcinoid”: Epidemiology of and Prognostic Factors for Neuroendocrine Tumors in 35,825 Cases in the United States

2008 ◽  
Vol 26 (18) ◽  
pp. 3063-3072 ◽  
Author(s):  
James C. Yao ◽  
Manal Hassan ◽  
Alexandria Phan ◽  
Cecile Dagohoy ◽  
Colleen Leary ◽  
...  

Purpose Neuroendocrine tumors (NETs) are considered rare tumors and can produce a variety of hormones. In this study, we examined the epidemiology of and prognostic factors for NETs, because a thorough examination of neither had previously been performed. Methods The Surveillance, Epidemiology, and End Results (SEER) Program registries were searched to identify NET cases from 1973 to 2004. Associated population data were used for incidence and prevalence analyses. Results We identified 35,618 patients with NETs. We observed a significant increase in the reported annual age-adjusted incidence of NETs from 1973 (1.09/100,000) to 2004 (5.25/100,000). Using the SEER 9 registry data, we estimated the 29-year limited-duration prevalence of NETs on January 1, 2004, to be 9,263. Also, the estimated 29-year limited-duration prevalence in the United States on that date was 103,312 cases (35/100,000). The most common primary tumor site varied by race, with the lung being the most common in white patients, and the rectum being the most common in Asian/Pacific Islander, American Indian/Alaskan Native, and African American patients. Additionally, survival duration varied by histologic grade. In multivariate analysis of patients with well-differentiated to moderately differentiated NETs, disease stage, primary tumor site, histologic grade, sex, race, age, and year of diagnosis were predictors of outcome (P < .001). Conclusion We observed increased reported incidence of NETs and increased survival durations over time, suggesting that NETs are more prevalent than previously reported. Clinicians need to be become familiar with the natural history and patterns of disease progression, which are characteristic of these tumors.

1999 ◽  
Vol 17 (12) ◽  
pp. 3697-3705 ◽  
Author(s):  
Sheri L. Spunt ◽  
Catherine A. Poquette ◽  
Yasmeen S. Hurt ◽  
Alvida M. Cain ◽  
Bhaskar N. Rao ◽  
...  

PURPOSE: The rarity and heterogeneity of pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) has precluded meaningful analysis of prognostic factors associated with surgically resected disease. To define a population of patients at high risk of treatment failure who might benefit from adjuvant therapies, we evaluated the relationship between various clinicopathologic factors and clinical outcome of children and adolescents with resected NRSTS over a 27-year period at our institution. PATIENTS AND METHODS: We analyzed the records of 121 consecutive patients with NRSTS who underwent surgical resection between August 1969 and December 1996. Demographic data, tumor characteristics, treatment, and outcomes were recorded. Univariate and multivariate analyses of prognostic factors for survival, event-free survival (EFS), and local and distant recurrence were performed. RESULTS: At a median follow-up of 9.2 years, 5-year survival and EFS rates for the entire cohort were 89% ± 3% and 77% ± 4%, respectively. In univariate models, positive surgical margins (P = .004), tumor size ≥ 5 cm (P < .001), invasiveness (P = .002), high grade (P = .028), and intra-abdominal primary tumor site (P = .055) adversely affected EFS. All of these factors except invasiveness remained prognostic of EFS and survival in multivariate models. Positive surgical margins (P = .003), intra-abdominal primary tumor site (P = .028), and the omission of radiation therapy (P = .043) predicted local recurrence, whereas tumor size ≥ 5 cm (P < .001), invasiveness (P < .001), and high grade (P = .004) predicted distant recurrence. CONCLUSION: In this largest single-institution analysis of pediatric patients with surgically resected NRSTS, we identified clinicopathologic features predictive of poor outcome. These variables should be prospectively evaluated as risk-adapted therapies are developed.


2021 ◽  
Author(s):  
Suayib Yalcin ◽  
Hande Turna ◽  
Ilan Shimon ◽  
Yi-Ming Shyr ◽  
Yan-Shen Shan ◽  
...  

Abstract Background: This 8-year observational, multicenter, registry study was undertaken to assess the incidence and regional trends in diagnosis, clinical management, and outcome assessments of patients with gastroenteropancreatic neuroendocrine tumors (GEP-NET) in the Asia-Pacific region, the Middle East, Turkey, and South Africa.Materials and Methods: Patient data including demographics, primary tumor site, tumor differentiation, diagnosis, treatment modalities, outcomes were collected via a web-based data entry portal.Results: Overall, 974 patients were enrolled, and 951 of which were evaluated (median age: 54 years). Pancreas was the most commonly reported primary tumor site. Non‑functioning and functioning (most common: carcinoid) tumors were reported in 61.9% and 27.9% of patients, respectively. Abdominal pain was the most commonly reported symptom. Surgery was the most-commonly employed initial treatment, followed by treatment with somatostatin analogs; >80% of patients had open surgery and R0 resection. Satisfactory response to the initial treatment modality was reported for 56.4% of patients. Overall survival rate was 96.3% at 6 months since diagnosis and 72.9% at 60 months. Median progression-free survival was 60.9 months since first diagnosis and 60.5 months after first treatment, with a 6-month progression-free survival of 95.0%. Conclusion: The GEP-NET registry provides important information relating to the diagnosis and treatment of patients in the participating countries. This analysis highlighted the need for improvements in the clinical practice to ensure better evaluation and treatment management.


Surgery ◽  
2016 ◽  
Vol 159 (1) ◽  
pp. 311-319 ◽  
Author(s):  
Xavier M. Keutgen ◽  
Naris Nilubol ◽  
Joanne Glanville ◽  
Samira M. Sadowski ◽  
David J. Liewehr ◽  
...  

2021 ◽  
Vol 10 (2) ◽  
pp. 257
Author(s):  
Francesco Panzuto

Neuroendocrine tumors (NET) are rare and heterogeneous diseases, whose prognosis is affected by several factors including the primary tumor site, grading, somatostatin receptor expression, and disease staging [...]


JMIR Cancer ◽  
10.2196/12849 ◽  
2019 ◽  
Vol 5 (2) ◽  
pp. e12849
Author(s):  
Jocelyn Reeders ◽  
Vivek Ashoka Menon ◽  
Anita Mani ◽  
Mathew George

Background Neuroendocrine tumors (NETs) are a heterogeneous group of malignancies with varying and often indolent clinicobiological characteristics according to their primary location. NETs can affect any organ and hence present with nonspecific symptoms that can lead to a delay in diagnosis. The incidence of NETs is increasing in Australia; data regarding characteristics of NETs were collected from the cancer registry of Hunter New England, Australia. Objective This study aimed to explore the clinical profiles and treatment and survival outcomes of patients with well-differentiated NETs in an Australian population. Methods We reviewed the data of all adult patients who received the diagnosis of NET between 2008 and 2013. The clinicopathological, treatment, and follow-up data were extracted from the local Cancer Clinical Registry. We also recorded the level of remoteness for each patient by matching the patient’s residential postcode to the corresponding Australian Bureau of Statistics 2011 remoteness area category. Univariate analysis was used to find the factors associated with NET-related mortality. Survival analysis was computed. Results Data from 96 patients were included in the study (men: 37/96, 38.5%, and women: 59/96, 61.5%). The median age at diagnosis was approximately 63 years. A higher proportion of patients lived in remote/rural areas (50/96, 52.1%) compared with those living in city/metropolitan regions (46/96, 47.9%). The most common primary tumor site was the gastroenteropancreatic tract, followed by the lung. The factors significantly associated with NET-related mortality were age, primary tumor site, surgical resection status, tumor grade, and clinical stage of the patient. At 5 years, the overall survival rate was found to be 62%, and the disease-free survival rate was 56.5%. Conclusions Older age, advanced unresectable tumors, evidence of metastasis, and higher-grade tumors were associated with poorer outcomes. Lung tumors had a higher risk of NET-related mortality compared with other sites.


2008 ◽  
Vol 109 (Supplement) ◽  
pp. 77-86 ◽  
Author(s):  
Daniel W. Golden ◽  
Kathleen R. Lamborn ◽  
Michael W. McDermott ◽  
Sandeep Kunwar ◽  
William M. Wara ◽  
...  

Object The authors conducted a study to determine whether prognostic factors and the applicability of prognostic systems vary by primary tumor site in patients treated with radiosurgery for brain metastases. Methods The authors evaluated data obtained in patients who underwent radiosurgery with or without whole-brain radiotherapy (WBRT) from 1991 to 2005 for newly diagnosed brain metastases. Four groups were analyzed: 1) all primary sites combined, 2) breast, 3) lung, and 4) melanoma primary sites. Kaplan–Meier, log-rank, Cox proportional hazard uni- and multivariate analysis, and recursive partitioning analysis (RPA) were used to assess prognostic factors and 4 prognostic systems: Radiation Therapy Oncology Group (RTOG) RPA, Graded Prognostic Assessment (GPA), basic score for brain metastases (BSBM), and the newly proposed Golden grading system (GGS). The GGS divides patients into 4 prognostic groups by age ≥ 65 years, Karnofsky Performance Scale (KPS) score < 70, and known presence of extracranial metastases. Results Data acquired in 479 newly diagnosed patients with 1664 lesions were analyzed. The median survival time from diagnosis of brain metastases was 12.1 months; the median follow-up was 25.4 months in 73 patients who were censored. Survival and prognostic factors were equivalent for 369 patients treated with radiosurgery compared with 110 patients treated with radiosurgery and WBRT, so these subsets were combined. Multivariate analysis of all primary sites combined demonstrated age < 65 years, KPS score ≥ 70, no known extracranial metastases, and ≤ 3 brain metastases were associated with longer survival, and primary tumor control was not. In subgroup multivariate analysis of patients with breast, lung, or melanoma primaries, favorable factors included only primary tumor control in 87 patients with breast primary; age < 65 years, no known extracranial metastases, and ≤ 3 brain metastases in 169 patients with lung primary; and KPS ≥ 70 years, primary tumor control, and ≤ 3 brain metastases in 137 patients with melanoma primary. The median survival for ≤ 3 versus > 3 metastases was 15.6 and 16.9 months, respectively, for breast, 16.5 and 11.3 months for lung, and 9.0 and 5.7 months for melanoma. Analysis of the 4 prognostic systems (RTOG RPA, BSBM, GPA, and GGS) showed that each prognostic system's clinical applicability varied depending on primary tumor site. The RPA confirmed that GGS and primary tumor site are significant variables for prognosis. Conclusions Favorable prognostic factors for patients with newly diagnosed brain metastases treated with radiosurgery vary by primary site. The 4 prognostic grading systems analyzed were applicable to different primary sites depending on which prognostic factors each individual system incorporated. Therefore, the authors recommend further development and use of primary-specific prognostic systems.


2018 ◽  
Author(s):  
Jocelyn Reeders ◽  
Vivek Ashoka Menon ◽  
Anita Mani ◽  
Mathew George

BACKGROUND Neuroendocrine tumors (NETs) are a heterogeneous group of malignancies with varying and often indolent clinicobiological characteristics according to their primary location. NETs can affect any organ and hence present with nonspecific symptoms that can lead to a delay in diagnosis. The incidence of NETs is increasing in Australia; data regarding characteristics of NETs were collected from the cancer registry of Hunter New England, Australia. OBJECTIVE This study aimed to explore the clinical profiles and treatment and survival outcomes of patients with well-differentiated NETs in an Australian population. METHODS We reviewed the data of all adult patients who received the diagnosis of NET between 2008 and 2013. The clinicopathological, treatment, and follow-up data were extracted from the local Cancer Clinical Registry. We also recorded the level of remoteness for each patient by matching the patient’s residential postcode to the corresponding Australian Bureau of Statistics 2011 remoteness area category. Univariate analysis was used to find the factors associated with NET-related mortality. Survival analysis was computed. RESULTS Data from 96 patients were included in the study (men: 37/96, 38.5%, and women: 59/96, 61.5%). The median age at diagnosis was approximately 63 years. A higher proportion of patients lived in remote/rural areas (50/96, 52.1%) compared with those living in city/metropolitan regions (46/96, 47.9%). The most common primary tumor site was the gastroenteropancreatic tract, followed by the lung. The factors significantly associated with NET-related mortality were age, primary tumor site, surgical resection status, tumor grade, and clinical stage of the patient. At 5 years, the overall survival rate was found to be 62%, and the disease-free survival rate was 56.5%. CONCLUSIONS Older age, advanced unresectable tumors, evidence of metastasis, and higher-grade tumors were associated with poorer outcomes. Lung tumors had a higher risk of NET-related mortality compared with other sites.


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