scholarly journals Clinical Profiles and Survival Outcomes of Patients With Well-Differentiated Neuroendocrine Tumors at a Health Network in New South Wales, Australia: Retrospective Study

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.

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.


2011 ◽  
Vol 29 (4_suppl) ◽  
pp. 293-293
Author(s):  
T. Terashima ◽  
C. Morizane ◽  
H. Ueno ◽  
M. Ikeda ◽  
Y. Saito ◽  
...  

293 Background: Extrapulmonary neuroendocrine tumors (EPNET) are a rare and heterogeneous disease entity, and little information regarding these tumors is available. The aim of the present study was to clarify the clinical behavior, treatment strategy, and outcome of EPNET arising from various primary sites. Methods: We retrospectively reviewed the medical records of 337 patients with pathologically confirmed NET arising from extrapulmonary site between January 2000 and December 2008 at the National Cancer Center Hospital of Japan. Results: The major primary tumor site was the rectum followed by the pancreas and stomach. Among the gastrointestinal tract tumors, 113 patients had NET from the foregut, 92 patients from the hindgut, and only 4 patients from the midgut. The median patient age was 60 years, and 57% of the patients were male. The histologic grade was as follows: 39% were well-differentiated neuroendocrine tumors (WDNET); 20% were well-differentiated neuroendcrine carcinomas (WDNEC); 33% were poorly differentiated neuroendocrine carcinomas (PDNEC); and 8% were mixed exocrine-endocrine tumors (Mixed). Most NETs from the rectum or duodenum are WDNET, whereas the ratio of PDNEC was high among NET from the genital organs or esophagus. The treatment strategy was associated with the clinical stage or the primary organ; in particular, 94% of the localized rectal NET were resected endoscopically. The 2-year survival rate for all the patients were 70%. A multivariate analysis demonstrated that distant disease of clinical stage and PDNEC of histological grade had a stronger impact on a poor outcome than the primary tumor site. The 2-year survival rate among the patients with WDNET, WDNEC and PDNEC were 97%, 80% and 36%, respectively, and 94%, 72%, and 32% of the patients had localized, regional, and distant disease, respectively. Conclusions: Japanese patients with EPNET have different clinicopathological features such as the primary organ or treatment procedure compared with previous reports in Western countries. Identifying the precise clinical stage and histological grade of patients with NET is essential because these factors influence the patient outcome. No significant financial relationships to disclose.


2019 ◽  
Vol 105 (6) ◽  
pp. 524-528 ◽  
Author(s):  
Rosa Falcone ◽  
Adriana Romiti ◽  
Marco Filetti ◽  
Michela Roberto ◽  
Riccardo Righini ◽  
...  

Background: Because of a lack of large-scale prospective studies there is no clear indication about the management of patients with small bowel adenocarcinoma (SBA). This study evaluated clinical outcome of patients diagnosed with SBA at our institution. Methods: Clinicopathologic features, treatments, and clinical outcome of patients diagnosed with SBA between 2006 and 2017 were retrospectively analyzed. Median time of survival was calculated and compared using the log-rank test. Multivariate Cox regression was used to test independence of significant factors in univariate analysis. Results: Forty patients were included in the study; the majority (82.5%) had a tumor in the duodenum (including ampulla of Vater) and an early stage disease at the diagnosis. Median overall survival (OS) in the whole study population was 26.5 months. Patients with a tumor of the lower part of the small intestine (jejunum, ileum, and appendix) showed a better OS compared with that of patients with upper SBA (40 months vs 26 months, respectively; P=0.09). Primary tumor site and stage were independent predictors of OS. Conclusions: Our results suggest a prognostic role for the primary tumor site. This finding deserves to be further investigated to ensure better classification as well as more effective management strategies for SBA.


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.


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.


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 [...]


2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 4104-4104
Author(s):  
Paul Girot ◽  
Eric Baudin ◽  
Helene Senellart ◽  
Nadia Bouarioua ◽  
Olivia Hentic ◽  
...  

4104 Background: Oxaliplatin-based regimens have shown promising antitumor activity in digestive neuroendocrine tumors (NETs), however the available data are limited. Our aim was to assess the tumor response and survival in a large series of patients treated with oxaliplatin and 5-fluorouracil (FOLFOX) for advanced digestive NETs.Oxaliplatin-based regimens have shown promising antitumor activity in digestive neuroendocrine tumors (NETs), however the available data are limited. Our aim was to assess the tumor response and survival in a large series of patients treated with oxaliplatin and 5-fluorouracil (FOLFOX) for advanced digestive NETs. Methods: All patients with advanced well-differentiated digestive NETs treated with at least 3 cycles of FOLFOX between 2004 and 2018 in 12 centers of the French GTE, were retrospectively included. Best response according to the RECIST 1.1 criteria, progression-free survival (PFS) and overall survival (OS) were evaluated. The prognostic factors for PFS were investigated by multivariate analysis using a Cox proportional hazard model including variables with a p value ⩽ 0.20 in univariate analysis. Results: One hundred and forty-nine patients were included. Primary tumor location was pancreas (n = 88), small intestine (n = 37), stomach (n = 7), rectum (n = 4) and unknown without lung tumor at CT scan (n = 13). Partial response rate was of 31% for pancreatic NETs, 13% for small intestine NETs, 14% for gastric NETs, 25% for rectal NETs and 38% for unknown primary NETs. Median PFS were, respectively, 9, 9, 14, 4 and 6 months, and median OS were 30, 28, 31, 25 and 15 months. Significant poor prognostic factors for PFS after FOLFOX in digestive NETs were: progressive disease (HR = 2.5, p = 0.018), hepatic involvement > 50% (HR = 1.8, p = 0.009), prior targeted therapy (HR = 1.5, p = 0.048) and rectal primary tumor (HR = 4.2, p = 0.01). Among pancreatic NETs, the 9 insulinomas had a 22 months PFS versus 9 months for the others (p = 0.025), and serum glucose normalization was obtained in 8 out of 9 cases. Conclusions: FOLFOX has a promising clinical activity for gastroenteropancreatic NETs, especially in insulinomas.


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