OBJECTIVE: To compare the clinicopathologic features and survival outcomes of neuroendocrine tumor of the uterine corpus (NET-U) to endometrioid type endometrial carcinoma (EC). METHODS: From 1993 to 2012, the Surveillance, Epidemiology and End Results cancer registry was queried for women diagnosed with EC or NET-U. Data regarding stage, grade, presence of extra-uterine disease, lymph node metastasis, receipt of adjuvant radiation, surgical intervention and overall survival (OS) was extracted. Chi-square tests, t-tests and Kaplan Meir curves were used for statistical analysis. RESULTS: A total of 98,363 patients were identified: 98,245 with EC and 118 with NET-U. The mean age at diagnosis for EC was 61.7 years and 64.8 years for NET-U (p=0.01). NET-U cases were more likely to be poorly differentiated (97.0% vs. 15.6%; p≤0.01) and have nodal metastasis (56.4% vs. 11.1%; p≤0.01) when compared to EC. Presence of extrapelvic disease at the time of diagnosis was observed more frequently in NET-U compared to EC, 49.1% vs. 4.8%, respectively (odds ratio=18; 95% confidence interval=13.1–27.2; p≤0.01). Significant improvement in OS was observed in NET-U patient who received radiation (OS: 7.7 vs. 3.3 years; p≤0.01) or underwent surgical management (5.6 vs. 0.9 years; p≤0.01). The OS for EC was 14.4 vs. 4.6 years for NET-U (p≤0.01). CONCLUSION: NET-U represents an aggressive form of uterine malignancy. When compared to EC, patients with NET-U present at more advanced stage, have more frequent extra-uterine disease and lower OS.
Carcinoma, Endometrioid ; Diagnosis ; Endometrial Neoplasms ; Epidemiology ; Female ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Uterine Neoplasms

BACKGROUND: Neuroendocrine neoplasms (NENs) are rare tumors characterized by variable biology and delayed diagnosis. However, the nationwide epidemiology of NENs has never been reported in China. We aimed to estimate the incidence and survival statistics of NENs in China, in comparison to those in the United States during the same period. METHODS: Based on the data from 246 population-based cancer registries covering 272.5 million people of China, we calculated age-specific incidence on NENs in 2017 and multiplied by corresponding national population to estimate the nationwide incidence in China. The data of 22 population-based cancer registries were used to estimate the trends of NENs incidence from 2000 to 2017 through the Joinpoint regression model. We used the cohort approach to analyze the 5-year age-standardized relative survival by sex, age group, and urban-rural area between 2008 and 2013, based on data from 176 high-quality cancer registries. We used data from the Surveillance, Epidemiology, and End Results (SEER) 18 program to estimate the comparable incidence and survival of NENs in the United States. RESULTS: The overall age-standardized rate (ASR) of NENs incidence was lower in China (1.14 per 100,000) than in the United States (6.26 per 100,000). The most common primary sites were lungs, pancreas, stomach, and rectum in China. The ASRs of NENs incidence increased by 9.8% and 3.6% per year in China and the United States, respectively. The overall 5-year relative survival in China (36.2%) was lower than in the United States (63.9%). The 5-year relative survival was higher for female patients than male patients, and was higher in urban areas than in rural areas. CONCLUSIONS The disparities in burden of NENs persist across sex, area, age group, and site in China and the United States. These findings may provide a scientific basis on prevention and control of NENs in the two countries.
Humans ; Male ; Female ; United States/epidemiology* ; Incidence ; Neuroendocrine Tumors/pathology* ; Neoplasms/epidemiology* ; Registries ; Urban Population ; China/epidemiology*

Objective To investigate the impact of prior non-pancreatic cancer on the survival outcomes of patients with localized pancreatic neuroendocrine tumors (PanNETs). Methods We reviewed the Surveillance, Epidemiology, and End Results database and selected patients with localized PanNETs diagnosed between 1973 and 2015. We divided the patients into two groups according to the presence or absence of prior non-pancreatic malignancy. Before and after propensity score matching, we compared the clinicopathological characteristics and studied the overall survival and cancer-specific survival. Results A total of 357 (12.9%) of 2778 patients with localized PanNETs had prior cancer. A total of 1211 cases with only a localized PanNET and 133 cases with a localized PanNET and prior cancer had complete data and met the inclusion criteria of the current study. Patients with prior cancer were associated with advanced age (>65 years, 57.9% prior cancer
Aged ; Female ; Humans ; Male ; Multivariate Analysis ; Neoplasms, Second Primary ; Neuroendocrine Tumors/epidemiology* ; Pancreatic Neoplasms/epidemiology* ; Propensity Score

BACKGROUND/AIMS: Neuroendocrine tumors (NETs) may originate from heterogeneous neuroendocrine cells. The incidence is increasing worldwide, and World Health Organization (WHO) updated its classification in 2010. We investigated clinical characteristics of gastroenteropancreatic NETs in a single center. METHODS: Clinicopathologic characteristics of patients with pathologically confirmed gastroenteropancreatic NET in Seoul St. Mary Hospital from March 2009 to August 2011 were retrospectively analyzed. The grade and stage were determined according to WHO 2010 classification and TNM Staging System for Neuroendocrine Tumors (7th ed., 2010) of American Joint Committee on Cancer. RESULTS: One hundred and twenty-five patients (median age, 50; male, 61.3%) were analyzed. Among 100,000 patients who visited the hospital, incidence was 24.1. Only two patients (1.6%) had a functional NET. The rectum (n = 99, 79.8%) was most common primary site and found in early stage. The prevalence by stages was 84.7% stage I, 8.9% stage IV, 4.8% stage II, and 1.6% stage III. The pathology grading was 74.5% grade 1, 12.7% grade 2, and 12.7% grade 3. Tumor stage correlated positively with pathologic grade (Spearman’s rank correlation coefficient, 0.644). CONCLUSIONS: Wide range of clinicopathological features of Korean gastroenteropancreatic NETs were demonstrated using WHO 2010 classification. Rectal NET was most frequent and found in early stage.
Classification ; Epidemiology ; Humans ; Incidence* ; Joints ; Korea* ; Male ; Neoplasm Staging ; Neuroendocrine Cells ; Neuroendocrine Tumors* ; Pathology ; Prevalence ; Rectum ; Retrospective Studies ; Seoul ; World Health Organization

BACKGROUND/AIMS: Neuroendocrine tumors (NETs) may originate from heterogeneous neuroendocrine cells. The incidence is increasing worldwide, and World Health Organization (WHO) updated its classification in 2010. We investigated clinical characteristics of gastroenteropancreatic NETs in a single center. METHODS: Clinicopathologic characteristics of patients with pathologically confirmed gastroenteropancreatic NET in Seoul St. Mary Hospital from March 2009 to August 2011 were retrospectively analyzed. The grade and stage were determined according to WHO 2010 classification and TNM Staging System for Neuroendocrine Tumors (7th ed., 2010) of American Joint Committee on Cancer. RESULTS: One hundred and twenty-five patients (median age, 50; male, 61.3%) were analyzed. Among 100,000 patients who visited the hospital, incidence was 24.1. Only two patients (1.6%) had a functional NET. The rectum (n = 99, 79.8%) was most common primary site and found in early stage. The prevalence by stages was 84.7% stage I, 8.9% stage IV, 4.8% stage II, and 1.6% stage III. The pathology grading was 74.5% grade 1, 12.7% grade 2, and 12.7% grade 3. Tumor stage correlated positively with pathologic grade (Spearman’s rank correlation coefficient, 0.644). CONCLUSIONS: Wide range of clinicopathological features of Korean gastroenteropancreatic NETs were demonstrated using WHO 2010 classification. Rectal NET was most frequent and found in early stage.
Classification ; Epidemiology ; Humans ; Incidence* ; Joints ; Korea* ; Male ; Neoplasm Staging ; Neuroendocrine Cells ; Neuroendocrine Tumors* ; Pathology ; Prevalence ; Rectum ; Retrospective Studies ; Seoul ; World Health Organization

PURPOSE: Appendiceal tumors are a heterogeneous group of diseases that include typical neuroendocrine tumors (TNET), goblet cell carcinoids (GCC), and atypical GCC. Atypical GCC are classified into signet-ring cell cancers (SRCC) and poorly differentiated appendiceal adenocarcinoids. The prognosis and management of these diseases is unclear because there are no prospective studies. The aim of this study is to assess the characteristics and outcome of appendiceal TNET, GCC, and SRCC patients. MATERIALS AND METHODS: Appendiceal TNET, GCC, and SRCC patients diagnosed between 1973 and 2011 were identified in the Surveillance Epidemiology and End Results (SEER) database. Demographics, type of surgery, and clinicopathologic characteristics were collected. Survival functions were estimated by the Kaplan-Meier method, and log-rank test was used to assess the difference in overall survival (OS) among the three histologies. RESULTS: The SEER database yielded 1,021 TNET patients, 1,582 with GCC, and 534 SRCC patients. TNET presented at a younger age (p < 0.001). Patients with SRCC presented with advanced stage disease (p < 0.001). The median OS (mOS) for GCC and TNET patients was not reached; mOS for SRCC was 24 months. Multivariate analysis stratified for stage revealed significantly longer survival for TNET and GCC than SRCC (p < 0.001). CONCLUSION: This is the largest report to date for appendiceal neuroendocrine tumor patients, suggesting a spectrum of diseases with different characteristics and outcomes. In this report, we present a treatment approach for this complex spectrum of disease, based on the experience of Ohio State and Emory Universities investigators.
Appendiceal Neoplasms ; Carcinoid Tumor ; Demography ; Disease Management ; Epidemiology ; Goblet Cells ; Humans ; Multivariate Analysis ; Neuroendocrine Tumors ; Ohio ; Prognosis ; Prospective Studies ; Research Personnel

Primary malignant small bowel tumor as a rare kind of intestinal tumor is associated with a poor prognosis. The pathological types were various and complicated, such as adenocarcinoma, neuroendocrine tumor, malignant lymphoma, and malignant stromal tumor. The atypical early stage symptom resulted in difficult diagnosis at early stage, high misdiagnosis rate and lack of standard therapy schemes and means. In the past, X-ray, CT, MRI, and PET-CT were the main examination methods for primary small bowel tumor. However, with the development of radiology, a series of new diagnosis methods, including electronic enteroscopy, capsule endoscopy, multi-slice spiral CT enteroclysis and so on, promotes the diagnosis accurate rate. Surgery is still the most important method in the small bowel tumor treatment, and the alternative of the surgical method should depend on the tumor location, size and relationship with the adjacent organs. Application of the laparoscopic surgery for the small bowel tumor is still in the initial stage. Besides, some researches have confirmed that chemotherapy, radiotherapy, target therapy and endocrinotherapy have effects on the specific kind of small bowel tumor. Therefore this article will review the epidemiology, pathology, diagnosis and treatment of the primary malignant small bowel tumors.
Adenocarcinoma ; diagnosis ; epidemiology ; pathology ; therapy ; Capsule Endoscopy ; Gastrointestinal Stromal Tumors ; diagnosis ; epidemiology ; pathology ; therapy ; Humans ; Intestinal Neoplasms ; diagnosis ; epidemiology ; pathology ; therapy ; Intestine, Small ; diagnostic imaging ; pathology ; Laparoscopy ; methods ; Lymphoma ; diagnosis ; epidemiology ; pathology ; therapy ; Neuroendocrine Tumors ; diagnosis ; epidemiology ; pathology ; therapy ; Tomography, Spiral Computed

Antibodies, Monoclonal, Humanized ; therapeutic use ; Antineoplastic Agents ; therapeutic use ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bevacizumab ; Gastrointestinal Neoplasms ; classification ; epidemiology ; therapy ; Humans ; Indoles ; therapeutic use ; Neuroendocrine Tumors ; classification ; epidemiology ; therapy ; Octreotide ; therapeutic use ; Pancreatic Neoplasms ; classification ; epidemiology ; therapy ; Peptides, Cyclic ; therapeutic use ; Pyrroles ; therapeutic use ; Sirolimus ; analogs & derivatives ; therapeutic use ; Somatostatin ; analogs & derivatives ; therapeutic use