Incidence, treatment, and survival analysis in esophageal neuroendocrine carcinoma population

Abstract

Background As a subtype of esophageal neuroendocrine neoplasms (ENENs), esophageal neuroendocrine carcinoma (ENEC) is rare. ENEC can be poorly differentiated and highly aggressive. This study aims to illustrate the incidence, treatment, and prognosis of ENEC by using a population-based database. Methods We collected clinicopathological data [1975–2016] of ENEC, esophageal adenocarcinoma (EACA), and esophageal squamous cell carcinoma (ESqCC) from the Surveillance, Epidemiology and End Results (SEER) database, and analyzed their incidence, treatment, and prognosis. Results A total of 60,238 cases were in our study cohort, including ENEC (n=686), EACA (n=26,575), and ESqCC (n=32,977). The incidence of ENEC in 2016 was 0.044 per 100,000 persons. The tumor grade of ENEC was significantly higher than that of EACA and ESqCC (P<0.001). Both cancer-specific survival (CSS) and overall survival (OS) of ENEC were significantly worse than those of EACA and ESqCC (P<0.001). Kaplan-Meier analysis showed significant improvement of CSS and OS by surgery, radiotherapy, and chemotherapy (all P<0.001). Besides, compared with monotherapies, combinational therapies brought more benefits to both CSS and OS of patients with ENEC. Conclusions Our population-based evidence revealed that ENEC, as a rare cancer, had a worse prognosis, compared with EACA and ESqCC. Surgery, chemotherapy, and radiotherapy can all improve the prognosis of ENEC patients. Combinational therapy can yield a better prognosis than monotherapy.

Clinical characteristics, prognostic factors, and survival trends in esophageal neuroendocrine carcinomas: A population‐based study

“…On the other hand, several studies from China and Japan reported that radical esophagectomy should be considered as the preferred treatment for limited‐stage ENECs 11,21,26 . Another study analyzing ENEC cases from SEER database found that surgery + chemotherapy, as well as surgery + radiotherapy, can bring more significant benefits than chemotherapy or radiotherapy alone 10 . However, the lack of randomized controlled trials and insufficient data made it impossible to conclude which modality should be recommended to ENEC patients.…”

“… 11 , 21 , 26 Another study analyzing ENEC cases from SEER database found that surgery + chemotherapy, as well as surgery + radiotherapy, can bring more significant benefits than chemotherapy or radiotherapy alone. 10 However, the lack of randomized controlled trials and insufficient data made it impossible to conclude which modality should be recommended to ENEC patients. Treatment could be more individualized, based on the location of the tumor, depth of invasion, lymph node or distance metastases, general condition of patients, and also the traditions of each institution.…”

“…[1][2][3][4][5][6] Due to high malignant potential and poor prognosis of ENECs, our understanding of this malignancy is mainly based on case reports and case series. [7][8][9] The prevalence of ENEC has gradually risen over the past 10 years, 10 but clinicians still lack treatment experience and suitable guidelines. A systematic description of its epidemiology feature, tumor characteristic, treatment strategy, and prognosis is needed.…”

“…The prevalence of ENEC has gradually risen over the past 10 years, 10 but clinicians still lack treatment experience and suitable guidelines. A systematic description of its epidemiology feature, tumor characteristic, treatment strategy, and prognosis is needed.…”

Oesophageal neuroendocrine tumours—case series of a rare malignancy

“…Oesophageal neuroendocrine neoplasms (NENs) encompass a group of rare and heterogeneous tumours that are associated with very poor outcomes [ 1 ]. They constitute only 2% of malignant tumours of the gastrointestinal tract, with the majority located in the small bowel [ 2 ].…”