• Dichotomous staging
• Esophagus
• Risk stratification system
• Small cell carcinoma
• TNM staging

• conversion surgery
• esophageal neuroendocrine carcinoma
• immune checkpoint inhibitor
• multidisciplinary therapy
• nivolumab

• Carcinoma, Small Cell/pathology
• Carcinoma, Small Cell/therapy
• Esophageal Neoplasms/pathology
• Humans
• Lymph Nodes/pathology
• Lymphatic Metastasis/pathology
• Neoplasm Staging
• Prognosis
• Retrospective Studies
• Survival Rate

• adjuvant therapy
• esophageal
• neuroendocrine carcinoma (nec)
• prognosis
• surgery

• Adult
• Aged
• Carcinoma, Neuroendocrine/mortality
• Carcinoma, Neuroendocrine/pathology
• Carcinoma, Neuroendocrine/therapy
• Esophageal Neoplasms/mortality
• Esophageal Neoplasms/pathology
• Esophageal Neoplasms/therapy
• Female
• Humans
• Lymphatic Metastasis
• Male
• Middle Aged
• Neoplasm Staging
• Prognosis
• Tumor Burden

• chemotherapy
• limited-stage disease
• prognosis
• small cell carcinoma of the esophagus
• surgery

Primary small cell carcinoma of the esophagus (SCCE) is a rare and extremely fatal disease. We aim to evaluate the efficacy of radical surgery for resectable SCCE and to explore potential prognostic factors.

We retrospectively reviewed 52 consecutive SCCE patients who underwent radical surgery from February 1993 to November 2014 at a single institution. The Kaplan-Meier estimator with log-rank test was used to assess overall survival (OS), disease-free survival (DFS) and median survival time. Univariate and multivariable analyses were used to evaluate prognostic factors through Cox proportional hazard regression model.

Twenty-five (48.1%) patients were treated with surgery alone, whereas 27 (51.9%) patients underwent adjuvant therapy after surgery. The median OS time was 17.4 months (95% CI: 13.5-21.3). The median DFS time was 13.4 months (95% CI: 7.7-19.0). Patients whose tumors were located in the lower part of thoracic esophagus and the esophagogastric junction showed significantly better OS (27.0 vs. 13.2 months, P = 0.016) and DFS (27.0 vs. 11.3 months, P = 0.017) than those located in the upper and middle parts. Patients with N0 status experienced significantly better OS (21.4 vs. 11.6 months, P = 0.012) and DFS (21.4 vs. 8.6 months, P = 0.012) than those with N+ status. Patients whose tumor lengths were shorter than 5 cm had a better OS (17.4 vs. 5.7 months, P = 0.035) than those longer than 5 cm. Patients who underwent chemotherapy experienced a significantly improved OS (21.0 vs. 14.1 months, P = 0.032) compared to surgery alone. Multivariable analysis showed that lower tumor location, shorter tumor length, pN0 status and chemotherapy independently predicted better OS; lower tumor location and pN0 status independently predicted better DFS.

Radical surgery in combination with chemotherapy has better outcomes than surgery alone for resectable SCCE. Higher tumor location, longer tumor length, lymph node metastasis and not undergoing chemotherapy independently predict worse prognoses.

• cancer therapy
• chemotherapy
• esophageal neoplasm
• small cell carcinoma
• surgery

Primary small cell carcinoma of the esophagus (PSCCE) is a rare and aggressive malignancy. It has a poor survival rate, and there is no consensus as to a standard therapeutic modality. In this study, we aimed to investigate the prognostic factors and evaluate the outcomes of patients with PSCCE who had been treated with different therapeutic methods.

We retrospectively evaluated 113 consecutive patients with PSCCE who received treatment at our center from 2003 to 2016. The primary endpoint was overall survival (OS). The Cox regression model was used to analyze the prognostic factors. The survival analysis was calculated using the Kaplan-Meier and log-rank methods.

The 12- and 36-month OS rates of all 113 enrolled patients were 45% and 12%, respectively. A significantly prolonged OS rate was associated with lymph node stages N0–N1 (P=0.022), the Veterans’ Administration Lung Study Group (VALSG) system limited-disease (LD) staging (P=0.040), and multimodality treatments (P=0.047). Patients with regional lymph node metastasis benefited more from surgery combined with chemotherapy than surgery or chemotherapy alone (P=0.046). Concerning chemotherapy, cisplatin plus etoposide was the regimen most commonly used to treat PSCCE patients (67.5%).

An early lymph node stage, the VALSG LD staging, and multimodality treatments were identified as independent prognostic factors of PSCCE. Surgery combined with adjuvant chemotherapy was especially necessary for LD stage PSCCE patients with lymph node stages N1–3.

• Primary small cell carcinoma
• esophagus
• prognosis
• treatment

• Case report
• Early stage
• Endoscopic submucosal dissection
• Endoscopic ultrasonography
• Poor prognosis
• Primary esophageal small cell carcinoma
• Small submucosal masses

Currently, there are no standard treatments for primary small cell carcinoma of the esophagus (PSCCE), particularly in cases of limited-stage disease. This retrospective study aimed to assess the treatment strategies and the relevant prognostic factors of limited-stage PSCCE (LS-PSCCE).

We retrospectively evaluated 129 patients with LS-PSCCE between June 2009 and December 2018. The χ2 test was performed to examine the frequencies between different groups. The Kaplan-Meier and log-rank methods were used to estimate and compare survival rates. Univariate and multivariate analyses were performed to determine the prognostic factors for overall survival (OS).

Through a median follow-up of 23 months, the median OS of all patients was 25.0 months and the median recurrence-free survival (RFS) was 15.0 months. Univariate and multivariate analyses showed that alcohol abuse (p=0.046) and TNM stage (p<0.001) were independent prognostic factors. There was no significant difference in OS and RFS rates between the patients treated with chemoradiotherapy (CRT) and those treated with surgery and chemotherapy with or without radiotherapy (S+CT±RT) (p>0.05). Patients who received concurrent CRT had better OS and RFS than those who received sequential CRT (p<0.05). Postoperative adjuvant RT for high-risk patients can further improve the local control rate but has no significant effect on OS.

LS-PSCCE patients treated with CRT had similar OS and RFS compared to those treated with S+CT±RT. This study shows that concurrent CRT confers a survival advantage for patients with LS-PSCCE compared to those with sequential CRT.

• chemoradiotherapy
• limited-stage
• primary small cell carcinoma of the esophagus
• surgery

Primary small cell carcinoma of the esophagus (PSCCE) is a highly malignant tumor that is diagnosed by endoscopic biopsy and immunohistochemistry. Because of its low incidence, a high degree of malignancy, and rapid progress, it is difficult to conduct large, randomized controlled trials and to establish a standard treatment plan for this disease. In recent years, several retrospective studies have been reported, and with the rise of emerging therapies, PSCCE has gradually become a focus of thoracic surgery. This paper reviews progress in the diagnosis and treatment of PSCCE in recent years.

• Primary small cell carcinoma
• esophagus
• treatment

• apatinib
• esophagectomy
• s-1
• salvage
• serratus anterior plane block

• Antimetabolites, Antineoplastic/therapeutic use
• Carcinoma, Small Cell/drug therapy
• Carcinoma, Small Cell/surgery
• Drug Combinations
• Drug Resistance, Neoplasm
• Drug Therapy, Combination
• Esophageal Neoplasms/drug therapy
• Esophageal Neoplasms/surgery
• Esophagectomy
• Humans
• Irinotecan/therapeutic use
• Male
• Middle Aged
• Oxonic Acid/therapeutic use
• Protein Kinase Inhibitors/therapeutic use
• Pyridines/therapeutic use
• Salvage Therapy
• Tegafur/therapeutic use

• SEER
• cause-specific mortality
• competing risks
• esophageal neuroendocrine carcinoma
• recursive partitioning analysis

Small cell carcinoma of the esophagus (SCCE) is characterized by its progressive feature and poor prognosis. There is no consensus on a standard therapeutic modality for SCCE. In this study, we aimed to characterize the outcomes of primary SCCE patients treated by radiation therapy as part of treatment and investigate prognostic factors.

We retrospectively analyzed the data of 42 SCCE patients who were treated by RT as part of treatment at the Sun Yat-sen University Cancer Center from 2001 to 2014. The Kaplan-Meier and log-rank method were used to analyze survival. Cox’s hazard regression model was applied to determine prognostic factors.

Of the 42 enrolled patients, 25 had limited disease (LD) and 17 with extensive disease (ED). The overall response rate (CR + PR) was 60.0% (21/35). The median overall survival time (OS) for whole and LD group were 12.9 and 36.8 months. The 1-, 3- and 5-year OS rates of the whole cohort were 64.9, 31.3, and 13.9%, respectively. OS was significantly longer in patients with ECOG performance score (ECOG PS) < 2 (p = 0.001), lesion length ≤ 5 cm (p = 0.001), and LD (p = 0.049). In the patients with LD, multivariate analysis indicated that combined with chemotherapy (P = 0.046) and higher radiation dose (P = 0.027) predicted better prognosis in OS. The overall rate of grade 3–4 toxicities in the whole cohort was 37.5%. In total, 65% (17/26) patients with recurrent disease died with the metastasis with or without the primary recurrence.

RT was one of the effective and safe treatments for locoregional control of SCCE. Lower ECOG PS score, shorter lesion length, treated with chemotherapy, and a higher dose of RT were identified as favorable independent prognostic factors.

• Chemotherapy
• IMRT
• Prognosis
• Radiation therapy
• Small cell carcinoma of esophagus

• Esophageal NEC
• Gastroenteropancreatic NET
• Large cell esophageal NEN
• MANEC
• Small cell carcinoma

The optimal standard treatment for primary small cell carcinoma of the esophagus (SCCE) remains undetermined. In this study, we conducted two areas of research on SCCE. First, we analyzed differences in SCCE characteristics between Chinese and U.S. patients. Second, we evaluated optimal treatment strategies for SCCE in the Chinese cohort.

Data from 137 Chinese SCCE patients collected from two cancer centers in China were compared with 385 SCCE patients registered in the U.S. SEER program. Prognostic factors were further analyzed in the Chinese group. Propensity score matching (PSM) was used to balance baseline features between the groups.

There were more Chinese SCCE patients with regional stage disease (41.6%) and surgery was the principal local therapy (78.1%), while 51.7% of U.S. patients was at advanced stages and tended to receive radiotherapy as the main therapy (45.2%). Median overall survival (MST) of Chinese patients was 15.0 months, compared with 8.0 months for U.S. patients (P < 0.001). However, the survival differences between groups disappeared after PSM (MST: 12.5 m vs 9.0 m, P = 0.144). Further analysis found that surgery tended to achieve clinical benefits only for patients with localized disease (T1‐4aN0M0). Radiotherapy and chemotherapy may prolong survival in patients with regional and extensive disease.

Although there are huge differences in the tumor characteristics and treatment modalities of SCCE between Chinese and U.S. patients, the prognosis of SCCE is equally poor in both. Surgery should be considered for patients with localized disease, while chemoradiotherapy is recommended for patients with regional and extensive disease.

• Primary small cell carcinoma of the esophagus
• SEER
• disparities
• multimodal treatment
• prognostic factors

• basaloid squamous cell carcinoma
• esophageal small cell carcinoma
• prognosis
• treatment

• Antineoplastic Combined Chemotherapy Protocols/therapeutic use
• Carcinoma, Basosquamous/drug therapy
• Carcinoma, Basosquamous/pathology
• Carcinoma, Basosquamous/surgery
• Carcinoma, Small Cell/drug therapy
• Carcinoma, Small Cell/pathology
• Carcinoma, Small Cell/surgery
• Chemotherapy, Adjuvant
• Esophageal Neoplasms/drug therapy
• Esophageal Neoplasms/pathology
• Esophageal Neoplasms/surgery
• Esophagectomy
• Female
• Humans
• Male
• Middle Aged
• Neoplasm Staging
• Survival Analysis

• Classification
• Diagnostic approach
• Esophagus
• Neuroendocrine tumors
• Therapeutic management

• Limited-stage
• Primary small cell carcinoma of the esophagus
• Prognostic factors
• Treatment

• Ki-67
• esophagus
• prognosis
• small cell carcinoma

• Neuroendocrine carcinoma (NEC)
• esophagus
• prognosis
• surgery