A Single Center Analysis of Thymic Neuroendocrine Tumors

Simple Summary Thymic neuroendocrine tumors are extremely rare and therefore few published studies currently exist. This study sought to investigate the basic clinical characteristics, treatment and prognosis of TNETs using single-center retrospective data and to address this gap in the literature. We found that thymic neuroendocrine tumors are a rare and aggressive disease with a high recurrence rate even in typical carcinoid tumors that are usually considered to have a good prognosis, with local recurrence and bone metastases being a common mode of treatment failure. Despite the widely accepted view that surgical resection should be the treatment of choice for thymic neuroendocrine tumors, we found that combination therapy including radiotherapy and chemotherapy is necessary to address the high recurrence rate of this typically aggressive tumor. In addition, patients who suffered from large vessel invasion in our study had a lower rate of overall survival and a high risk of tumor progression, other therapeutic regimes should be explored for these patients. Purpose: Thymic neuroendocrine tumors (TNETs) are a collection of slow-progressing neoplasms located in the anterior mediastinum. Relatively few previously published studies have focused on thymic carcinomas. This study investigated the basic clinical characteristics, treatment, and prognosis of TNETs. Methods: Patients were enrolled in the study from January 2003 to December 2017 who had been diagnosed with TNETs through pathological screening and treated at our institution. Demographic data from each patient, the Masaoka stage, histology and size of the tumor, tumor invasion characteristics, and therapeutic strategies were gathered. The Kaplan-Meier method was used to assess patient survival. In addition, the log-rank test was used to carry out univariate analyses. Results: Twenty-six patients were eligible for inclusion in the study. The median age of the patients was 46.5 (25-69) years. The tumor median maximum diameter was 7.9 cm (from 3 to 19 cm). Twenty-four patients were treated surgically. Nineteen patients completed radiation therapy, and sixteen patients underwent chemotherapy. A median follow-up time of 54.95 months was observed. The survival rate for three years was 75.0% and 70.6% for five years. The corresponding progression-free survival rates for three and five years were 55.7% and 37.7%, respectively. The local, regional recurrence-free survival (LRFS) rates were 87.2% and 81.7%, and the distant metastasis-free survival (DMFS) rates were 55.7% and 37.7%, at three and five years, respectively. Local recurrence (six patients) and bone metastasis (six patients) were observed as the most frequent failures. Conclusion: TNET was observed to be an aggressive but rare malignant lesion. While the predominant treatment was complete resection, chemotherapy and radiotherapy were also required due to the high recurrence rate.

Automated summary

Thymic neuroendocrine tumors (TNETs) are rare and aggressive, with a high recurrence rate even in typical carcinoid tumors. Surgical resection is the main treatment, however, combination therapy including chemotherapy and radiotherapy is necessary to improve the treatment outcome. Patients with large vessel invasion have a poor prognosis and alternative therapeutic approaches should be explored.