Thymic Neuroendocrine Tumor With Sjogren’s Syndrome Cured by Neoadjuvant Chemotherapy and Thymectomy：A Case Report

Abstract Background: Thymic neuroendocrine tumors are rare neoplasms usually accompanied with excessive secretion functional hormones of endocrine glands, such as multiple endocrine neoplasia 1(MEN1) and ectopic ACTH syndrome(EAS). As a kind of autoimmune disease, Sjogren’s syndrome(SS) is characterized by destruction of exocrine glands resulting in oral and ocular dryness and is usually related to thymic lesion. However, we present herein a rare case of thymic neuroendocrine tumor with primary Sjogren’s syndrome cured by neoadjuvant chemotherapy and thymectomy.Case presentation: A 70-year-old woman was diagnosed as neuroendocrine carcinoma by puncture with Sjogren’s syndrome(SS). Considering that the boundary between the tumor and the surrounding tissue is not clear，we chose two cycles of neoadjuvant chemotherapy which regimen was etoposide and cisplatin. After treatment, the symptoms of Sjogren’s syndrome were partial relieved and therefore the patient underwent R0 resection. After one year of follow-up, apparent remission of the symptoms was achieved.Conclusions: We believe neoadjuvant chemotherapy combined with radical surgery is an alternative treatment for locoregional resectable thymic neuroendocrine tumors. Because chemotherapy can lead to necrosis, it is a question whether the pathological grade of neuroendocrine tumors will be changed after neoadjuvant chemotherapy. Meanwhile, despite the fact that the association between thymic neuroendocrine tumors and Sjogren’s syndrome remains unclear, the latter may be co-treated by oncologic therapy of the former，thus it also provides a new treatment for Sjogren's syndrome.