Advances In Diagnosis And Treatment Of Primary Esophageal Small Cell Carcinoma

Background: Primary esophageal small cell carcinoma is a rare highly malignanttumor. It is prone to early occurrence of lymph node and distant metastasis with poorprognosis. Standard treatment are not well established because of the paucity of casesand a lack of large case-control study. In this article, we will review the recent advancesin diagnosis and treatment of primary esophageal small cell carcinoma.Objective: The purpose of this study is to evaluate the clinical and pathologic factorsassociated with survival in patients with primary esophageal small cell carcinoma, andto explore diagnosis, treatment and prognosis through literature review. So we canunderstand PESC better and improve the diagnosis rate, treatment and prognosis.Methods:1. The papers were searched from CNKI, Wan Fang data base, Pubmed andSpringerlink with the keywords “Esophageal neoplasms; Small cell carcinoma;Diagnosis; Treatment” respectively or combinedly.2. Retrieval criteria:1) the clinicaland pathologic factors associated with survival in patients with PESC;2) Imaging ofPESC;3) diagnosis of PESC;4) Staging of PESC;5) the results of treatment includingsurgery, chemotherapy, radiation therapy and combinative therapy with PESC;6) theprognosis analysis of PESC. Totally49papers were synthesized.Results: PESC was a rare and high-grade malignant esophageal neoplasms. It wascharacterized by frequent and early lymph node and distant metastases, resulting in arelatively poorer prognosis than other subtypes of esophageal neoplasms. Diagnosis wasbased on presence of small and round cancer cells, and confirmed byimmunohistological staining. The optimal treatment strategies for this aggressive tumorhad not yet been determined. Combined therapy was recommended. PESC is a systemicdisease. The tumor stage (LD or ED), length of the primary lesion and chemotherapyare independent prognostic factors. So this kind of patients should have diagnosis earlyand have a suitable treatment scheme to improve the prognosis and the life quality ofpatients.Conclusions: Primary esophageal small cell carcinoma is a systemic disease with arapid tumor growth. It is aggressive with early metastases. Patients with PESC have a poor prognosis, which is emphasized on early diagnosis and comprehensive treatment.Due to its rarity and the lack of large case-control study, a sufficient number of patientsto enroll is needed to explore the best treatment methods.