收稿日期: 2025-01-14
网络出版日期: 2025-06-19
基金资助
国家自然科学基金资助项目(82172865)
Treatment strategies for limited⁃stage primary small cell carcinoma of esophagus
Received date: 2025-01-14
Online published: 2025-06-19
原发性食管小细胞癌（primary small cell carcinoma of esophagus， PSCCE）是一种罕见的且具有高度侵袭性的神经内分泌肿瘤，占所有食管恶性肿瘤的0.05% ~ 3.1%。其生物学特征与肺小细胞癌相似，表现为极强的侵袭性及早期播散倾向，常通过淋巴道及血行途径快速转移，临床预后极不乐观，5年总生存率不足15%。由于缺乏大规模的病例随机对照研究，所以标准治疗策略尚未统一。局限期原发性食管小细胞癌的治疗一直是临床研究的热点。在传统治疗模式中，极早期可行内窥镜治疗，相对早期患者以根治性手术为主，而局部晚期患者，临床上最常采用两种治疗模式：一种是基于手术的综合治疗方案，另一种是以根治性放化疗为核心的治疗方案，最佳的治疗模式尚无定论。与此同时，免疫治疗与靶向治疗等新兴策略在临床应用中疗效显著，为局限期原发性食管小细胞癌带来新的治疗机会。本文旨在综述近年来关于局限期原发性食管小细胞癌治疗的研究进展，总结诊疗现状及未来方向。
邱小琳 , 范秉杰 , 王琳琳 . 局限期原发性食管小细胞癌的治疗策略[J]. 实用医学杂志, 2025 , 41(11) : 1603 -1610 . DOI: 10.3969/j.issn.1006-5725.2025.11.001
Primary small cell carcinoma of esophagus （PSCCE） is a rare and highly aggressive neuroendocrine tumor， accounting for 0.05% to 3.1% of all esophageal malignancies. Its biological characteristics are similar to those of small cell lung cancer， with highly aggressive behavior and early dissemination tendency. It often metastasizes rapidly through lymphatic and hematogenous pathways.The prognosis is extremely poor， with a 5-year overall survival rate of less than 15%. There are no large-scale randomized controlled trials， and no standard treatment strategies have been established. In recent years， the treatment of limited-stage PSCCE has become a focal point of research. In traditional treatment paradigms， endoscopic therapy is feasible for very early-stage cases， while radical surgery serves as the primary approach for relatively early-stage patients. For locally advanced cases， two predominant treatment modalities are commonly employed in clinical practice： a surgery-based comprehensive treatment regimen and a radical chemoradiotherapy-centered therapeutic protocol， with no definitive conclusion yet reached regarding the optimal treatment strategy. Concurrently， emerging therapeutic strategies such as immunotherapy and molecularly targeted therapy have demonstrated remarkable clinical efficacy， thereby providing novel therapeutic opportunities for limited-stage PSCCE. This article aims to review the recent advances in the treatment of limited-stage PSCCE， summarize the current diagnostic and therapeutic landscape， and outline future directions in this field.
Key words： small cell carcinoma; esophageal carcinoma; treatment; survival
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