Department of Head and Neck, Esophageal Medical Oncology, National Cancer Center Hospital, Tokyo, Japan.
Department of Surgery, Keio University School of Medicine, Tokyo, Japan.
Jpn J Clin Oncol. 2024 Feb 7;54(2):111-120. doi: 10.1093/jjco/hyad144.
Esophageal cancer is common worldwide, including in Japan, and its major histological subtype is squamous cell carcinoma. However, there are some rare esophageal cancers, including neuroendocrine neoplasm, gastrointestinal stromal tumor, carcinosarcoma and malignant melanoma. The biological and clinical features of these cancers differ from those of esophageal squamous cell carcinoma. Therefore, different treatment strategies are needed for these cancers but are based on limited evidence. Neuroendocrine neoplasm is mainly divided into neuroendocrine tumor and neuroendocrine carcinoma by differentiation and the Ki-67 proliferation index or mitotic index. Epidemiologically, the majority of esophageal neuroendocrine neoplasms are neuroendocrine carcinoma. The treatment of neuroendocrine carcinoma is similar to that of small cell lung cancer, which has similar morphological and biological features. Gastrointestinal stromal tumor is known to be associated with alterations in the c-KIT and platelet-derived growth factor receptor genes and, if resectable, is treated in accordance with the modified Fletcher classification. Carcinosarcoma is generally resistant to both chemotherapy and radiotherapy and requires multimodal treatments such as surgery plus chemotherapy to achieve cure. Primary malignant melanoma is resistant to cytotoxic chemotherapy, but immune checkpoint inhibitors have recently demonstrated efficacy for malignant melanoma of the esophagus. This review focuses on the current status and future perspectives for rare cancer of the esophagus.
食管癌在全球范围内较为常见，包括在日本，其主要组织学亚型为鳞状细胞癌。然而，还有一些罕见的食管癌，包括神经内分泌肿瘤、胃肠道间质瘤、癌肉瘤和恶性黑色素瘤。这些癌症的生物学和临床特征与食管鳞状细胞癌不同。因此，这些癌症需要不同的治疗策略，但基于有限的证据。神经内分泌肿瘤主要通过分化和 Ki-67 增殖指数或有丝分裂指数分为神经内分泌瘤和神经内分泌癌。从流行病学角度来看，大多数食管神经内分泌肿瘤为神经内分泌癌。神经内分泌癌的治疗与具有相似形态和生物学特征的小细胞肺癌相似。胃肠道间质瘤已知与 c-KIT 和血小板衍生生长因子受体基因的改变有关，如果可切除，则根据改良的 Fletcher 分类进行治疗。癌肉瘤通常对化疗和放疗均有耐药性，需要多模式治疗，如手术加化疗，以达到治愈。原发性恶性黑色素瘤对细胞毒性化疗耐药，但免疫检查点抑制剂最近已证明对食管恶性黑色素瘤有效。本综述重点介绍了食管罕见癌症的现状和未来展望。
