Tomiyama Takashi, Orino Masahiro, Nakamaru Koh, Tanaka Toshihiro, Suzuki Ryo, Okazaki Takashi, Fukata Norimasa, Ando Yugo, Danbara Naoyuki, Fukui Toshiro, Miyasaka Chika, Okazaki Kazuichi
Division of Gastroenterology and Hepatology, Third Department of Internal Medicine, Kansai Medical University, Osaka, Japan.
Department of Pathology and Laboratory Medicine, Kansai Medical University, Osaka, Japan.
Case Rep Gastroenterol. 2018 May 31;12(2):234-239. doi: 10.1159/000489295. eCollection 2018 May-Aug.
Esophageal large-cell neuroendocrine carcinoma (NEC) is a rare malignant tumor that is characterized by high-grade malignancy and a poor prognosis. However, the rarity of esophageal NEC has prevented the development of an established treatment, and no reports have described a discrepancy in the effectiveness of cisplatin plus irinotecan between primary and metastatic lesions. A 43-year-old Japanese man was referred to our hospital with refractory epigastralgia. A previous gastrointestinal endoscopy had revealed a 50-mm type 2 tumor in the abdominal esophagus. The pathological findings indicated poorly differentiated squamous cell carcinoma. Contrast-enhanced computed tomography revealed a metastatic liver tumor. One cycle of fluorouracil and cisplatin was not effective, and endoscopy was repeatedly performed. The pathological findings indicated a large-cell malignant tumor with tumor cells that were positive for CD56, synaptophysin, and Ki-67 (> 80%). Based on a diagnosis of esophageal large-cell NEC with a metastatic liver tumor, the patient received cisplatin plus irinotecan biweekly. After 4 months, computed tomography revealed marked shrinkage of the metastatic tumor, but the patient complained of dysphagia. Endoscopy revealed enlargement of the primary tumor, which was then treated using radiotherapy plus fluorouracil and cisplatin. The primary tumor subsequently shrank, and the patient's symptoms were relieved, but the metastatic tumor grew. Thus, chemoradiotherapy could be an option for managing a primary esophageal large-cell NEC that does not respond to chemotherapy alone. However, the possibility of an inconsistent response to therapy in primary and metastatic lesions should be considered.
食管大细胞神经内分泌癌（NEC）是一种罕见的恶性肿瘤，其特点是恶性程度高、预后差。然而，食管NEC的罕见性阻碍了既定治疗方法的发展，且尚无报道描述顺铂联合伊立替康在原发性和转移性病变中的疗效差异。一名43岁的日本男性因难治性上腹痛转诊至我院。先前的胃肠内镜检查发现腹部食管有一个50毫米的2型肿瘤。病理结果显示为低分化鳞状细胞癌。增强计算机断层扫描显示有肝转移瘤。氟尿嘧啶和顺铂的一个疗程治疗无效，遂反复进行内镜检查。病理结果显示为大细胞恶性肿瘤，肿瘤细胞CD56、突触素和Ki-67呈阳性（>80%）。基于食管大细胞NEC伴肝转移瘤的诊断，患者接受顺铂联合伊立替康的双周治疗。4个月后，计算机断层扫描显示转移瘤明显缩小，但患者出现吞咽困难。内镜检查显示原发性肿瘤增大，随后采用放疗联合氟尿嘧啶和顺铂进行治疗。原发性肿瘤随后缩小，患者症状缓解，但转移瘤增大。因此，放化疗可能是治疗对单纯化疗无反应的原发性食管大细胞NEC的一种选择。然而，应考虑原发性和转移性病变对治疗反应不一致的可能性。
