Takenaka Shunsuke, Tsuji Toshikatsu, Doden Kenta, Hayashi Saki, Shimada Mari, Saito Hiroto, Yamamoto Daisuke, Okamoto Koichi, Ikeda Hiroko, Moriyama Hideki, Kinoshita Jun, Sato Yasunori, Ninomiya Itasu, Inaki Noriyuki
Department of Gastrointestinal Surgery, Kanazawa University Hospital, 13-1 Takara-Machi, Kanazawa, Ishikawa, 920-8641, Japan.
Department of General and Digestive Surgery, Kanazawa Medical University Hospital, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa, 920-0293, Japan.
Surg Case Rep. 2024 Sep 11;10(1):217. doi: 10.1186/s40792-024-02011-8.
Mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) is a rare malignant gastrointestinal tumor. The prognosis of patients with MiNEN is poor because of the high frequency of recurrence and metastases. We report a case of esophagogastric junction MiNEN (EGJ-MiNEN) with a long-term recurrence-free survival of 5.5 years.
A 58-year-old male patient underwent thoracoscopic esophagectomy for esophagogastric junction adenocarcinoma. The patient's postoperative course was uneventful. R0 resection was achieved, and the pathological diagnosis of the surgical specimen was pT3N2M0 Stage IIIA (according to the Japanese Classification of Gastric Cancer, 4th edition). Based on the pathology results, the patient was treated with postoperative adjuvant therapy with oral S-1. The patient maintained recurrence-free survival for 5.5 years postoperatively. However, 6 years postoperatively, the patient visited our department with cachexia. Computed tomography (CT) revealed a large amount of ascites and pleural effusion. He rapidly developed a poor circulatory and respiratory status and died 16 days after admission. An autopsy revealed severe bloody ascites and pleural effusion, as well as numerous nodules on the pleura and mesentery. Immunohistochemistry of the nodules revealed positivity for chromogranin A, Synaptophysin A, neural cell adhesion molecule (NCAM or CD56), and insulinoma-associated protein 1 (INSM1). The specimen showed a mixture of adenocarcinoma and neuroendocrine cell carcinoma and was diagnosed as MiNEN. Retrospective immunostaining of the surgical specimen showed similar results, and we diagnosed the patient with recurrence of EGJ-MiNEN.
MiNEN has a poor prognosis; however, in some cases, long-term recurrence-free survival is achieved with radical resection and adjuvant chemotherapy.
混合性神经内分泌-非神经内分泌肿瘤（MiNEN）是一种罕见的胃肠道恶性肿瘤。由于复发和转移频率高，MiNEN患者的预后较差。我们报告一例食管胃交界部MiNEN（EGJ-MiNEN）患者，其无复发生存期长达5.5年。
一名58岁男性患者因食管胃交界部腺癌接受了胸腔镜下食管切除术。患者术后恢复顺利。实现了R0切除，手术标本的病理诊断为pT3N2M0 IIIA期（根据日本胃癌分类第4版）。根据病理结果，患者接受了口服S-1辅助化疗。患者术后保持了5.5年的无复发生存期。然而，术后6年，患者因恶病质前来我院就诊。计算机断层扫描（CT）显示大量腹水和胸腔积液。他很快出现循环和呼吸功能不良，并在入院后16天死亡。尸检显示严重血性腹水和胸腔积液，以及胸膜和肠系膜上有许多结节。结节的免疫组织化学检查显示嗜铬粒蛋白A、突触素A、神经细胞黏附分子（NCAM或CD56）和胰岛素瘤相关蛋白1（INSM1）呈阳性。标本显示腺癌和神经内分泌细胞癌混合，诊断为MiNEN。手术标本的回顾性免疫染色显示了类似结果，我们诊断该患者为EGJ-MiNEN复发。
MiNEN预后较差；然而，在某些情况下，通过根治性切除和辅助化疗可实现长期无复发生存。
