Kulshrestha Asmita, Jawale Deeksha, Sharma Ankit, Bhoj Priyanka, Pandagle Amitabh
Department of Radiation Oncology, Cama and Albless Hospital, Mumbai, IND.
Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, IND.
Cureus. 2025 Feb 28;17(2):e79807. doi: 10.7759/cureus.79807. eCollection 2025 Feb.
Cancers of the esophagus tend to be aggressive with a poor prognosis. Neuroendocrine carcinoma (NEC) of the esophagus is very rare and currently does not have any established treatment regimens. This is in part due to its rare prevalence. This case report presents an unusual case of distal esophageal NEC, which was complicated by liver metastasis and paraneoplastic syndrome. A 56-year-old postmenopausal woman came with complaints of weight loss, pain in the epigastric region, and intermittent dysphagia to solids for three months. An endoscopy revealed a 2 cm x 1 cm ulceronodular lesion located at the gastroesophageal junction. The biopsy showed NEC on histological examination, and the immunohistochemistry was positive for multiple neuroendocrine markers. A positron emission tomography (PET) scan revealed a stage IV metastatic disease that had spread to the liver as well as gastrohepatic and peri-gastric lymph nodes. Subsequently, the patient received palliative intent chemotherapy with Etoposide and Cisplatin for four cycles, which resulted in a partial response. Due to this, she was advised four more cycles of chemotherapy, but after two cycles, she developed signs of the syndrome of inappropriate antidiuretic hormone secretion (SIADH). Following management with hypertonic saline, her health deteriorated, resulting in death within one year of diagnosis. NECs of the esophagus tend to be detected at later stages and show a worse prognosis and disease course than squamous cell carcinoma or adenocarcinoma of the esophagus. With improvements in investigation methods, an increase in the detection of NEC in the esophagus can be expected in the future. Early diagnosis can help overall survival and boost quality of life. Further research and clinical trials are needed to assess ideal treatment plans.
食管癌往往具有侵袭性,预后较差。食管神经内分泌癌(NEC)非常罕见,目前尚无既定的治疗方案。部分原因是其发病率低。本病例报告呈现了一例罕见的食管远端NEC,伴有肝转移和副肿瘤综合征。一名56岁绝经后女性因体重减轻、上腹部疼痛和间歇性固体食物吞咽困难三个月前来就诊。内镜检查发现位于胃食管交界处有一个2 cm×1 cm的溃疡结节性病变。活检组织学检查显示为NEC,免疫组化多个神经内分泌标志物呈阳性。正电子发射断层扫描(PET)显示为IV期转移性疾病,已扩散至肝脏以及胃肝和胃周淋巴结。随后,患者接受了依托泊苷和顺铂的姑息性化疗四个周期,部分缓解。因此,建议她再进行四个周期的化疗,但两个周期后,她出现了抗利尿激素分泌不当综合征(SIADH)的症状。经高渗盐水治疗后,她的健康状况恶化,在诊断后一年内死亡。食管NEC往往在晚期被发现,与食管鳞状细胞癌或腺癌相比,预后和病程更差。随着检查方法的改进,未来预计食管NEC的检出率会增加。早期诊断有助于提高总体生存率和改善生活质量。需要进一步的研究和临床试验来评估理想的治疗方案。