A 73-year-old male patient arrived at our hospital reporting difficulty in swallowing. He had no pain in swallowing, no heartburn and no nausea. He lost approximately 4 kg from a baseline weight of 75 kg despite having a normal appetite. He had history of smoking (Brinkman Index = 1200). There was no family history of gastrointestinal malignancies. Upper gastrointestinal endoscopy revealed a large esophageal mass with normal mucosa on the surface at 33 cm distant from an incisor tooth (Fig. 1). NEC was pathologically confirmed from the biopsy specimens. Computed tomography (CT) showed a poorly enhanced large mass in the lower esophagus (Fig. 2a). Positron emission tomography/computed tomography (PET-CT) revealed a hypermetabolic esophageal tumor (standardized uptake value max = 7.89) with no evidence of distant metastasis (Fig. 2b).
We performed open subtotal esophagectomy with lymphadenectomy. The patient was discharged on postoperative day 31 with no serious complications. Histopathological examination showed a NEC of the large-cell type in view of irregular strands of large polygonal cells. In addition, synaptophysin, chromogranin A and CD56 were positive (Fig. 3) and the Ki-67 labeling index was 80% by immunohistochemical staining. The pathological stage was pT1b pN2 M1, pStage IV. We performed adjuvant chemoradiation therapy (total 45 Gy/25 fr) with combined chemotherapy that consisted of docetaxel (60 mg/m2 on day 1), fluorouracil (350 mg/m2 on days 1-5) and cisplatin (6 mg/m2 on days 1-5) for two cycles after the operation. However, 5 months after the operation, a CT scan showed metastasis in segment 6 of the liver (Fig. 4a). Furthermore, PET-CT showed metastasis in the right eighth rib (Fig. 4b). We then performed open partial hepatectomy for the liver metastasis. The patient was discharged on postoperative day 10 with no serious complications. Then, we performed radiation therapy (total 51 Gy/17 fr) for the rib metastasis. The metastasis at the right eighth rib disappeared; however, 2 months after radiation therapy, PET-CT showed a new metastasis of a hilar lymph node in the left lung (Fig. 5a) and sacral bone (Fig. 5b). We started chemotherapy with cisplatin (60 mg/m2 on day 1) and irinotecan (60 mg/m2 on days 1, 8, and 15). After two cycles of chemotherapy, the size of the left lung hilar lymph node reduced from 24 mm to 17 mm. Finally, after 7 months from the start of the last chemotherapy (20 months after subtotal esophagectomy), the patient died of disease progression.