Multicenter retrospective analysis of systemic chemotherapy for advanced neuroendocrine carcinoma of the digestive system

Abstract

This study analyzed outcomes of systemic chemotherapy for advanced neuroendocrine carcinoma (NEC) of the digestive system. Clinical data from 258 patients with unresectable or recurrent NEC of the gastrointestinal tract (GI) or hepato-biliary-pancreatic system (HBP), who received chemotherapy, were collected from 23 Japanese institutions and analyzed retrospectively. Patients had primary sites in the esophagus (n = 85), stomach (n = 70), small bowel (n = 6), colorectum (n = 31), hepato-biliary system (n = 31) and pancreas (n = 31). Median overall survival (OS) was 13.4 months the esophagus, 13.3 months for the stomach, 29.7 months for the small bowel, 7.6 months for the colorectum, 7.9 months for the hepato-biliary system and 8.5 months for the pancreas. Irinotecan plus cisplatin (IP) and etoposide plus cisplatin (EP) were most commonly selected for GI-NEC and HBP-NEC. For patients treated with IP/EP (n = 160/46), the response rate was 50/28% and median OS was 13.0/7.3 months. Multivariate analysis among patients treated with IP or EP showed that the primary site (GI vs HBP; hazard ratio [HR] 0.58, 95% confidence interval [CI] 0.35–0.97) and baseline serum lactate dehydrogenase levels (not elevated vs elevated; HR 0.65, 95% CI 0.46–0.94) were independent prognostic factors for OS, while the efficacy of IP was slightly better than for EP (HR 0.80, 95% CI 0.48–1.33; P = 0.389). IP and EP are the most common treatment regimens for NEC of the digestive system. HBP primary sites and elevated lactate dehydrogenase levels are unfavorable prognostic factors for survival. A randomized controlled trial is required to establish the appropriate chemotherapy regimen for advanced NEC of the digestive system. This study was registered at UMIN as trial number 000005176.

Dynamic computed tomography is useful for prediction of pathological grade in pancreatic neuroendocrine neoplasm

“…9,12 In Japan, combination chemotherapy using platinum plus irinotecan is also used. 13 However, no satisfactory survival rates have been achieved in these cases yet. 9,13 In this way, there is significant difference between G1, G2, and G3 cases on therapeutic strategy and survival duration.…”

“…13 However, no satisfactory survival rates have been achieved in these cases yet. 9,13 In this way, there is significant difference between G1, G2, and G3 cases on therapeutic strategy and survival duration. Therefore, the identification of pathological grade is very important for unresectable case to be treated appropriately.…”

Neuroendocrine carcinoma of the ampulla of Vater causing ectopic adrenocorticotropic hormone-dependent Cushing's syndrome

“…Chemotherapeutic regimens, including cisplatin, irinotecan, etoposide, doxorubicin and vincristine have been reported. Large-scale retrospective analyses of advanced gastrointestinal and pancreatic neuroendocrine carcinoma by a Japanese group demonstrated that irinotecan plus cisplatin and etoposide plus cisplatin are the most commonly used regimens (16).…”

Unusual endoscopic findings of gastric neuroendocrine tumor

“…Kulke et al (18) reported a very low response rate to cisplatin plus irinotecan for extra-pulmonary NETs, however, Okita et al (10) reported the response rate to cisplatin plus irinotecan for gastric poorly differentiated NEC was 75%. Large -scale retrospective analyses for advanced neuroendocrine carcinoma of the digestive system by Japanese group demonstrate that irinotecan plus cisplatin (IP) and etoposide plus cisplatin (EP) are the most commonly used regimens (19). IP was the most commonly selected regimen, especially for the gastrointestinal tract in the Japanese study (19), while EP was the most commonly selected regimen in the Nordic study (20).…”

“…Large -scale retrospective analyses for advanced neuroendocrine carcinoma of the digestive system by Japanese group demonstrate that irinotecan plus cisplatin (IP) and etoposide plus cisplatin (EP) are the most commonly used regimens (19). IP was the most commonly selected regimen, especially for the gastrointestinal tract in the Japanese study (19), while EP was the most commonly selected regimen in the Nordic study (20). The response rate of IP was slightly better than that of EP for the treatment of NEC, even after adjusting patient background by multivariate analysis.…”