Multicenter retrospective analysis of systemic chemotherapy in poorly differentiated neuroendocrine carcinoma of the digestive system.

Abstract

274 Background: Poorly differentiated neuroendocrine carcinoma (PDNEC) is a rare and aggressive disease. No standard regimen has yet been established for advanced PDNEC, although regimens for small-cell lung carcinoma such as irinotecan + cisplatin (IP) or etoposide + cisplatin (EP), are usually adopted. The aim of this study was to investigate the outcomes according to the patient’s characteristics and treatment regimens for patients with PDNEC of the digestive system. Methods: Data was collected from the medical records of patients at 23 hospitals. The selection criteria were as follows: 1) histologically proven PDNEC, small cell carcinoma, mixed endocrine-exocrine carcinoma with a PDNEC component, or histologically proven neuroendocrine tumor with rapidly progressive clinical course; 2) primary tumor arising from the gastrointestinal tract (GI) or the hepato-biliary-pancreatic system (HBP); and 3) inoperable or recurrent disease treated with systemic chemotherapy between April 2000 and March 2011. Results: There were 258 patients (pts). The median age was 62.5 years (range, 26-81); male/female, 182/76 pts; the primary site was the esophagus/stomach/small bowel/colorectum/hepato-biliary system/pancreas in 85/70/6/31/31/35 pts. According to these primary sites, the median overall survival period (mOS) was 13.4/13.3/29.7/7.6/7.9/8.5 months, respectively. The most commonly used regimen was IP (160 pts, 62%), followed by EP (46 pts, 18%). For the patients treated with IP/EP, the response rates (RR) were 50%/27%, the progression free survival periods (mPFS) were 5.2/4.0 months, and mOS were 13.0/7.3 months. The subgroup outcome data for patients with HBP or GI cancers are shown in Table. A multivariate analysis demonstrated that a primary HBP cancer (HR=1.96, p=0.002), and a poor PS (HR=2.33, p=0.01) were independent unfavorable prognostic factors. Conclusions: PDNEC of the HBP has a poorer prognosis than GI. IP was the most commonly selected treatment regimen, and seemed to have a favorable treatment outcome. [Table: see text]

Chemotherapy for advanced poorly differentiated pancreatic neuroendocrine carcinoma

“…In regard to EP therapy, Moertel et al reported a response rate of 67%, a response duration of 8 months, and a median survival time of 19 months , while Mitry et al reported a response rate of 41.5% and a median survival time of 17.6 months ; according to major reports published to date, the response rate ranges from 14% to 67% and the median survival time ranges from 5.8 to 19 months (Table ). In regard to IP therapy, the response rate has been reported to range from 7% to 60% and the median survival time from 10 to 14 months (Table ). All of these reports, nevertheless, are from small‐scale retrospective studies conducted in patients with NEC including gastrointestinal NEC and P‐NEC, and there has been no report of prospective studies carried out on a sufficient number of patients with P‐NEC alone.…”

“…[15]; according to major reports published to date, the response rate ranges from 14% to 67% and the median survival time ranges from 5.8 to 19 months [5,15,16,19,20] ( Table 2). In regard to IP therapy, the response rate has been reported to range from 7% to 60% and the median survival time from 10 to 14 months [20][21][22][23][24][25] (Table 3). All of these reports, nevertheless, are from small-scale retrospective studies conducted in patients with NEC including gastrointestinal NEC and P-NEC, and there has been no report of prospective studies carried out on a sufficient number of patients with P-NEC alone.…”

“…In a multicenter retrospective analysis of systemic chemotherapy for advanced poorly differentiated NEC carried out in 2011 by Yamaguchi et al , 34 of 52 patients with hepatobiliary and pancreatic NEC received EP therapy and the remaining 18 patients received IP therapy. The response rates to EP therapy and IP therapy were 12% and 39%, respectively, and the median survival times were 6.9 months and 10.1 months, respectively; thus, the results tended to be more favorable for IP therapy.…”

Clinicopathologic Characteristics and Multidisciplinary Treatment of Neuroendocrine Carcinoma of Gallbladder： Report of Three Cases with an Update

“…In 2012 ASCO, a multicenter retrospective study was reported. For 206 GEP-NEC patients treated with IP/EP, the RR were 50%/27%, the PFS were 5.2/4.0 months, and mOS were 13.0/7.3 months [15]. Similar with GEP-NEC, 3 cases received platinum-based chemotherapy and achieved long survival (Figure 8).…”

Diagnosis, Assessment, and Therapeutic Strategy for Colorectal Mixed Adenoneuroendocrine Carcinoma

“…Yamaguchi et al [59,60] reported that the combination of cisplatin and irinotecan offers a response rate of 51% and a progression-free survival of 5.2 months for neuroendocrine carcinomas of various organs, as well as an overall survival of 7.6 months for colorectal neuroendocrine carcinomas in particular. Hainsworth et al [61] used a more aggressive regimen including paclitaxel, carboplatin, and etoposide and reported a response rate of 53% (complete response rate: 15%) and a median progression-free survival of 7.5 months.…”