This study was performed to determine the outcome of systemic chemotherapy for extra-pulmonary poorly differentiated neuroendocrine carcinoma (NEC). Clinical data from 41 patients with unresectable or recurrent NECs, who received systemic chemotherapy, were collected from Chonnam National University Hwasun Hospital and analyzed retrospectively.
Patients had primary sites in the gut (n=17), pancreatico-hepatobiliary system (n=15), genitourinary (n=4), head and neck (n=3) and other sites (n=2). Etoposide plus cisplatin (EP) was most commonly selected for first line chemotherapy. For patients treated with EP, the response rate was 42.5%, median progression free survival (PFS) was 7.4 months, and median overall survival (OS) was 13.7 months. 53% of patients (n=22) were received second line chemotherapy. For patients treated with second-line chemotherapy, Irinotecan plus cisplatin (IP) was most commonly selected, the response rate was 9.5%, median progression free survival (PFS) was 3.0 months, and median overall survival (OS) was 6.8 months.
A randomized controlled trial is required to establish the appropriate chemotherapy regimen for extrapulmonary poorly differentiated NEC.