Introduction
Small cell neuroendocrine carcinoma of the uterine cervix (SCNEC) is a rarely seen tumor that accounting for <3% of all cervical cancers [1-3], with an age-standardized incidence rate (ASR) of 0.1 per 100,000 women[4]. The nature of SCNEC significantly differs from the squamous cell or adenocarcinoma of the cervix, and is characterized by a high incidence of early nodal and distant metastases [5-7], resulting in poor prognosis with 5-year survival rates lower than 30%[2-4, 6-7]. Hence, systemic treatment was essential for the treatment of SCNEC even of early stage, to reduce the risk of distant metastasis. Additionally, resemble to the small cell lung cancer (SCLC), prognosis of SCNEC is significantly associated with the extent of disease. As reported by Zivanovic et al, patients with limited-stage disease (IA-IIA) had significantly longer survival than those with advanced-stage disease (IIB-IV) (median overall survival, 31.2 vs6.4 months) [8]. Advanced stage, large tumor size, presence of lymph node metastases was shown to be adverse prognostic factors for patients with SCNEC [9-13]. Thus, systemic treatment intensification was required for the patients with advanced stage or bulky SCNEC.
Nonetheless, the role of chemotherapy in SCNEC was under determined because of the limited evidence in SCNEC owing to the rarity of this disease. Hence, the current treatment option for SCNEC is heavily based on experience from SCLC and data from retrospective studies of small sample sizes. Empirically, concerning the aggressive nature of this disease, multimodality regimens are recommended for SCNEC patients even at an early stage. Clinicians favor to choose radical hysterectomy in combination with systemic chemotherapy (with or without radiation therapy) for early-stage disease, definitive chemo-radiation therapy for locoregionally advanced disease, and palliative chemotherapy for metastatic disease[1,7,14-15]. However, crucial management issues in terms of chemotherapy regimen and courses for SCNEC remain controversial. Consequently, SCNEC remains a great therapeutic challenge for gynecological oncologists. Here, we then conduct this study by including a relative large-scale well-characterized cohort encompassing early to locally advanced stages of disease from an academic center, aiming to investigate the appropriate chemotherapy algorithm for SCNEC.