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.