Small cell neuroendocrine carcinoma of the cervix: Analysis of outcome, recurrence pattern and the impact of platinum-based combination chemotherapy O. Zivanovic a , M.M. Leitao Jr. a , K.J. Park b , H. Zhao b , J.P. Diaz a , J. Konner d , K. Alektiar c , D.S. Chi a , N.R. Abu-Rustum a , C. Aghajanian d, ⁎ a Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA b Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA c Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA d Department of Medicine, Memorial Sloan-Kettering Cancer Center,1275 York Ave., New York, NY 10065, USA abstract article info Article history: Received 7 October 2008 Available online 24 December 2008 Keywords: Small cell neuroendocrine carcinoma Cervix Platinum-based chemotherapy Objectives. To analyze progression-free (PFS) and overall survival (OS) in patients with small cell neuroendocrine carcinoma of the cervix (SCNEC), and to determine whether platinum-based combination chemotherapy is beneficial for this population. Methods. We performed a retrospective analysis of all patients with SCNEC who were treated at our institution between 1/1990 and 2/2007. Patients were excluded if pathologic diagnosis was not confirmed at our institution. Standard statistical methods were utilized. Results. Seventeen patients met inclusion criteria. The estimated 3-year PFS and OS rates for the entire group were 22% and 30%, respectively. Median time to progression was 9.1 months. Extent of disease was the only significant prognostic factor. Median OS for patients with early stage disease (IA1–IB2) was 31.2 months and 6.4 months for patients with advanced stage disease (IIB–IV, P = 0.034). In the early-stage disease group, the 3-year distant recurrence-free survival rate was 83% for patients who received chemotherapy and 0% for patients who did not receive chemotherapy as part of their initial treatment (P = 0.025). The estimated 3-year OS rate was 83% for patients who received and 20% for patients who did not receive chemotherapy as part of their initial treatment (P = 0.36). Conclusion. Given the rarity of SCNEC this retrospective analysis is limited by a small number of patients. However, the natural history of this rare disease is akin to small cell lung cancer and the prognosis is poor due to the tumor's propensity for distant spread. The treatment should conform to the treatment of small cell lung cancer. © 2008 Elsevier Inc. All rights reserved. Introduction Small cell neuroendocrine carcinoma of the uterine cervix (SCNEC) was first described in 1957 [1]. It is a rare finding, representing 2%–5% of all cervical malignancies [2–5]. The natural history of this disease differs from the more commonly seen squamous cell or adenocarci- noma of the cervix [6]. Patients diagnosed with SCNEC are more likely to have lymph node metastases and lymph vascular space invasion, and their clinical course is frequently marked by local and distant failure [5,7,8]. Five-year survival rates vary from 0% to 30% [9,10]. Long- term survival can be achieved only in patients with limited stage disease [9,11,12]. Like small cell lung cancer, outcome of SCNEC is significantly associated with the extent of disease. Limited stage disease, which is defined as disease that can be encompassed within a radiation field, is treated with curative intent with combined modality therapy, with approximately 30% of patients achieving a cure. Patients with exten- sive stage disease – defined as disease outside of these confines – have a dismal prognosis with few surviving beyond two years [9,13]. Due to the rarity of this disease, it has been difficult to conduct prospective trials. Based on retrospective studies and treatment paradigms established for small cell lung carcinoma, many clinicians favor the use of combined modality therapy (surgery followed by combined chemoradiation therapy) for limited stage disease, defini- tive chemo-radiation therapy for locoregional advanced disease, and palliative chemotherapy for metastatic disease. It is not known if these treatment modalities ultimately improve survival. Clear treatment recommendations for SCNEC have not been defined. We performed a retrospective review to explore the outcomes and pattern of recurrence in patients with SCNEC and to determine the effects of chemotherapy on the recurrence-free and overall survival in patients with early stage disease. Gynecologic Oncology 112 (2009) 590–593 ⁎ Corresponding author. Fax: +1 212 717 3214. E-mail address: gynbreast@mskcc.org (C. Aghajanian). 0090-8258/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.ygyno.2008.11.010 Contents lists available at ScienceDirect Gynecologic Oncology journal homepage: www.elsevier.com/locate/ygyno