Elsevier

Gynecologic Oncology

Volume 112, Issue 3, March 2009, Pages 590-593
Gynecologic Oncology

Small cell neuroendocrine carcinoma of the cervix: Analysis of outcome, recurrence pattern and the impact of platinum-based combination chemotherapy

https://doi.org/10.1016/j.ygyno.2008.11.010Get rights and content

Abstract

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.

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], [3], [4], [5]. The natural history of this disease differs from the more commonly seen squamous cell or adenocarcinoma 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 extensive 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, definitive 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.

Section snippets

Methods

After obtaining Institutional Review Board approval, we used the prospectively-maintained Virginia K. Pierce database to identify all patients with the diagnosis of SCNEC from January 1990 to February 2007.

All available pathology slides and reports were obtained and examined by two gynecologic pathologists (KJP and HZ). Histologically, SCNEC is similar to small cell carcinoma of other sites. Characteristic findings on hematoxylin–eosin staining include a diffusely infiltrating tumor composed of

Results

Between January 1990 and February 2007, 22 patients were identified. Five of the 22 cases were excluded. In 2 cases slides were not available for evaluation and in 3 cases findings did not meet the histologic criteria for SCNEC. Of the 17 patients who were diagnosed SCNEC, 13 (77%) had positive staining for one or more neuroendocrine markers.

Clinical characteristics are listed in Table 1. All 8 patients selected to undergo primary surgery underwent a lymph node dissection (Table 2). Three of 8

Discussion

SCNEC is rare and the prognosis is limited due to the tumor's propensity for distant hematogenous metastases. Given the rarity of SCNEC, prospective studies are missing or had to be discontinued preterm because of inadequate accrual [13]. Retrospective reports have shown that the natural history of this disease differs from the more commonly seen squamous cell or adenocarcinoma of the cervix in that patients diagnosed with small cell carcinoma of the cervix are more likely to have lymph node

Conflict of interest statement

The authors have no conflicts of interest to declare.

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