Abstract

ObjectiveThis study aimed to explore the prognostic factors and outcomes of patients with neuroendocrine tumors (NETs) of the cervix and to determine appropriate treatment.MethodsA single-institution retrospective analysis of 172 patients with NETs was performed based on the new International Federation of Gynecology and Obstetrics (FIGO 2018) staging system.ResultsAmong the 172 eligible patients, 161 were diagnosed with small cell neuroendocrine carcinoma (SCNEC), six with large cell neuroendocrine carcinoma, four with typical carcinoid tumors and one with SCNEC combined with an atypical carcinoid tumor. According to the FIGO 2018 staging guidelines, 31 were stage I, 66 were stage II, 57 were stage III, and 18 were stage IV. The 5-year survival rates of patients with stage I–IV disease were 74.8%, 56.2%, 41.4% and 0%, respectively. The 5-year progression-free survival rates of patients with stage I–IV disease were 63.8%, 54.5%, 30.8% and 0%, respectively. In the multivariate analysis, advanced FIGO stage, large tumor and older age were identified as independent variables for 5-year survival in patients with stage I–IV disease. FIGO stage, tumor size and para-aortic lymph node metastasis were independent prognostic factors for 5-year progression-free survival in patients with stage I–IV disease. For the patients receiving surgery (n = 108), tumor size and pelvic lymph node metastasis were independent prognostic factors for 5-year survival, and pelvic lymph node metastasis for 5-year progression-free survival. In stage IVB, at least six cycles of chemotherapy (n = 7) was associated with significantly better 2-year OS (83.3% vs. 9.1%, p < 0.001) and 2-year PFS (57.1% vs. 0%, p = 0.01) than fewer than six cycles of chemotherapy(n = 11).ConclusionAdvanced FIGO stage, large tumor, older age and lymph node metastasis are independent prognostic factors for NETs of the cervix. The TP/TC and EP regimens were the most commonly used regimens, with similar efficacies and toxicities. Standardized and complete multimodality treatment may improve the survival of patients with NETs.

Highlights

  • Neuroendocrine cervical tumors (NETs) are a rare but highly aggressive form of cervical cancer (Gardner, Reidy-Lagunes & Gehrig, 2011; Satoh et al, 2014)

  • NETs are divided into four categories, typical carcinoid tumors, atypical carcinoid tumors (ACTs), Small cell carcinomas (SCNECs), and large cell neuroendocrine carcinomas (LCNECs), according to World Health Organization classifications

  • Well-differentiated typical and atypical carcinoids are categorized as low-grade neuroendocrine tumors (LGNETs), and the small and large cell types are categorized as high-grade neuroendocrine carcinomas (HGNECs)

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Summary

Introduction

Neuroendocrine cervical tumors (NETs) are a rare but highly aggressive form of cervical cancer (Gardner, Reidy-Lagunes & Gehrig, 2011; Satoh et al, 2014). NETs are divided into four categories, typical carcinoid tumors, atypical carcinoid tumors (ACTs), Small cell carcinomas (SCNECs), and large cell neuroendocrine carcinomas (LCNECs), according to World Health Organization classifications. Well-differentiated typical and atypical carcinoids are categorized as low-grade neuroendocrine tumors (LGNETs), and the small and large cell types are categorized as high-grade neuroendocrine carcinomas (HGNECs). Small cell carcinoma is the most common type of NET, yet it accounts for less than 5% of all cervical carcinomas (Albores-Saavedra et al, 1997; Viswanathan et al, 2004). We performed a retrospective review of 172 patients to analyze the clinicopathologic behaviors and prognostic factors of patients with NETs and to determine appropriate treatment. We evaluated the prognosis of NETs according to the FIGO 2018 staging system. We discuss the appropriate chemotherapy regimen and number of cycles for NETs

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