Abstract: |
Neuroendocrine tumors of the cervix are epithelial neoplasms with morphologic and immunophenotypic evidence of neuroendocrine differentiation. This group of tumors includes low-grade neuroendocrine tumors (grades 1–2) and high-grade neuroendocrine carcinomas (grade 3), the latter of which may be either small cell or large cell type. Overall, neuroendocrine tumors are rare comprising 2% of all invasive cervical neoplasms, the majority of which are small cell carcinomas. In most cases, high-risk human papillomavirus (HPV) type 16 or 18 is identified as an etiologic factor. Treatment for early-stage, high-grade neuroendocrine carcinomas is radical hysterectomy, followed by adjuvant chemotherapy or chemoradiation. The prognosis of low-grade neuroendocrine tumors of the cervix is related to the disease stage. Grade 1 neuroendocrine tumors of the cervix portend the best prognosis. High-grade neuroendocrine carcinomas are associated with poor clinical outcome for all stages, with high recurrence rates and a 5-year survival of 15% to 40%. © Springer Nature Switzerland AG 2021. |