Neuroendocrine Carcinoma of Cervix: Comprehensive Review of Epidemiology, Pathology, and Advanced Imaging Modalities

Neuroendocrine carcinoma of the cervix (NECC) is a rare subtype of cervical cancer, making up only about 1%-2% of cases, yet it contributes disproportionately to cancer-related deaths. These tumors are classified into well-differentiated tumors and poorly differentiated small- and large-cell neuroendocrine carcinomas. High-grade NECC is associated with high-risk HPV18 and HPV16 and usually has alterations in TP53, PIK3CA, KRAS, and the Rb and DNA damage response pathways. Immunohistochemical expression of markers such as synaptophysin, chromogranin, CD56, and INSM1 supports the diagnosis. These tumors tend to invade lymphovascular spaces early, involve pelvic and para-aortic nodes, and spread hematogenously at an early stage. On MRI, NECC often appears as a fairly homogeneous, T2-hyperintense cervical mass with marked diffusion restriction and correspondingly low apparent diffusion coefficient values. Treatment is generally multimodal and depends on stage and resectability, most often combining radical hysterectomy with platinum-based chemotherapy and radiotherapy. In this review, we bring together current data on the epidemiology, pathology, imaging characteristics, and treatment strategies for NECC, with attention to how advanced imaging can be used to guide management.