Imaging and staging of neuroendocrine cervical cancer.

Abstract

Neuroendocrine cervical cancer (NECC) is a rare and aggressive subtype of cervical cancer, accounting for less than 2% of cervical tumors. They are divided into low-grade and high-grade tumors. High-grade NECC is associated with human papillomavirus (HPV) 18 and to a smaller extent type 16. The most common molecular alterations in NECC include PIK3CA, KRAS, and TP53 mutations. Immunohistochemical staining for CD56, synaptophysin, and chromogranin is a helpful tool in the diagnosis. NECCs pose a significant clinical and therapeutic challenge because of their aggressive nature which is explained by their tendency towards early nodal and hematogenous spread. They have a median survival of 21-22 months, compared to 10 years in cervical squamous cell carcinomas. NECCs have a homogeneous high T2 signal intensity, homogeneous contrast enhancement and lower ADC values in MRI, compared to non-neuroendocrine tumors of the cervix. It is recommended to treat NECC with a multimodality therapeutic approach combining radical hysterectomy, systemic chemotherapy, and radiotherapy. The objective of this manuscript is to address the pathogenesis of NECC, elaborate the role of radiological imaging in the diagnosis and staging of NECCs, evaluate their prognosis, and summarize the suggested management plans for this lethal disease.