HPV18-positive Small Cell Neuroendocrine Carcinoma of the Uterine Cervix Treated With Immunotherapy: A Case Report.

BACKGROUND

Small cell neuroendocrine carcinoma of the cervix (SCNEC) has an extremely poor prognosis. We report a case of recurrent SCNEC with CD274 [Programmed Death-Ligand 1 (PD-L1)] gene amplification and HPV18 positivity, which showed a favorable response to the immune checkpoint inhibitor (ICI) nivolumab.

CASE REPORT: A 42-year-old woman presented with a lower abdominal mass and was referred to our department. Imaging revealed a uterine cervical tumor with multiple enlarged pelvic lymph nodes. Biopsy confirmed HPV18-positive SCNEC. After one cycle of neoadjuvant chemotherapy, surgery was performed, revealing stage IIIC2p (ypT1b1, ypN1, M0) SCNEC with regional lymph-node invasion, followed by adjuvant chemotherapy. Two months later, enlargement of the para-aortic and left pelvic lymph nodes was detected. Concurrent chemoradiotherapy (CCRT) was administered, resulting in a reduction of lymphadenopathy. Seven months later, an enlarged right supraclavicular lymph node was detected. Nivolumab therapy was initiated because CD274 (PD-L1) gene amplification was detected in tumor tissue. The lymph node decreased in size, and no signs of relapse had been observed for more than three years.

CONCLUSION

Comprehensive multimodal therapy, including standard surgery, neoadjuvant and adjuvant chemotherapy, chemoradiation for recurrent lymph nodes, and immune checkpoint inhibition, can achieve a remarkable response in advanced SCNEC harboring CD274 (PD-L1) amplification and HPV18 infection.