Objective To explore the strategy of pregnancy-preserving and maternal- fetal management in patients with primary gynecologic neuroendocrine tumors (gNETs) during pregnancy. Methods We performed whole genome sequencing (WGS) for analyzing maternal and fetal somatic and germline single nucleotide variations (SNVs) and small insertions and deletions (InDels) for a 29-year-old pregnant woman diagnosed with stage IB2 large cell neuroendocrine carcinoma (LCNEC) and adenocarcinoma in the cervix. A systematic literature review was performed to explore the strategies for treatment of such rare histological type while

maintaining pregnancy. Results Global case analysis of cervical NETs during pregnancy suggested that negative lymph node metastasis and an early FIGO stage were potentially associated with a good prognosis of the patients. In the case presented herein, a pregnancy-preserving strategy was adopted and favorable maternal-fetal outcomes were achieved after neoadjuvant

chemotherapy, radical surgery and postoperative systemic chemotherapy. At 35 + 5 weeks, the fetus was delivered by caesarian

section, and the patient has by now had a disease-free survival of 19 months postoperatively. WGS analysis revealed 6 missense

somatic pathogenic mutations in two cancer tissues of the patient, and among them KARS and VEGFA were related with targeted

therapy. Five pathogenic germline variants were detected in the patient and her son, suggesting the necessity of a long-term follow-up schedule including precise genetic counselling for both the mother and the child. Conclusion Although gNETs in pregnancy are rare and highly risky, pregnancy-preserving managements of gNETs can still be considered and favorable maternal-fetal outcomes are possible with proper assessment of the clinical indications and implementation of multimodal treatments. Precise treatment and follow-up strategies based on the results of WGS for risk-reducing intervention of cancer recurrence or occurrence can potentially benefit the patient and the neonate.