Background: Neuroendocrine carcinoma of the vagina is extremely rare. Treatment options in pregnancy are complex owing to the aggressive tumor type and poor prognosis.
Case: We report the case of a gravid woman diagnosed with stage IVB neuroendocrine carcinoma of the vagina at 16 weeks of gestation. Disease responded to chemotherapy, and pregnancy was continued until onset of preterm labor at 28 5/7 weeks of gestation, with subsequent delivery of a viable fetus. Despite aggressive treatment with chemoradiation postpartum, the patient died of progressive disease 11 months after initial diagnosis.
Conclusion: A multidisciplinary team should review treatment options and potential complications with the patient. Because of the tendency toward early tumor dissemination, the optimal treatment for neuroendocrine carcinoma in pregnancy involves systemic chemotherapy.