Introduction: Primary vaginal malignant tumors are rare, especially neuroendocrine tumors. The primary small cell carcinoma of the vagina is an aggressive tumor with poor prognosis because of early recurrence and metastasis. Aim: to report the complexity of treatment of primary neuroendocrine carcinoma of vagina, and its metastasis. Material &methods: we report a retrospective case of primary neuroendocrine carcinoma of vagina treated in our institution. Results: We report a case of a 37-year-old woman who was diagnosed with a small cell carcinoma of the vagina without distant metastasis. The primary vaginal tumor was treated by surgery, brachytherapy, and chemotherapy. The first recurrence appeared seven years after initial diagnosis, which was a pulmonary metastasis, treated surgically. Seven years after this recurrence, she was treated with chemotherapy and surgery for another pulmonary metastasis. Fifteen years after the initial diagnosis, the patient underwent surgery for brain metastasis. She died eighteen months after the third recurrence. Conclusions: This case splendidly illustrates a long survival term of a tumor known with poor prognosis.
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