Introduction/Background Primary small cell neuroendocrine tumor of the vagina was first reported by Sculty et al in 1984. This is a rare neoplasm with only a few cases reported in literature. This type of tumor exhibits aggressive clinical behavior and has a poor prognosis. Since the disease is so rare, there is still no consensus regarding the optimal therapy. This is a case report of a malignant polypoid mass in a postmenopausal woman and was managed by surgical approach followed by radiation therapy.

Methodology Treatment modalities were surgical excision of the mass with ligation of bleeders followed by radiation therapy.

Results Standard therapy has not been established because of the very small number of cases. Surgical resection with partial or total vaginovulvectomy with bilateral or ipsilateral lymphadenectomy followed by radiotherapy and chemotherapy may provide increased local control. Radical hysterectomy, partial vaginectomy and pelvic lymphadenectomy can be done to small lesions involving the upper third of the vagina. Surgery was proven to be the main therapeutic approach but surgery alone has a high recurrence rate of 70–75%.

Conclusion Small cell neuroendocrine tumor of the vagina is a rare tumor with aggressive behavior and poor prognosis commonly seen in postmenopausal women as in this case. Its clinical behavior is usually early with widespread dissemination. Most patient, like in this case, presents with an introital mass associated with vaginal bleeding. Mortality is secondary to distant metastasis regardless of the extent of the tumor upon diagnosis. Standard therapy has not been established for the disease, which needs further cases and discussions.

Disclosure Nothing to disclose