Primary poorly differentiated carcinoma of the vagina with focal neuroendocrine differentiation: a tumour with aggressive behaviour

Abstract

Primary vaginal neuroendocrine tumours are extremely rare but aggressive. We report a case of primary poorly differentiated vaginal carcinoma with focal neuroendocrine differentiation. The clinical stage was cT3N1M0, FIGO stage III. The patient received six cycles of cisplatin-based concurrent chemoradiation therapy (CCRT) followed by six cycles of adjuvant chemotherapy (IEP protocol: ifosfomide, epirubicin and cisplatin). Pelvic MRI scans obtained after treatment completion revealed no residual tumour in the vagina. However, the patient experienced severe dyspnoea 2 months later. Chest X-ray revealed a reticulonodular interstitial pattern over bilateral lungs with suspicion of lymphangitic carcinomatosis. Further chest, abdominal and pelvic CT scans showed bilateral lung metastases with multiple mediastinal, left lower neck and left axilla, intra-abdominal and pelvic lymphadenopathies. For this rare tumour, cisplatin-based CCRT followed by IEP protocol adjuvant chemotherapy may have a limited treatment effect. Further studies are necessary to provide more information on clinical management.