Primary Small Cell Neuroendocrine Carcinoma of Vagina - A Rare Entity at a Rare Site

Primary small cell neuroendocrine carcinoma of the vagina is extremely rare and its clinical behavior is aggressive. To our knowledge, only 25 patients with this tumor have been reported in the literature to date .We describe a case of primary neuroendocrine carcinoma arising from the anterior vaginal wall of a 60 year old woman who presented with dysuria and growth in vagina. The mass was 2 x 1 x 1 cm in size. Histopathological study revealed stratified squamous epithelium with underlying tumor tissue arranged in solid sheets. The cells were small sized with scanty ill defined cytoplasm, hyperchromatic nuclei with coarse to finely granular chromatin and inconspicuous nucleoli. The typical neuroendocrine architecture was absent excluding the possibility of typical and atypical carcinoid. Immunohistochemistry for neuroendocrine marker confirmed the diagnosis. Primary small cell neuroendocrine carcinoma of vagina is very rare. Infrequently small cell component is seen in association with a primary vaginal adenocarcinoma. In the present case there was no associated adenocarcinomatous component in the vagina. Chest X-ray and lower genital tract of the patient also did not reveal any abnormality. So a confirmed dignosis of primary small cell neuroendocrine carcinoma of vagina was rendered.