Primary Small Cell Neuroendocrine Carcinoma of the Vagina: A Clinicopathologic Study

Abstract

Context.—Primary small cell neuroendocrine carcinoma of the vagina is extremely rare, and its clinical behavior is aggressive. To our knowledge, 22 patients with this tumor have been reported in the English literature to date. Objective.—To investigate 3 patients with this tumor clinically and pathologically. Design.—The pathology database at the University of Texas Medical Branch at Galveston was searched, and 3 cases of primary small cell neuroendocrine carcinoma of the vagina were found. The histologic, immunohistochemical, and ultrastructural profiles of the tumors were investigated. The medical charts of the patients were reviewed, and the patients were followed up. Patients.—Women with the diagnosis of primary small cell neuroendocrine carcinoma of vagina. Results.—All 3 patients presented with advanced disease, and 2 patients died within 4 months of the initial diagnosis. One 38-year-old patient was newly diagnosed, and her clinical outcome had not yet been determined. The histologic features of all 3 tumors were similar to those of their pulmonary counterpart. All cases were positive for cytokeratin, chromogranin A, and synaptophysin. The expression pattern of thyroid transcription factor 1 was examined in all 3 patients, of whom 2 were negative and 1 was positive with negative clinical and radiologic thyroid or pulmonary findings. Ultrastructural evaluation showed scattered intracytoplasmic electron-dense neurosecretory granules. Conclusion.—Primary small cell neuroendocrine carcinoma of the vagina has histologic, immunohistochemical, and ultrastructural features similar to those of its pulmonary counterpart. Because thyroid transcription factor 1 can be positive, it should not be used to differentiate primary from metastatic disease. The current therapies have usually resulted in poor outcomes, and new therapeutic modalities should be explored.

Human Papilloma Virus 18-Positive Submucosal Small Cell Neuroendocrine Carcinoma of the Vagina: An Immunohistochemical and Genomic Study

“…16 SCLS, LCNEC, and vaginal SCNEC are often positive for TTF-1; therefore, IHC for TTF-1 is not useful for the differential diagnosis of primary and metastatic vaginal NEC. 8,16 Although the 2 cases in our study were negative for TTF-1, a comprehensive judgment by clinical findings and diagnostic imaging for the search of the primary site were essential for the differential diagnosis. Primary vaginal MCC is another differential diagnosis for our cases because of its small cell morphology and negative TTF-1 expression.…”

“…2-5 As primary uterine cervical SCNEC and large cell NEC (LCNEC) are more aggressive and more likely to metastasize than squamous cell carcinoma of the cervix, 6,7 primary vaginal SCNEC is considered as an aggressive tumor with a very poor prognosis. 8 With respect to primary uterine cervical NEC, high-risk human papillomaviruses (HPVs), such as types 16 and 18, are known to be associated with the onset of carcinogenesis. 9 Consequently, prophylactic HPV16 and HPV18 vaccination may prevent most SCNEC and LCNEC of the uterine cervix.…”

Secondary small cell vaginal cancer after operative therapy for endometrial cancer

“…The small cell carcinoma is rare and accounts for 2% of malignancies diagnosed in the genital tract of female. Most common sites were the ovary and uterine cervix in previous reports [1][2][3][4][5][6]. It resembles small cell lung cancer.…”

“…Small cell carcinoma is one of types of neuroendocrine cancers. Most common site is the lungs, accounting for 20% of lung cancers [1][2][3][4][5][6]. The small cell carcinoma is rare and accounts for 2% of malignancies diagnosed in the genital tract of female.…”

“…Neuroendocrine carcinoma of the genital tract of the female is rare, and most common sites were the ovary and uterine cervix in previous reports [1]. Primary neuroendocrine vaginal cancer is very rare, and approximately 40 cases have been reported [1][2][3][4][5][6]. There is no reported case of secondary neuroendocrine vaginal cancer.…”