Primary small cell neuroendocrine carcinoma of the vagina: a clinicopathologic study.
BACKGROUND
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.


METHODS
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.


METHODS
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.


CONCLUSIONS
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.
Department of Pathology, The University of Texas Medical Branch, Galveston, TX 77555-0588, USA.
Zhanyong
Z
Bing
Lyuba
L
Levine
Joseph A
JA
Lucci
Sandra S
SS
Hatch
Mahmoud A
MA
Eltorky
D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000556:secondary; D001918:Brachytherapy; D018288:Carcinoma, Small Cell / Q000737:chemistry / Q000150:complications / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy / Q000556:secondary; D053379:Chromogranin A; D002864:Chromogranins / Q000032:analysis; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D005047:Etoposide / Q000008:administration & dosage; D017809:Fatal Outcome; D005260:Female; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D007633:Keratins / Q000032:analysis; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000032:analysis; D009687:Nuclear Proteins / Q000032:analysis; D011867:Radioisotope Teletherapy; D016708:Synaptophysin / Q000032:analysis; D000074482:Thyroid Nuclear Factor 1; D014157:Transcription Factors / Q000032:analysis; D014623:Vaginal Diseases / Q000209:etiology; D014625:Vaginal Neoplasms / Q000737:chemistry / Q000150:complications / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy
D053379:Chromogranin A; D002864:Chromogranins; C000614412:NKX2-1 protein, human; D009363:Neoplasm Proteins; D009687:Nuclear Proteins; D016708:Synaptophysin; D000074482:Thyroid Nuclear Factor 1; D014157:Transcription Factors; D007633:Keratins; D005047:Etoposide; D002945:Cisplatin