General
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• Poorly differentiated neuroendocrine tumor with characteristic morphological features, arising in vagina with no evidence of metastasis from any other site
• Tumor has histological, immunohistochemical and ultrastructural features similar to small cell carcinoma arising in any other organ including female genital tract (Patholog Res Int 2011;2011:306921)

Terminology
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• Previously called oat cell carcinoma

Epidemiology
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• Extremely rare (Rare Tumors 2013;5:e58, Patholog Res Int 2011;2011:306921)
• Most common site of involvement in female genital tract is cervix (Case Rep Obstet Gynecol 2013;2013:827037)
• Wide age range from 30's-80's (Rare Tumors 2013;5:e58, Patholog Res Int 2011;2011:306921, Case Rep Obstet Gynecol 2013;2013:827037, New York Medical Journal)

Sites
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• Can occur anywhere in vagina - no specific predilection

Clinical features
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• Vaginal bleeding, vaginal discharge, pelvic pain or an ulcerated mass (Rare Tumors 2013;5:e58, Case Rep Obstet Gynecol 2013;2013:827037, Cytojournal 2013;10:17, New York Medical Journal)
• Can present with obstructive symptoms like difficulty in urination (Patholog Res Int 2011;2011:306921)
• Can present with Cushing's syndrome due to ectopic ACTH production (Rare Tumors 2013;5:e58, J Low Genit Tract Dis 2008;12:140, Cytojournal 2013;10:17)
• Paraneoplastic syndromes such as syndrome of inappropriate antidiuretic hormone, hypercalcemia, or hypoglycemia may also be seen (Cytojournal 2013;10:17)

Diagnosis
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• Clinical findings and pathological features with no evidence of similar tumor elsewhere in the body

Laboratory
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• Tumor markers can be elevated, including NSE (Rare Tumors 2013;5:e58)
• Chromogranin A assay can also be done in plasma or serum (Patholog Res Int 2011;2011:306921)

Radiology
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• Whole body screening is necessary to rule out metastasis from other sites such as lung (Rare Tumors 2013;5:e58)
• Somatostatin receptor scintigraphy (SRS) can be performed for tumor localization (Patholog Res Int 2011;2011:306921)

Prognostic factors
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• Poor prognosis and aggressive behavior
• Most patients present with metastasis and die within 2 years (Rare Tumors 2013;5:e58)

Case reports
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• 32 year old woman with vaginal small cell carcinoma mimicking Bartholin's gland abscess (Gynecol Oncol 1998;68:297)
• 51 year old woman (Oncology 2000;58:300)
• 61 year old woman with rare ectopic ACTH syndrome in small cell carcinoma of the vagina (J Low Genit Tract Dis 2008;12:140)
• 65 year old woman (Cancer 1992;70:784)
• 69 year old woman (Gynecol Oncol 2003;88:451)
• Small cell carcinoma of the vagina causing Cushing's syndrome (Gynecol Oncol 1997;65:526)

Treatment
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• No standard therapy
• Various management options are surgical resection, chemotherapy, radiotherapy or combinations of these therapies
• Combination cisplatin + etoposide can be used (Rare Tumors 2013;5:e58)
• Small lesions localized to upper vagina can be treated by radical hysterectomy, partial vaginectomy, and pelvic lymphadenectomy (Case Rep Obstet Gynecol 2013;2013:827037)

Clinical images
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Vaginal tumor

MRI, PET-CT

Solid mass

Gross description
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• Hemorrhagic polypoid to greyish white globular mass (Rare Tumors 2013;5:e58, Patholog Res Int 2011;2011:306921)

Gross images
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No hemorrhage or necrosis

Micro description
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• Diffuse solid pattern or rosette-like structures (Rare Tumors 2013;5:e58)
• Scant cytoplasm, high N/C ratio with fine powdery chromatin with inconspicuous nuclei (Patholog Res Int 2011;2011:306921, Case Rep Obstet Gynecol 2013;2013:827037)
• Cells may show nuclear molding and smear artifact
• Mitotic figures are common and karyorrhectic debris can be identified in the background

Micro images
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Various images

Diffuse infiltration of small darkly stained cells

Scant cytoplasm, inconspicuous nucleoli

H&E, AE1/AE3, chromogranin A, p63

Synaptophysin+

Chromogranin

NSE

Chromogranin, synaptophysin

Cytology description
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• Tumor cells are dispersed as single cells or arranged as loosely cohesive sheets or gland-like aggregates
• Cells range from small to large with pleomorphic, angulated hyperchromatic nuclei that may show nuclear molding and smear artifact
• Atypical cells have high N/C ratio or bare hyperchromatic nuclei (Rare Tumors 2013;5:e58)
• Mitotic figures are common and karyorrhectic debris can be identified in the background (Cytojournal 2013;10:17, Diagn Cytopathol 2001;24:46)

Cytology images
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ThinPrep, Pap

Positive stains
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• Synaptophysin, chromogranin, non-specific enolase (Rare Tumors 2013;5:e58, Patholog Res Int 2011;2011:306921)
• Cytokeratin AE1/AE3, CD57 (Case Rep Obstet Gynecol 2013;2013:827037)
• CD56, carcinoembryonic antigen (CEA) can also be positive
• Variable immunoreactivity for serotonin, intestinal polypeptide or somatostatin (Cytojournal 2013;10:17)
• TTF1 can be positive (Cytojournal 2013;10:17, New York Medical Journal)

Negative stains
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• p63 (Case Rep Obstet Gynecol 2013;2013:827037)
• CD3, CD20, CD45

Electron microscopy description
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• Occasionally cytoplasmic, neurosecretory-type granules can be present which is a feature of neuroendocrine cells (J Reprod Med 1989;34:486, Patholog Res Int 2011;2011:306921)

Differential diagnosis
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• Endometrial adenocarcinoma
• Follicular cervicitis
• High-grade squamous intraepithelial lesion (of small cell type)
• Lymphoma (especially on cytology smears)
• Metastatic adenocarcinoma or neuroendocrine tumors from other sites should be ruled out
• Non-keratinizing squamous cell carcinoma (Diagn Cytopathol 2001;24:46, Cytojournal 2013;10:17)
• Undifferentiated carcinoma

Additional references
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• Arch Pathol Lab Med 2004;128:857, Arch Gynecol Obstet 2005;271:316, Diagn Cytopathol 2001;24:46

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