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3,170 (Estimated new cases for 2014 in the United States)

Incidence of clear cell adenocarcinoma as a result of in utero diethylstilbestrol

(DES) exposure estimated at 1/1000

Stage at presentation
Stage I: 28%
Stage II: 43%
Deaths: Estimated 880 in 2014 Stage III: 16%
Median age: Squamous cell cancer (60–65 years); DES-related adenocarcinoma/clear cell (19 years) Stage IV: 13%


Daling JR et al. Gynecol Oncol 2002;84:263–270

Siegel R et al. CA Cancer J Clin 2014;64:9–29

Tedeschi C et al. J Low Genit Tract Dis 2005;9:11–18


Histologic Classification of Vaginal Neoplasia

VAIN (VAginal Intraepithelial Neoplasms)

These are pre-malignant lesions of the vaginal squamous epithelium that can develop primarily in the vagina or as an extension from the cervix. Histologically, VAIN is defined in the same way as cervical intraepithelial neoplasia (CIN). Classification includes three grades: Grade 1 (VAIN I = mild dysplasia); Grade 2 (VAIN II = moderate dysplasia); and Grade 3 (VAIN III = severe dysplasia or carcinoma in situ)

Invasive carcinoma:

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1. Squamous cell carcinoma 88%
2. Adenocarcinoma 5%
3. Other epithelial cell types (adenosquamous, adenoid cystic, undifferentiated) 1–2%
4. Mesenchymal tumors (leiomyosarcoma, sarcoma botryoides, endometrioid sarcoma) 2%
5. Mixed epithelial and mesenchymal tumors <1%
6. Other histologies (melanoma, sarcoma, yolk sac tumors, lymphoma, carcinoid, small cell) 3–4%


Higinia R et al. Vagina. In: Hoskins WJ, Perez CA, Young RC, eds. Principles and Practice of Gynecologic Oncology. 4th ed. Philadelphia: Lippincott-Raven, 2005:707–742

Zaino RJ, Robboy SJ, Kurman RJ. Diseases of the vagina. In: Blaustein's Pathology of the Female Genital Tract. 5th ed. New York: Springer-Verlag, 2002:178–195



VAIN (vaginal intraepithelial neoplasia):

  1. H&P, including bimanual examination, palpation, and colposcopic examination of the vagina, vulva, and cervix

  2. Multiple site-directed biopsies, including cervical and vulvar biopsies, to rule out invasive disease and metastatic lesions


Invasive carcinoma:

  1. H&P including bimanual examination and palpation of vagina

  2. Multiple site-directed biopsies, including cervical biopsies to rule out invasive disease and primary cervical cancer

  3. Studies allowable for staging as per FIGO guidelines: chest X-ray, cystoscopy, proctosigmoidoscopy, and intravenous pyelogram. Although not part of staging, pelvic MRI or CT scan may aid in planning of patient care

  4. If clinically warranted, barium enema and CAT scan or MRI


Staging is best performed by gynecologic and radiation oncologists with the patient under general anesthesia. Additional biopsies of the vagina should be done to determine the limits of abnormal vaginal mucosa


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TNM Category FIGO Stage Primary Tumor (T)
TX   Primary tumor cannot be assessed
T0   No evidence of primary tumor

Carcinoma in situ

T1 I Tumor confined to vagina
T2 II Tumor invades paravaginal tissues but not to pelvic wall

Tumor extends to pelvic wall


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