Nejm 1987;316:514

Cause: Clear cell and squamous cell types; latter from human papilloma virus infection chronically (Nejm 1986;315:1052)

Pathophys: In clear cell type, adenosis (uterine cervical columnar cells) is present in vagina, then a 2nd carcinogen hits this susceptible tissue and the cells undergo malignant degeneration? May also start in cervix

Clear cell type increased by maternal estrogen (esp DES) use during first trimester 1/1000 in utero-exposed females get clear cell type; DES exposure does not incr any other cancers (Jama 1998;280:630). Peak onset age 19 yr, 91% are age 15-27 yr; h/o maternal estrog use in 72%

Squamous type, occurs usually in postmenopausal female; 30-50% of squamous type occur in women who have had a hysterectomy for human papillomavirus disease

Sx: Irregular menses or spotting in young female in clear cell type.

Si:

Carcinomatous mass in both types

In clear cell type, poor I₂ staining of vaginal mucosa = adenosis

Clear cell type is very malignant, survivals to date only in lesions <1 cm²; recurrences can be late

Clear cell, distant mets; squamous cell, local invasive disease

Lab:

Path: Pap smear in clear cell type shows adenosis present in 11% of cervical, 27% of vaginal pool specimens. In squamous type, 20% false-negative Paps; Paps post-hysterectomy, probably should be done if h/o HPV but otherwise useless (Nejm 1996;335:1559, 1599)

Biopsy areas that stain poorly with I₂, bleed, or have abnormal texture to touch

Rx:

Preventive (description of New York State clear cell screening program—Nejm 1981;304:47)

Surgery for both types as primary rx; radiation is equally effective and used for advanced invasive disease