_____________ is an uncommon anomaly that arises from failure of total fusion of the müllerian ducts and accompanies a double uterus (uterus didelphys). These and other anomalies of the external genitalia may be the manifestations of genetic syndromes, in utero exposure to diethylstilbestrol (DES) used to prevent threatened abortions in the 1940s through 1960s, or other disturbances associated with abnormalities in reciprocal epithelial-stromal signaling during fetal development.
Septate, or double, vagina
______________ is a remnant of columnar, endocervical-type epithelium that during embryonal development extends from the endocervix and covers the ectocervix as well as the upper vagina and is subsequently replaced by the squamous epithelium advancing upwardly from the urogenital sinus. Small patches of unreplaced glandular epithelium may persist focally into adult life.
Adenosis presents clinically as ____________areas contrasting with the normal pale-pink vaginal mucosa. On microscopic examination, adenosis consists of columnar mucinous epithelium indistinguishable from endocervical epithelium. Adenosis, while normally present in a small percentage of adult women, has been reported in 35% to 90% of women exposed to DES in utero. Rare cases of clear cell carcinoma ( Fig. 22-12 ) arising in DES-related adenosis were recorded in teens and young women in the 1970s and 1980s, resulting in discontinuation of DES treatment.
______________ are relatively common lesions found along the lateral walls of the vagina and derived from wolffian (mesonephric) duct rests. They are 1- to 2-cm fluid-filled cysts that occur in the submucosal location.
Gartner duct cysts
Other cysts, including mucus cysts, which occur in the proximal vagina, are derived from müllerian epithelium.
Another müllerian-derived lesion,_____________ (described later), may occur in the vagina and simulate a neoplasm.
Premalignant and Malignant Neoplasms
Most of the benign tumors of the vagina occur in reproductive-age women and include_________, _____________ and __________-.
tumors (stromal polyps), leiomyomas, and hemangiomas
The most common malignant tumor of
the vagina is ________________, followed by a primary squamous cell
carcinoma of the vagina.
carcinoma metastatic from the cervix
Infants may develop a unique, rare malignancy—_________________________
rhabdomyosarcoma (sarcoma botryoides).
VAGINAL INTRAEPITHELIAL NEOPLASIA AND SQUAMOUS CELL CARCINOMA
___________ is an extremely uncommon cancer (about 0.6 per 100,000 women yearly) accounting for about 1% of malignant neoplasms in the female genital tract.
Almost all of these tumors are squamous cell carcinomas associated with high oncogenic risk HPVs.
Primary carcinoma of the vagina
The greatest risk factor of primary ca of the vagina is a __________________; 1% to 2% of women with an invasive cervical carcinoma eventually develop a vaginal squamous cell carcinoma.
previous carcinoma of the cervix or vulva
_______________ of the vagina arises from a premalignant lesion, vaginal intraepithelial neoplasia, analogous to cervical squamous intraepithelial lesions (SILs, see under “Cervix”).
Squamous cell carcinoma
Most often the invasive tumor affects the ________________, particularly along the posterior wall at the junction with the ectocervix.
upper posterior vagina
The lesions in the lower two thirds of the vagina metastasize to the inguinal nodes, whereas upper lesions tend to involve the regional iliac nodes.
Know your anatomy
Also called sarcoma botryoides, this uncommon vaginal tumor is most frequently found in infants and in children younger than 5 years of age and consists predominantly of malignant embryonal rhabdomyoblasts.  These tumors tend to grow as polypoid, rounded, bulky masses that sometimes fill and project out of the vagina; they have the appearance and consistency of grapelike clusters (hence the designation botryoides = grapelike) ( Fig. 22-13 ).
On histologic examination, the tumor cells are small and have oval nuclei, with small protrusions of cytoplasm from one end, resembling a tennis racket.
Rarely, striations can be seen within the cytoplasm.
What is the reason behind the mistaken diagnosis the Malignant Rhabdomyosarcoma is benign?
Beneath the vaginal epithelium, the tumor cells are crowded in a so-called cambium layer, but in the deep regions they lie within a loose fibromyxomatous stroma that is edematous and may contain many inflammatory cells. For this reason the lesions can be mistaken for benign inflammatory polyps, leading to unfortunate delays in diagnosis and treatment.
These tumors tend to invade locally and cause death by penetration into the peritoneal cavity or by obstruction of the urinary tract. Conservative surgery, coupled with chemotherapy, seems to offer the best results in cases diagnosed sufficiently early.