Benign & Malignant Conditions of the Vulva & Vagina

Question 1

Androgen insensitivity is caused by a genetic deficiency in androgen receptors resulting in external female phenotypic development + undescended testes. What is the karyotype and inheritance pattern associated with androgen insensitivity?

Answer 1

Karyotype = 46, XY

Most commonly an X-linked recessive disorder

Question 2

Most common benign solid tumors of the vulva; usually slow growing (most range from 1-10 cm but can become quite large)

Answer 2

Fibromas

Question 3

Most common vulvovaginal tumor that presents with unilateral swelling

Answer 3

Bartholin’s cyst

Question 4

What is the next step in management of a women age 40+ y/o that presents with a Bartholin’s cyst?

Answer 4

Biopsy! Need to rule out a Bartholin’s carcinoma

[Bartholin’s gland carcinoma usually presents as a painless vulvar mass without history of previous bartholin’s gland disorders. Tx is radical vuluvectomy and bilateral lymphadenectomy with postoperative radiation; recurrence is common]

Question 5

90% of vulvar neoplasms are what type?

Answer 5

Squamous cell carcinomas

Question 6

Condition caused by masculinization in utero of the female fetus, usually presenting with clitoromegaly, hypospadic urethral meatus, and a malpositioned vaginal orifice

Answer 6

Female pseudohermaphroditism

Question 7

Condition commonly resulting from mosaicism and can occur with varying degrees of virulization and mullerian development

Answer 7

Male pseudohermaphroditism

Question 8

Severe pruritic raised yellow retention cyst in the axilla and labia majora and minora resulting from keratin-plugged inflammation of apocrine glands

Answer 8

Fox-Fordyce disease

Question 9

Small, fleshy red outgrowth at the distal edge of the urethra

Answer 9

Urethral caruncles

[in children these are caused by spontaneous prolapse of urethral epithelium; in postmenopausal women secondary to contraction of hypoestrogenic vaginal epithelium]

Question 10

Rare condition in which one or more of the minor vestibular glands becomes infected; presents with 1-4 mm erythematous lesions that are extremely tender, and pt complains of severe introital dyspareunia and occasionally vulvar pain

Answer 10

Vulvar vestibulitis

Question 11

Local thickening of epithelium that results from prolonged itch-scratch cycle; presents with pruritis and exam reveals white or reddish thickened, leathery raised surface appearing similar to psoriasis

Answer 11

Lichen simplex chronicus

[bx reveals elongated rete ridges and hyperkeratosis of keratin layer; tx with moderate strength steroid ointments with antipruritic agents]

Question 12

Potentially precancerous lesion of the vulva characterized by intense pruritis, dyspareunia, burning pain, and exam reveals thin white inelastic skin with crinkled tissue paper appearance

Answer 12

Lichen sclerosis

[tx with clobetasol]

Question 13

Dermatologic condition of the vulva characterized by purplish, polygonal papules that may appear in an erosive form; pts complain of vulvar burning and severe insertional dyspareunia

Answer 13

Lichen planus

[tx with topical and systemic steroids]

Question 14

______ of the vaginal wall consists of islands of columnar cells in normal squamous epithelium, seen in women who have been exposed to DES in utero

Answer 14

Adenosis

Question 15

Vulvar intraepithelial neoplasia (VIN) type III/squamous cell CIS is used to denote high grade squamous lesions and is subdivided into what 2 types?

Answer 15

VIN usual-type = associated with HPV, smoking, and immunocompromised state

Differentiated type VIN = NOT associated with HPV or smoking; more commonly associated with vulvar dermatologic conditions like lichen sclerosis

Question 16

Clinical features of squamous cell vulvar carcinoma

Answer 16

Typically occurs in post menopausal females between 70-80 y/o as vulvar lump

Usually pruritic, raised, ulcerated, pigmented, and/or warty in appearance — usually on labia majora

Definitive dx requires bx

Question 17

Microecology of the vagina including normal vaginal pH

Answer 17

Lactic acid and hydrogen peroxide producing lactobacilli predominate — resulting in vaginal pH of 3.8-4.2

[altered by things like abx, douching, intercourse, and foreign bodies]