Twenty Eight Flashcards
(18 cards)
What does the vulva consist of anatomically? What is it lined by?
I. BASICPRINCIPI.ES
A. Anatomically includes the skin and mucosa of the female genitalia external to the
hymen (labia major a, labia minora, mons pubis, and vestibule)
B. Lined by squamous epithelium
What is a bartholin cyst? What is the pathophys? Epidemiology? Presentation?
II. BARTHOLIN CYST
A. Cystic dilation of the Bartholin gland
1 . One Bartholin gland is present on each side of the vaginal canal and produces mucus-like fluid that drains via ducts into the lower vestibule.
B. Arises due to inflammation and obstruction of gland
1 , Usually occurs in women of reproductive age
C. Presents as a unilateral, painful cystic lesion at the lower vestibule adjacent to the vaginal canal
What is a condyloma? What is the etiology? What are they like histologically? Risk for CA?
A. Warty neoplasm of vulvar skin, often large B. Most commonly due to HPV types 6 or 1 1 (condyloma acuminatum. Fig. 13.1 A); secondary syphilis (condyloma latum) is a less common cause. Both are sexually transmitted. C. Histologically, HPV-associated condylomas are characterized by koilocytes (hallmarkofHPV-infected cells. Fig. 13.IB). D. Condylomas rarely progress to carcinoma (6 and 1 1 are low-risk HPV types).
What is lichen sclerosis? How is it characterized? How does it present? Epidemiology? Etiology? Risk for CA?
IV. LICHEN SCLEROSIS
A. Characterized by thinning of the epidermis and fibrosis (sclerosis) of the dermis
B. Presents as a white patch (leukoplakia) with parchment-1 ike vulvar skin
C. Most commonly seen in postmenopausal women; possible autoimmune etiology
D. Benign, but associated with a slightly increased risk for squamous cell carcinoma
How is lichen simplex chronicus characterized? Presentation? What is it associated with? Risk for CA?
V. LICHEN SIMPLEX CHRONICUS
A. Characterized by hyperplasia of the vulvar squamous epithelium
B. Presents as leukoplakia with thick, leathery vulvar skin
C. Associated with chronic irritation and scratching
D. Benign; no increased risk of squamous cell carcinoma
What does vulvar CA arise from? Epidemiology? Presentation? How is diagnosis made? What are two possible etiologies? What are some risk factors for the two etiologies?
VI. VULVAR CARCINOMA
A. Carcinoma arising from squamous epithelium lining the vulva
B. Relatively rare, accounting for only a small percentage of female genital cancers
C. Presents as leukoplakia; biopsy may be required to distinguish carcinoma from other
causes of leukoplakia.
D. Etiology may be HPV related or non-HPV related.
E. HPV-related vulvar carcinoma is due to high-risk HPV types 1 6 and 18
1 . Risk factors are related to HP V exposure and include multiple partners and early first age of intercourse; generally occurs in wome n of reproductive age
2. Arises fro m vulvar intraepithelial neoplasia (V1N), a dysplastic precurso r lesion characterized by koilocytic change, disordered cellular maturation, nuclear atypia, and increased mitotic activity
F, Non-HP V related vulvar carcinoma arises, most often, fro m long-standing lichen sclerosis,
1 . Chronic inflammatio n and irritation eventually lead to carcinoma.
2. Generally seen in elderly wome n (average age is > 70 years)
What is extramammary paget disease? How is it characterized? How does it present? What does it represent? What must it be distinguished from? How is that done?
VII. E X T R A M A M M A R Y PAGET DISEAS E
A. Characterized by malignant epithelial cells in the epidermis ot the vulva (Fig, 13.2)
B. Presents as erythematous , pruritic, ulcerated vulvar ski n
C. Represents carcinoma in situ, usually with no underlying carcinoma
1 . Pager disease of the nipple is also characterized by malignant epithelial cells in the epidermis of the nipple, hut it is almost always associated with an underlying
carcinoma.
D. Must be distinguished fro m melanoma, which rarely can occur on the vulva
L, Paget cells are PAS+. keratin+, and S100-.
2. Melanoma is PAS-, keratin-, and S100+
What is the vagina? What is it lined with?
I. BASIC PRINCIPLE S
A. Canal leading to the cervix
B. Mucosa is lined by non-keratinizing squamous epithelium
What is adenosis? Explain the pathophys. Who is at increased risk?
II. ADENOSI S
A. Focal persistence of columnar epithelium in the upper 1/3 of the vagina
I. During development, squamous epithelium fro m the lower 2/3 of the vagina (derived fro m the urogenital sinus) grows upward to replace the columnar
epithelium lining of the upper 1/3 of the vagina (derived from the Miillerian ducts).
B. Increased incidence in females who were exposed to diethylstilbestrol (DES) in utero
What is clear cell adenocarcinoma? Etiology? Epidemiology?
III CLEA R CELL A D E N O C A R C I N O M A
A. Malignant proliferation of glands with clear cytoplasm
B. Rare, but feared, complication of DES-associated vaginal adenosis
C. Discovery of this complication (along with other t)ES-induced abnormalities of the gynecologic tract such as abnormal shape of the uterus} led to cessation of DES
usage.
What is embryonal rhabdomyosarcoma? HOw does it present? What is another name for it? What is the characteristic cell and how it is characterized?
IV EMBRYONAL RHABDOMYOSARCOMA
A. Malignant mesenchymal proliferation of immature skeletal muscle; rare
B. Presents as bleeding and a grape-1 ike mass protruding fro m the vagina or penis of a
child (usually < 5 yrs of age); also known as sarcoma botryoides (Fig. 13.3)
C. Rhabdomyoblast , the characteristic cell, exhibits cytoplasmic cross-striations and positive imniunohistochemical staining for desmin and myogenin.
What is vaginal carcinoma? What is its precursor? What is it related to? Describe its spread to lymph nodes.
V. VAGINAL C A R C I N O M A
A. Carcinoma arising from squamous epithelium lining the vaginal mucosa
B- Usually related to high-risk HPV
C. Precursor lesion is vaginal intraepithelial neoplasia (VAIN).
D. Whe n spread to regional lymph nodes occurs, cancer from the lower 2/3 of vagina goes to inguinal nodes, and cancer fro m the upper 1/3 goes to regional iliac nodes.
What is the cervix? What are the two parts? How are they lined? What is the transformation zone?
A. Anatomically, comprises the “neck” of the uterus
B. Divided into the exocervix (visible on vaginal exam) and endocervix). Exocervix is lined by nonkeratinizing squamous epithelium,
2. Endocervix is lined by a single layer of columnar cells.
3 . function between the exocervix and endocervix is called the transformatio n zone (Fig. 13.’1A).
What is HPV? Where does it infect generally? Specifically? What are possible results of it? What are some high risk types? Low risk? Describe the pathophys of high risk HPV.
II. HP V
A. Sexually transmitted DNA virus that infects the lower genital tract, especially the
cervix in the transformation zone
B. Infection is usually eradicated by acute inflammation; persistent infection leads to an increased risk for cervical dysplasia (cervical intraepithelial neoplasia, CIN),
C. Risk of CIN depends on HPV type, which is determined by DNA sequencing.
1 . High-risk—HPV types 16, 18, 31, and 33
2. Low-risk—HPV types 6 and 1 1
D. High-risk HPV produce E6 and E7 proteins which result in increased destruction of
p5 3 and Rb, respectively. Loss of these tumo r suppressor proteins increases the risk
for CIN.
What is CIN? How is it characterized? Describe the different grades? Describe the progression and what determines progression and regression.
III. C E R V IC A L I N T R A E P I T H E L I A L NEOPLASI A
A. Characterized by koiloeytic change, disordered cellular maturation, nuclear atypia, and increased mitotic activity within the cervical epithelium.
B. Divided into grades based on the extent of epithelial involvement by immature
dysplastic cells
1 . CIN I involves < 1/3 of the thickness of the epithelium.
2. CIN II involves < 2/3 of the thickness of the epithelium,
3 . CIN III involves slightly less than the entire thicknes s of the epithelium (Fig.
13.4B).
4. Carcinoma in situ (CIS) involves the entire thickness of the epithelium.
C. CIN classically progresses in a stepwise fashion through CIN I, CIN II, CIN 111, and CIS to become invasive squamous cell carcinoma.
1 . Progression is not inevitable (e.g., CIN 3 often regresses).
2, The higher the grade of dysplasia, the more likely it is to progress to carcinoma
and the less likely it is to regress to normal.
What is cervical CA? Epidemiology? How does it present? What is the key risk factor? What are two secondary risk factors? What are the most common subtypes? What are they associated with? Describe what happens in advanced tumors.
IV. CERVICA L C A R C I N O M A
A. Invasive carcinoma that arises fro m the cervical epithelium
B. Most commonl y seen in middle-aged women (average age is 40-5 0 years)
C. Presents as vaginal bleeding, especially postcoital bleeding, or cervical discharge
D. Key risk factor is high-risk HP V infection; secondary risk factors include smoking and immunodeficienc y (e.g., cervical carcinoma is an AlDS-definlng illness).
E. Most commo n subtypes of cervical carcinoma are squamous cell carcinoma (80% of
cases) and adenocarcinoma (15% of cases). Both types are related to HP V infection.
P. Advanced tumors often invade through the anterior uterine wall into the bladder, blocking the ureters. Hydronephrosis with postrenal failure is a commo n cause of death in advanced cervical carcinoma.
Describe the goals and guidelines of screening of cervical CA. What is the gold standard? How is it performed? How are the findings then classified? What is the confirmatory test? What are some limitations of the gold standard? How successful is it?
V. S C R E E N I N G A N D P R E V E N T I O N O F C E R V I C A L C A R C I N O M A
A. The goal of screening is to catch dysplasia (CIN) before it develops into carcinoma.
1 . Progression fro m CIN to carcinoma, on average, takes 10-20 years.
2. Screening begins at age 21 and is initially performe d yearly.
B. Pap smear is the gold standard for screening.
1 . Cells are scraped fro m the transformatio n zone using a brus h and analyzed u n d e r a microscope.
2. Dysplastic cells are classified as low grade (CIN I) or high grade (CIN II and 111).
3 . High-grade dysplasia is characterized by cells with hyperchroniatic (dark) nuclei and high nuclear to cytoplasmic ratios (Fig. 13.4C).
C. Pap smear is the most successful screening test developed to date.
. It is responsible for a significant reduction in tbe morbidity and mortality of
cervical carcinoma (cervical carcinoma went from being the mos t commo n to
one of the least commo n types of gynecologic carcinoma in the US).
2. Women who develop invasive cervical carcinoma usually have not undergone screening
D. An abnormal Pap smear is followed by confirmatory colposcopy (visualization of cervix with a magnifying glass) and biopsy.
P. Limitations of the Pap smear include inadequate sampling of the transformatio n zone (false negative screening) and limited efficacy in screening for adenocarcinoma,% Despite Pap smear screening, the incidence of adenocarcinoma has not
decreased significantly
What does the HPV vaccine cover? How long does it last? What else shold be done to prevent these diseases? Why?
F. Immunizatio n is effective in preventing 11PV infections.
1 . The quadrivalent vaccine covers HP V types 6,11,16 , and 18,
2. Antibodies generated against types 6 and 1 1 protect against condylomas,
3 . Antibodies generated against types 16 and I8 protect against CIN and carcinoma.
4. Protection lasts for 5 years.
5 . Pap smears are still necessary due to the limited numbe r of HP V types covered by the vaccine
