Diseases of Vulva Flashcards
1
Q
Inflammatory Diseases of the Vulva
A
- Condyloma Acuminatum
- Molluscum Contagiosum
- Herpesvirus Infection
- Most common type of vulvar disease
- Include sexually and non-sexually transmitted infections
- May present as
– Papillary growths
– Ulcers
– Redness, discoloration
- May be asymptomatic
- Symptoms, if present, include
– Itching
– Burning
– Pain
2
Q
Inflammatory Diseases of the Vulva: Condyloma Acuminatum
A
- Condylomata acuminata develop as a result of infection with low risk subtypes of human papillomavirus, primarily types 6 and 11.
- They may involve the perianal and perineal regions, and occasionally the vagina and cervix, as well as the vulva, and are usually multiple.
- Grossly, they are exophytic warty growths that may vary in size from millimeters to over 10 centimeters.
- Histologically, they consist of a branching, tree-like proliferation of stratified squamous epithelium supported by a fibrous stroma.
- The squamous epithelium shows acanthosis, parakeratosis, hyperkeratosis, and, most significantly, the signature viral cytopathic effect (koilocytotic atypia), characterized by hyperchromatic nuclei, with irregular nuclear membranes and prominent perinuclear clearing of the cytoplasm.
3
Q
Inflammatory Diseases of the Vulva: Molluscum Contagiosum
A
- Molluscum contagiosum is another common viral infection of the vulva.
- Grossly, the lesions are small papules with a central punctuation.
- Microscopically, the lesions are very distinctive, with the center of the lesion beneath the punctuation showing prominent large, pink to purple viral inclusions filling the nuclei of the squamous cells.
- May be sexually or non-sexually transmitted
- Regresses spontaneously over many months
4
Q
Inflammatory Diseases of the Vulva: Vulvar Candidiasis
A
- Candida is a fungus that likes to grow in dark, damp places
- Very common
– On vulvar skin, causes itchy/burning red rash
- Affects children and adults
- NOT sexually transmitted
5
Q
Inflammatory Diseases of the Vulva: Herpesvirus Infection
A
•The lesions of genital herpesvirus infection may involve the vagina and cervix as well, but the disease is usually diagnosed by the presence of ulcerated vulvar lesions in which the characteristic multinucleated cells containing viral inclusions are identified.
6
Q
Non-Neoplastic Diseases of the Vulvar Squamous Epithelium - Vulvar Dermatoses
A
- Lichen Sclerosus
- Lichen Simplex Chronicus
- Not specific to vulva
- Frequently seen in association with intraepithelial neoplasia
- Some histologic features overlap with other benign conditions or with those of intraepithelial neoplasia
– Important consideration in differential diagnosis of vulvar lesions
7
Q
Non-Neoplastic Diseases of the Vulvar Squamous Epithelium - Vulvar Dermatoses: Lichen Sclerosus
A
- Lichen sclerosus of the vulva leads to atrophy, fibrosis, and scarring of the vulva.
- On physical examination, the vulvar skin is pale and parchment-like.
- Microscopically, there is atrophy of the epidermis, hydropic degeneration of the basal cell layer, severe fibrosis of the dermis, and band-like monoclonal lymphocytic infiltration of the lower dermis.
- Although the pathogenesis is not completely understood, many of its features suggest an autoimmune origin.
- This disorder can occur at any age, but has a predilection for postmenopausal women.
- It tends to be insidious and progressive.
- In long-standing cases, the labia become atrophied, and the introitus narrowed.
- Small risk of developing squamous carcinoma with long-standing disease
8
Q
Non-Neoplastic Diseases of the Vulvar Squamous Epithelium - Vulvar Dermatoses: Lichen Simplex Chronicus
A
- Lichen simplex chronicus is a nonspecific condition that manifests clinically as thickened, roughened gray or white patches which develop as the result of rubbing or scratching the vulvar skin.
- It can be a response to infections (tinea, candida, etc.), mucosal irritation due to chemical exposure, allergic responses and any other cause of pruritus.
- Histologically, this disorder is characterized by acanthosis (thickening of the squamous epithelium), hyperkeratosis, and variable leukocytic infiltration of the dermis.
9
Q
Neoplastic Diseases of the Vulvar Squamous Epithelium
A
- Intraepithelial Lesions
- High Grade Squamous Intraepithelial Lesion (HSIL)
- Differential VIN (dVIN)
- Paget Disease
- Squamous Carcinoma of the Vulva
- Malignant Melanoma
- Squamous
– HPV associated
- Morphology identical to that seen in HPV-associated lesions in other sites of the lower anogenital tract
- Low grade lesions are controversial on the vulva
– Rare vs. nonexistent
• Almost all are high grade
– HPV independent (differentiated type)
• Non-squamous
– Paget disease
– Others
10
Q
Neoplastic Diseases of the Vulvar Squamous Epithelium: Intraepithelial Lesions
A
- Intraepithelial lesions of the vulvar squamous epithelium may present with a wide variety of appearances which can mimic any number of other benign conditions.
- Lesions may appear red, white, or pigmented, and may be elevated or eroded/ulcerated.
- It is not possible to tell from the gross appearance whether a lesion is neoplastic or reactive, which makes biopsy for pathologic evaluation imperative.
11
Q
Neoplastic Diseases of the Vulvar Squamous Epithelium: High Grade Squamous Intraepithelial Lesion (HSIL)
A
- Most intraepithelial neoplasia of the vulva is caused by high risk subtypes of HPV (16, 18 and others).
- Formerly known as “vulvar intraepithelial neoplasia (VIN)”, these HPV-related lesions are now termed “high grade squamous intraepithelial lesions (HSILs)” and are morphologically similar to the HPV-related lesions occurring throughout the lower anogenital tract.
- HSIL is frequently multifocal (presenting with multiple lesions of the vulva) and multicentric (presenting with lesions in multiple anatomic sites, which may include the vulva, vagina, cervix and anus/perianal region).
- Microscopically, these lesions are characterized by nuclear atypia, increased mitotic activity, and lack of epithelial differentiation.
- Lesions may have a warty morphology, characterized by an exophytic papillary growth pattern with prominent hyper- and parakeratosis, and cells with the typical pink cytoplasm of squamous cells, or, when there is complete loss of maturation, a basaloid morphology in which the cells show only scant cytoplasm.
- Unlike condyloma acuminatum, which is caused by low risk subtypes of HPV, HSIL of the vulva is considered a precursor lesion to carcinoma, and may progress to squamous carcinoma if left untreated. The risk of transformation to invasive carcinoma is increased in women older than 45 years, and in immunosuppressed patients.
- The treatment consists of surgical removal of these lesions.
12
Q
Neoplastic Diseases of the Vulvar Squamous Epithelium: Differentiated VIN (dVIN)
A
- A minority of intraepithelial neoplasia of the vulva is not HPV-related and does not have a morphologic counterpart in any other site in the lower anogenital tract.
- This type of lesion is known as “VIN, differentiated type (dVIN)” and appears to progress more quickly to invasive disease.
- Patients with dVIN tend to be older, as a group, then patients with HSIL, tend to have only a single lesion, and do not have involvement of other anatomic sites.
- The gross appearance is similar to HSIL, but the microscopic appearance is very different.
- In dVIN, the surface layers of the epithelium appear normal, and rather than a lack of maturation, as is seen in HSIL, there is an excess of maturation in dVIN, with the maturation extending downward towards the basal layers of epithelium.
- dVIN is often seen in association with associated lichen sclerosus or lichen simplex chronicus, and is rarely seen without an adjacent invasive squamous carcinoma, which is why it is believed to progress quickly to the invasive stage.
13
Q
Neoplastic Diseases of the Vulvar Squamous Epithelium: Paget Disease
A
- Paget disease of the vulva is an unusual type of intraepithelial neoplasia in which neoplastic cells with glandular differentiation percolate through the otherwise normal squamous epithelium.
- It typically presents as a pruritic, red, sharply demarcated, geographic lesion usually located on the labia majora.
- Microscopic examination shows the tumor cells, which have relatively abundant, finely granular, pale cytoplasm that stains with mucin stains, intermixed with the squamous cells in the epithelium.
- Although similar, grossly and microscopically, to Paget disease of the nipple, Paget disease of the vulva (primary cutaneous Paget disease) is almost always confined to the epidermis and its appendages, and is only rarely associated with an underlying tumor.
- When it does occur as the result of spread from an adjacent adenocarcinoma (anorectal or urothelial) it is called secondary Paget disease.
14
Q
Neoplastic Diseases of the Vulvar Squamous Epithelium: Paget Disease Classification
A
• Disease of vulvar origin (Primary cutaneous Paget disease)
- Intraepithelial
- Invasive (rare)
- Spread from an underlying or adjacent cutaneous or glandular vulvar neoplasm
• Disease of non-vulvar origin (Secondary Paget disease)
– Spread of adjacent adenocarcinoma (i.e. anal or rectal carcinoma)
– Spread of urothelial intraepithelial or invasive carcinoma
15
Q
Neoplastic Diseases of the Vulvar Squamous Epithelium: Squamous Carcinoma of the Vulva
A
- The most common malignant disease of the vulva is squamous carcinoma.
- It is derived from intraepithelial neoplasia, either HSIL or dVIN, which is why it is so important to identify and treat such lesions before they become invasive.
- This is an uncommon malignant neoplasm that accounts for only approximately 3% of all genital cancers in women. Approximately 85% of vulvar carcinomas are squamous cell carcinomas and most cases occur in postmenopausal patients. Based on etiologic and clinical features, these carcinomas can be divided into two groups:
- Carcinomas associated with high-risk HPV
- HPV-independent carcinomas
•The prognosis of vulvar carcinoma is dependent on the age and immune status of the patient, presence of lymph node metastasis, and size of the tumor. The treatment involves vulvectomy and lymphadenectomy.
18
Q
Neoplastic Diseases of the Vulvar Squamous Epithelium: Malignant Melanoma
A
- Malignant melanoma of the vulva is a rare lesion that accounts for less than 5% of all malignant vulvar cancers and 2% of all melanomas in women.
- Most cases occur in the sixth of seventh decades of life.
- The microscopic features and clinical behavior are similar to those of melanomas located elsewhere.
- Grossly and microscopically, vulvar melanoma may resemble Paget disease.
- The 5-year survival rate is less than 32% and the prognosis is closely linked to the depth of invasion of the tumor.
20
Q
Glandular Diseases of the Vulva: Bartholin Cyst and Abscess
A
- Although the majority of the vulva is composed of squamous epithelium, there are a few specialized glands there as well, the largest of which, and the most commonly encountered in pathology practice, is the Bartholin gland.
- These glands are very inconspicuous, and generally only come to clinical attention when they are enlarged by disease.
- Acute infection of the Bartholin gland can lead to adenitis (acute inflammation of the gland) and abscess formation.
- Bartholin cysts are relatively common, can occur at any age, and result from obstruction of the gland duct, which is usually secondary to infection.
- These cysts can become quite large (up to 5 cm) resulting in significant local discomfort and pain.
- The treatment is surgical removal or marsupialization of the cyst.
21
Q
Glandular Diseases of the Vulva: Papillary Hidradenoma
A
- This is the most common glandular tumor of the vulva.
- It is histologically identical to the intraductal papilloma of the breast, and was once thought to be derived from ectopic mammary tissue, though it is now understood to arise from specialized anogenital sweat glands.
- It presents as a well-circumscribed subcutaneous nodule in the labia majora or interlabial folds, sometimes with ulceration of the overlying epithelium.
- Microscopically, it is characterized by tubular glands and papillary formations lined by nonciliated columnar cells and surrounded by a layer of myoepithelial cells.
24
Q
Diseases of the Vagina: Malignant Neoplasms of the Vagina - Adenocarcinoma
A
- Adenocarcinoma of the vagina is a rare neoplasm.
- Patients whose mothers received diethylstilbestrol (DES) during pregnancy are at an increased risk of developing clear cell adenocarcinoma.
- Most of these cases occur during the second decade of life.
- The tumors are usually located on the anterior wall of upper third of the vagina, and range in size from a 0.2 to 10 cm.
- Microscopically, they are characterized by vacuolated, glycogen-containing glandular cells.
- Vaginal adenosis appears to be the precursor lesion and occurs in 35% to 90% of patients with history of antenatal exposure to DES.
– Vaginal adenosis is a congenital abnormality where glands are present
25
Q
Diseases of the Vagina: Malignant Neoplasms of the Vagina - Embryonal Rhabdomyosarcoma
A
- Embryonal rhabdomyosarcoma, also known as sarcoma botryoides, is an uncommon vaginal tumor that presents during infancy and early childhood.
- On gross examination, the lesion has a characteristic appearance: semi translucent polypoid masses that resemble clusters of grapes (hence the term botryoides that means grapelike).
- The tumor is composed of malignant rhabdomyoblasts lying within a myxomatous stroma. There is a characteristic crowding of the neoplastic cells immediately beneath the vaginal epithelium, forming the so-called cambium layer.
- Because of the presence of inflammatory cells, these lesions are sometimes mistaken for inflammatory pseudopolyps.
- These lesions invade locally and cause death by invasion into the adjacent organs and peritoneal cavity.
- Conservative resection and chemotherapy are the treatment of choice in patients diagnosed early
26
Q
Diseases of the Vagina: Bacterial Vaginosis
A
- Overgrowth of bacteria, probably multiple species, including Gardnerella vaginalis
- Diagnosed by detection of “clue cells” (squamous cells covered in coccobacilli) on cytologic preparations in the appropriate clinical setting
- In pregnant women, increases the chances of chorioamnionitis and premature delivery
27
Q
Diseases of the Vagina: Candida Vaginitis
A
- Very common
- Symptoms:
– Itching/burning
– Thick white discharge
- Diagnosis is usually made clinically (pathologist involvement is unnecessary)
- Common incidental finding on pap tests
28
Q
Diseases of the Vagina: Trichomonas
A
- Amoebic infection
- Very common sexually transmitted disease
- Symptoms include itching, pain, copious yellow green discharge, but often patients are asymptomatic
- May be seen on pap test (dead), or on wet-preps (alive and swimming!)
