Vulvar Lesion with Pruritus/Neoplasia



 

A 70-year-old woman complains of vulvar itching for a year. She has been treated with multiple steroid medications with no relief. On pelvic examination there is a well-defined, 1-cm white lesion of the left labia minora. There are no other lesions in the vulva noted; however, there is a clinical enlargement of a left inguinal node.

 

Clinical Presentation. The most common symptom of both benign as well as malignant lesionsis vulvar itching resulting in scratching.

 

Differential Diagnosis. This includes sexually transmitted diseases, benign vulvar dermatosis,or cancers.

 

 

Premalignant vulvar dermatosis

 

These are benign lesions with malignant predisposition. The most common symptom is vulvar itching, but most lesions are asymptomatic.

 

Squamous hyperplasia. These lesions appear as whitish focal or diffuse areas that arefirm and cartilaginous on palpation. Histologically, they show thickened keratin and epithelial proliferation. Management is fluorinated corticosteroid cream.

 

Lichen sclerosus. This appears as bluish-white papula that can coalesce into whiteplaques. On palpation they feel thin and parchment-like. Histologically, they show epi-thelial thinning. Management is Clobetasol cream.

 

Squamous dysplasia. These lesions appear as white, red, or pigmented, often multifocalin location. Histologically, they show cellular atypia restricted to the epithelium without breaking through the basement membrane. The appearance is almost identical to cervical dysplasia. Management is surgical excision.

 

CIS. The appearance is indistinguishable from vulvar dysplasia. Histologically, thecellular atypia is full thickness but does not penetrate the basement membrane. Management is laser vaporization and vulvar wide local excision.

 

Malignant vulvar lesions

 

Epidemiology. Vulvar carcinoma is an uncommon gynecologic malignancy, with a mean ageat diagnosis of 65 years. It is the fourth most common gynecologic malignancy. Risk factors include older age, cigarette smoking, HIV, premalignant vulvar dermatosis.

 

Squamous cell (90%). The most common type of invasive vulvar cancer is squamouscell carcinoma, which has been associated with HPV. Pathogenesis is chronic inflam-mation (for older women) and HPV infection (for younger women). The most com-mon stage at diagnosis is Stage 1.


 

 

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Chapter 3 l Disorders of the Vagina and Vulva

 

 

Melanoma (5%). The second most common histologic type of vulvar cancer is mela-noma of the vulva, and the most important prognostic factor for this type of tumor is the depth of invasion. Any dark or black lesion in the vulva should be biopsied and considered for melanoma.

 

Paget disease. An uncommon histologic lesion is Paget disease of the vulva. Paget dis-ease is characteristically a red lesion, which is most common in postmenopausal white women. Any patient with a red vulvar lesion must be considered for the possibility of Paget disease. Most of the time Paget disease is an intraepithelial process; however, in approximately 18–20% of cases invasion of the basement membrane has been identi-fied. Patients with Paget disease of the vulva have a higher association of other cancers mainly from the GI tract, the genitourinary system, and breast.

Diagnosis. Biopsy. All vulvar lesions of uncertain etiology should be biopsied. Patients withvulvar pruritus should be considered for the possibility of preinvasive or invasive vulvar carci-nomas if there is a vulvar lesion. A biopsy of this patient’s lesion reveals invasive squamous cell carcinoma of the vulva.

 

Pattern of spread. It starts with local growth and extension that embolizes to inguinal lymphnodes and finally, hematogenous spread to distant sites.

 

Staging. Staging is surgical.

 

Stage 0: CIS (basement membrane is intact)

 

Stage I: Tumor confined to the vulva with size ≤2 cm; nodes not palpable

 

IA. Invasion ≤1 mm deep

IB. Invasion >1 mm deep

 

Stage II: Tumor confined to the vulva with size >2 cm; nodes not palpable

 

Stage III: Tumor any size with spread to lower urethra, vagina, or anus; unilateral nodes

 

Stage IV: Widespread metastases

 

IVA. Involves upper urethra, bladder or rectum, pelvic bone, bilateral nodes

 

IVB. Distant metastasis

 

Management

 

Wide local excision only: used only for stage IA; risk of metastasis is negligible so nolymphadenectomy is needed

 

Modified radical vulvectomy: involves radical local excision

 

– Ipsilateral inguinal dissection is used only if stage is IB & unifocal lesion >1 cm from midline AND no palpable nodes

 

– Bilateral inguinal dissection is used if at least stage IB or a centrally located lesion OR palpable inguinal nodes or positive ipsilateral nodes

 

Radical vulvectomy: involves removal of labia minora & majora, clitoris, perineum,perineal body, mons pubis; seldom performed due to high morbidity

 

Pelvic exenteration. In addition to radical vulvectomy, it involves removal of cervix,vagina and ovaries in addition to lower colon, rectum and bladder (with creation of appropriate stomas); seldom indicated or performed due to high morbidity.

 

Radiation therapy: used for patients who cannot undergo surgery

 

 

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USMLE Step 2 l Gynecology

 

 


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