Diseases of the VulvaDiseases of the Vulva

Azza AlyamaniAzza Alyamani

DepartmentDepartmentofof

Obstet. & Gynecol.Obstet. & Gynecol.

Vulvo-vaginal problems are among 10 leading

disorders encountered by primary care clinicians.

* Benign lesions of the vulva are mentioned in three

categories :

1. Epithelial conditions.

2. Benign neoplastic disorders.

3. Dermatologic disorders.

* VIN

* Cancer vulva

Benign Conditions

of the Vulva

(1) Epithelial Conditions

1) Lichen simplex .

2) Lichen sclerosis.

3) Lichen planus,

erosive lichen planus.

1) Lichen Simplex “ squamous cell hyperplasia “

* it is a local thickening of the epithelium resulting from a prolonged itching . * symptoms : pruritus and pain. * signs : white or reddish thickened ,leathery ,raised surface.

usually discrete lesion but may be multiple.

* treatment : • moderate-strength steroid ointment. • antipruritic agent.

lichen simplex

2) Lichen Sclerosis

* it is a chronic progressive disease which constrict and destroy the normal genital anatomy . In the long term ,labia minora are lost ,labia majora flatten ,clitoris becomes inverted .

* frequently found on the vulva of postmenopausal women & can involve all the genital area from mons to the anal area.

* combinations of lichen sclerosis & epithleal hyperplasia or carcinoma are possible.

* symptoms: intense pruritus , dyspareunia and burning pain. * signs: thin inelastic atrophic skin ,white with a crinkled , tissue paper appearance.

* diagnosis: multiple biopsies is necessary. it reveals a thin atrophic epithelium with inflammatory cells lining the basement membrane.* treatment: ● potent topical steroids. 80% of lesions respond. long term therapy with low potent steroids may be necessary. ● other local treatments are: esrtogen cream and anaesthetics.

lichen sclerosis advanced

3) Lichen planus

* it is a purplish ,polygonal papules that may appear in their erosive form. * it involve the vulva ,the vagina and the mouth ( vulval – vaginal –gingival syndrome ). * symptoms: vulval burning , severe dyspareunia when vaginal stenosis develop in advanced stages. * treatment: topical and systemic steroids .

erosive lichen planus lichen planus

of vulva & vagina

(2) Benign Neoplastic condions

1) epidermal inclusion and sebaceous cysts.

2) vulvar varicosities.

3) fibromas and lipomas.

4) clitoromegaly.

1) epidermal inclusion & sebaceous cysts

* they are nontender , mobile , spherical ,slow

growing cysts located below the epidermis. * sebaceous cysts are firmer bec. they are filled with dry caseous material.

* treatment : most of inclusion cysts require no ttt. if they are asymptomatic, or surgical excision.

2) Vulval Varicosities

Can enlarge especially during pregnancy to cause discomfort and carry a possible risks for rupture or thrombosis.

3) Fibromas and Lipomas Fibromas: * are the most common benign solid tumors that arise in the deeper connective tissue of the vulva. * they are slow growing 1–10 cm in diameter, but may become huge .

Lipomas: * slow growing tumors composed of adipose cells.

Vulval Fibroma

4) Clitoromegaly

* may develop after birth in response to excessive androgen exposure . It is a sign virillization.

* diagnosed when the clitorial length exceeds 30 mm or the width at the base exceeds 10 mm.

clitoromegaly

( 3) Dermatologic Disorders

1) Psoriasis.

2) Behcet ′s syndrome.

3) Crohn ΄s disease .

4) Acanthosis nigricans .

1) Psoriasis

appears velvety but lack the characteristic

scaly patches found on the knees & elbows.

2) Behcet ′s syndrome

* ulcers in the vulval , oral and ocular areas.

* genital lesions can result over time in a scarred vulva. * etiology : is unknown. * diagnosis : based on the concurrence ulcers in vulva ,mouth & ocular involvement ,the recurrent nature of the disease and exclusion of syphilis and Crohn’s disease. * treatment : no effective ttt.

oral ulcer vulvar ulcer Behcet′ s disease

3) Crohn’s disease

* vulval ulcers can precede the development

of GIT ulcerations .

* vulval ulcers are slit-like or knife – cut ulcers with prominent edema. Draining sinuses and fistulas to the rectum may occur.

4) Acanthosis nigricans

* most commonly found in the axilla or the

nape of the neck then vulva. * characterized by its darky pigmented velvety or warty surface . * etiology : related to insulin resistance.

Vulval Neoplasms

Introduction * uncommon 5 % of female genital tract malign.

most tumors are squamous cell carcinomas ,may be melanomas , adenocarcinomas and sarcomas.

* postmenopausal women ,mean age 65 years.

* a history of chronic vulval itching is common.

Epidemiology

Two different etiologic types of vulval cancers :

1. A less common type: * in younger women .

* related to HPV infection and smoking. * commonly associated with VIN .

2. The more common type: * in old women .

* unrelated to HPV infection or smoking. * concurrent VIN is uncommon. * long standing lichen sclerosis is common.

5% of patients have +ve serologic tests for syphilis , lymphogranuloma venereum and granuloma inguinale.

Vulval Intraepithelial Neoplasia (VIN)

2 types of VIN :

1. squamous cell carcinoma in situ

VIN III or Bowen’s disease.

2. Adenocarcinoma in situ

VIN III or Paget’s disease.

Squamous cell carcinoma in situ: VIN III ( Bowen′s disease )

* mean age 45 years. * symptoms: 50% asymptomatic. itching is the most common symptom.

* signs: most lesions are elevated ,white ,red ,pink , brown or grey in color. 20% of lesions are warty in appearance.

* diagnosis: 1.careful inspection of the vulva in bright light and with the aid of a magnifying glass. 2. 5% acetic acid aceto white areas.

* treatment :

1. local superficial excision. with margins of 5 mm are adequate. 2. skinning vulvectomy in extensive lesions. 3. laser therapy if lesions involves the clitoris , labia minora or perineal area.

Adenocarcinoma in situ VIN III ( Paget′ s disease )

* occurs in white postmenopausal elderly women. also occurs in the nipple area of the breast. * 20% is associated with adenocarcinoma. * symptoms: itching and tenderness are common. * signs: well demarcated and eczematus with white plaque like lesions. * growth may progresses beyond the vulva to the mons pubis ,buttocks & thighs.

* diagnosis histologically: adenocarcinoma in situ characterized by large ,pale , pathognomonic Paget’ s cells, typically located both in the epidermic and in the adnexal structures.

* treatment: 1. local superficial excision. with margins 5-10 mm. 2. laser therapy in recurrences which are common.

Paget′ s disease

Invasive Cancer Vulva A. Squamous cell carcinoma

* 90% of vulval cancers. * symptoms: • vulval lump or ulcer. • long standing pruritus.

* signs: • raised ,ulcerated ,pigmented or warty lesion. however , ulceration is usually an early sign. • most lesions occur on labia majora and labia

minora. Less common sites , the clitoris or the perineum. • 5% of lesions are multifocal.

squamous cell carcinoma of vulva

* spread : • direct extension to adjacent structures as the vagina , urethra and anus.

• lymphatic embolisation

inguino femoral nodes. = initially to the superficial inguinal LN. = then to deep femoral LN. located medial to the femoral vein, LN of Cloquet′s is the most common of this group.

=then spread occurs to pelvic nodes especially the external iliac LN.

= LN metastases occurs 50% in cancer vulva.

5% of patients have metastases to pelvic LN , usually 3 or more +ve unilateral inguino femoral LN.

• hematogenous occurs late to the lungs , liver and bone rarely in the absence of lymphatic metastases.

FIGO Staging of Cancer Vulva

Stage I

Ia Ib

Stage II

Stage III

Tumor limited to the vulva or perineum or both ,and

2 cm or < in diameter ,and no nodal metastases.

as above + stromal invasion < 1mm.

as above + stromal invasion > 1 mm.

Tumor limited to the vulva or perineum or both ,and

> 2 cm in diameter ,and no nodal metastases.

Tumor of any size with :

• adjacent spread to the urethra &/or vagina &/or

anus

• unilateral regional LN. metastasis or combination.

Stage IVIVa

IVb

Tumor invades any of the following pelvic : upper urethra ,bladder mucosa ,rectal mucosa ,pelvic bone or bilateral regional node metastasis ,or a combination.

Any distant metastasis including pelvic lymph nodes.

Management A) Early vulval cancer * Stage I a ( penetration depth < 1mm below the basement membrane & no nodal metastases ) radical local excision é surgical margins 1cm, patient do not need groin dissection.

* Stage I b & Stage II ( penetration > 1mm ) radical local excision +ipsilateral inguinal femoral lymphadenectomy if the lesion is unilateral and bilateral groin dissection in

the midline lesions .

B) Advanced vulval cancer * Stage III ( involves the proximal urethra ,anus or rectovaginal septum ) radical vulvectomy which includes a bowel, urinary stroma or rectovaginal septum. + bilateral groin dissection.

Preoperative radiation or chemo-radiation should be used to shrink the 1ry tumor ,followed by more conservative surgical excision.

C) Positive lymph nodes Radiation used with > one nodal mico metastasis (<5mm), or evidence of extra nodal spread . postoperative radiation to both groins and to the pelvis.

Prognosis: = it correlate significantly with LN status. with –ve nodes have a 5-ys survival rate is 90%. with +ve nodes have a 5-ys survival rate is 50%.

= patient with no involved node have a good prognosis regardless of stage.

Malignant Melanoma

* the 2nd most common vulvar cancer.

* may arise de novo or from a preexisting nevus. commonly involve labia minora or clitoris. * occurs in postmenopausal white women.

* diagnosis : any pigmented lesion of the vulva requires excisional biopsy for histopathology. * usually smaller lesions and tend to metastasized early.

malignant melanoma of the vulva

* prognosis: correlates to the depth of penetration into the dermis. The 5-ys survival rate is 30%.

* superficial lesion radical local excision alone with margins of 1 cm, is adequate. * deeper lesions 1 mm or > radical local excision + ipsilateral inguinal femoral lymphadenectomy.

* adjuvant therapy: = nonspecific immuno stimulants. = chemotherapy. = vaccines.

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