Diseases of the Vulva Azza Alyamani Department of

Diseases of the Vulva Azza Alyamani Department of 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 of vulva & vagina lichen planus

(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 Tumor limited to the vulva or perineum or both , and 2 cm or < in diameter , and no nodal metastases. as above + stromal invasion < 1 mm. as above + stromal invasion > 1 mm. Stage II Tumor limited to the vulva or perineum or both , and > 2 cm in diameter , and no nodal metastases. Stage III Tumor of any size with : • adjacent spread to the urethra &/or vagina &/or anus • unilateral regional LN. metastasis or combination.

Stage IV IVa 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 < 1 mm below the basement membrane & no nodal metastases ) radical local excision é surgical margins 1 cm, patient do not need groin dissection. * Stage I b & Stage II ( penetration > 1 mm ) 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 1 ry tumor , followed by more conservative surgical excision.

C) Positive lymph nodes Radiation used with > one nodal mico metastasis (<5 mm), 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 2 nd 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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