diseases of the vulva azza alyamani department of obstet. & gynecol
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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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