Diseases of the Vulva Azza Alyamani Department of Obstet.

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Diseases of the VulvaAzza AlyamaniDepartment
ofObstet. Gynecol.
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• 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

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• Benign Conditions
• of the Vulva

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• (1) Epithelial Conditions
• 1) Lichen simplex .
• 2) Lichen sclerosis.
• 3) Lichen planus,
• erosive lichen planus.

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• 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.

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• lichen simplex

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• 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.

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• 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.

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• 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.

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• lichen sclerosis
  advanced

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• 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 .

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• erosive lichen planus lichen
  planus
• of vulva vagina

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• (2) Benign Neoplastic condions
• 1) epidermal inclusion and sebaceous cysts.
• 2) vulvar varicosities.
• 3) fibromas and lipomas.
• 4) clitoromegaly.

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• 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.

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• 2) Vulval Varicosities
• Can enlarge especially during pregnancy
• to cause discomfort and carry a possible
• risks for rupture or thrombosis.

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• 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 110 cm in
  diameter,
• but may become huge .
• Lipomas
• slow growing tumors composed of
  adipose
• cells.

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• Vulval Fibroma

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• 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.

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• clitoromegaly

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• ( 3) Dermatologic Disorders
• 1) Psoriasis.
• 2) Behcet 's syndrome.
• 3) Crohn ?s disease .
• 4) Acanthosis nigricans .

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• 1) Psoriasis
• appears velvety but lack the
  characteristic
• scaly patches found on the knees
  elbows.

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• 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 Crohns disease.
• treatment no effective ttt.

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• oral ulcer
  vulvar ulcer
• Behcet' s
  disease

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• 3) Crohns 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.

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• 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.

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• 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.

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• 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 .

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• 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.

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• Vulval Intraepithelial Neoplasia (VIN)
• 2 types of VIN
• 1. squamous cell carcinoma in situ
• VIN III or Bowens disease.
• 2. Adenocarcinoma in situ
• VIN III or Pagets disease.

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• 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.

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• 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.

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• 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.

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• 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.

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• 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.

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• Paget' s disease

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• 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.

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squamous cell carcinoma of vulva
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• 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.

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• 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.

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• FIGO Staging of Cancer Vulva

Tumor limited to the vulva or perineum or both ,and 2 cm or lt in diameter ,and no nodal metastases. as above stromal invasion lt 1mm. as above stromal invasion gt 1 mm. Tumor limited to the vulva or perineum or both ,and gt 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 I Ia Ib Stage II Stage III
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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. Stage IV IVa IVb

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• Management
• A) Early vulval cancer
• Stage I a
• ( penetration depth lt 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 gt 1mm )
• radical local excision
  ipsilateral inguinal
• femoral lymphadenectomy if the
  lesion is
• unilateral and bilateral
  groin dissection in
• the
  midline lesions .

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• 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.

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• C) Positive lymph nodes
• Radiation
• used with gt one nodal mico metastasis
  (lt5mm),
• 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.

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• 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.

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• malignant melanoma of the vulva

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• 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 gt radical
  local
• excision ipsilateral inguinal femoral
• lymphadenectomy.
• adjuvant therapy
• nonspecific immuno stimulants.
• chemotherapy.
• vaccines.

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Thank You