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BENIGN AND MALIGNANT LESIONS OF THE VULVA

4.05a
DR. MARIA CRISTINA ESTRELIA-SANTOS 02/22/2017

URETHRAL CARUNCLE

BARTHOLIN’S CYST
 Most common large cyst of the vulva
 Cystic dilation of an obstructed Bartholin’s gland
 Asymptomatic cysts seen in 2% of cases but becomes more
symptomatic as it enlarges
 Related to trauma and infection not with STDs
 Bartholin’s ducts are lined by transitional epithelium
 Small (1-2cm) fleshy outgrowth of the distal edge of the  These ducts are easily obstructed, usually near the distal
urethra orifice
 Most frequent in postmenopausal women  Following obstruction, there is continued secretion of
 Initially appears as an eversion of the urethra glandular fluid, which results in the cystic dilation
 Aside from ectropion of the posterior urethral wall, it is  Usually unilateral, tense and nonpainful
associated with retraction and atrophy of the  Treatment:
postmenopausal vagina. o If asymptomatic: no treatment
 Growth is secondary to chronic irritation or infection o May become symptomatic due to:
 Often secondarily infected, producing ulceration and  Size
bleeding  When it becomes infected in young
 The tissue of the outgrowth is soft, smooth, friable and women less than 40
bright red  For acute adenitis without abscess formation: broad
 The lesions may be small, single, sessile or pedunculated spectrum antibiotics, frequent hot Sitz baths
 Symptoms are variable  Treatment of Choice: for a symptomatic cyst or
o Asymptomatic abscess: development of a fistulous tract from the
o Dysuria, frequency, urgency dilated duct to the vestibule (Marsupialization) but if
 Point tenderness after contact with undergarments or 40 years old and above do excision biopsy due to
during intercourse risk of Bartholin’s gland carcinoma
 Ulcerative lesions produce spotting on contact  Simple incision and drainage: has tendency to recur
 Differential diagnosis:
o Primary carcinoma of the urethra
o Prolapse of urethral mucosa (mostly in
children)
 Diagnosis
o Biopsy under local anesthesia
o Histologically
 Transitional and stratified
squamous epithelium with loose
connective tissue
 Subdivided into:
o Papillomatous
o Granulomatous
o Angiomatous
 Therapy
o Small and asymptomatic lesion:
no treatment needed
o Oral or topical estrogen and avoidance of
irritation: if does not regress or is symptomatic
o Destroy lesion by cryosurgery, laser therapy,
fulguration or operative excision
o After operation, a Foley catheter is left in
place and follow up to prevent urethral
stenosis

URETHRAL PROLAPSE
 Seen mostly in children and pre-menarcheal female
 Majority asymptomatic but may have dysuria VULVAR CYST
 Grossly:  Vulvar cysts are either:
o The annular rosette of the friable, edematous, o Epidermal Inclusion Cyst
prolapsed mucosa o Sebaceous Cysts
o It does not have the bright-red color of a  Most common small vulvar cysts
caruncle  Cannot be differentiated from each other
 Therapy:
o Primarily Hot sitz bath and antibiotics
o Estrogen is sometimes effective
o Excision of redundant mucosa may be
needed in some

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GYNECOLOGY
BENIGN AND MALIGNANT LESION OF THE VULVA
NEVUS OR MOLE
EPIDERMAL INCLUSION CYST

 More frequent than sebaceous cysts


 Location:
o Beneath the epidermis  Vulvar area
o Commonly on the anterior half of the labia o Makes up only 1% of the skin body surface
majora area
 Grossly: o But 5% to 10% of all malignant melanomas
o They are white or yellow cysts arise from this region
o The contents are caseous, thick cheese- like o Estimated that 50% of malignant
o Usually multiple, freely movable, round, slow melanomas arise from a pre-existing
growing, and non-tender, unless infected. nevus
 They are firm to shotty in consistency, and contents are  Generally asymptomatic
usually under pressure.  Most women:
 Local scarring of the adjacent skin sometimes occurs o Do not closely inspect their vulvar skin
 When rupture of the contents of the cyst produces an o Are unaware of biologic changes in gross
inflammatory reaction in the subcutaneous tissue. appearance of these lesions
 Differential Diagnosis for Large epidermal cysts: o Usually recognized when become pigmented
o Fibromas at the time of puberty
o Lipomas  Majority of women who develop melanomas are in
o hidradenomas their 50s
 May develop following trauma when:  Localized nest or cluster of melanocytes
o An infolding of squamous epithelium has  Undifferentiated cells that arise from the embryonic
occurred beneath the epidermis neural crest and are present from birth
o In the site of a previous episiotomy or  One of the most common benign neoplasms
obstetric laceration  Grossly:
o The squamous epithelium which has infolded, o Blue to dark brown to black, but some
continue to produce keratin which is amelanotic
responsible for the caseous, thick cheese-like o Diameter of a few mms to 2 cm
material inside o May either be flat, elevated, or pedunculated
 Alternative theories of histogenesis include:  Differential diagnosis:
o Embryonic remnants o Hemangiomas
o Occlusion of pilosebaceous ducts of sweat o Endometriosis
glands o Malignant Melanoma
 Histology: o Vulvar Intraepithelial Neoplasia
o Characterized by an epithelial lining of o Seborrheic Keratosis
keratinized, stratified squamous  Histologically:
epithelium o Lesions are subdivided into: junctional,
o A center of cellular debris that grossly compound, and intradermal nevi
resembles sebaceous material o Junctional activity is common in vulvar nevi
o Most don’t have sebaceous cells or material  Many irritants to which vulvar skin is exposed may lead
identified on microscopic examination to malignancy
 Treatment:  Family history of melanoma is one of the strongest
o Most require no treatment risk factors for the disease.
o However, if the cyst becomes infected  Epidemiology
o Treatment consists of local heat application o The lifetime risk of a woman developing
with incision and drainage melanoma from a congenital junctional
o Cysts that become recurrently infected or nevus that measures greater than 2cm in
produce pain should be excised when the diameter is approximately 10%.
acute inflammation has subsided o The lifetime risk of a melanoma forming in
SEBACEOUS CYST women with dysplastic nevi is 15 times that
of the general population.
 Clinical features of early malignant melanoma: ABCD
o Asymmetry
o Border irregularity
o Color variegation
o Diameter usually > 6 mm
 Indication for Excision biopsy:
o Recent changes in growth or color
o Ulceration
o Bleeding
o Pain
 Benign cyst o Development of satellite lesions
 Closed sac found just under the skin
 Found deeper than the epithelial inclusion cyst DYSPLASTIC NEVUS
 Contains pasty- or cheesy-looking skin secretions
made up of keratin (usually yellow discharge)
 Most often arise from swollen hair follicles, or as a
result of skin trauma
 May grow large, become inflamed and tender
 Diagnosis:
o Physical appearance or by excision
 Treatment:
o Excision

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GYNECOLOGY
BENIGN AND MALIGNANT LESION OF THE VULVA

 A nevus > 5mm in diameter


o With irregular borders and patches of
variegated pigment
o Treatment
o Proper excisional biopsy
 Three-dimensional
 Approximately 5-10mm of normal skin around the
nevus should and underlying dermis be included
 Done under local anesthesia
 Procedure may be elective or during delivery or
gynecologic surgery

HEMANGIOMA
 Purple or dark red papules
 With irregular verrucoid surface rarely > 2cm
 Occur in women between 30-50 years old
 Noted for their rapid growth and tendency to
bleed during strenuous exercise
 Treatment: Excisional biopsy
 Differential diagnosis:
o Kaposi’s sarcoma
o Angiosarcoma

VENOUS MALFORMATIONS AND LYMPHANGIOMAS

 Both are rare


 Are rare malformations of blood vessels rather than  Venous malformations
true neoplasms o May become symptomatic at any age
 Vulvar hemangiomas frequently are discovered initially o Relatively prone to thrombosis
during childhood usually single, 1 to 2 cm in diameter, o Differential diagnosis: Vulvar
flat, and soft, and they range in color from brown to red varicosities
or purple o Treatment:
 Diagnosis:  Sclerotherapy only after
o Usually established by gross inspection of venography and Doppler
the vascular lesions ultrasound has verified the
 Types diagnosis
o Strawberry and Cavernous hemangiomas  Different from vulvar varicosities which are
o Senile or cherry angoimas exacerbated with pregnancy and tend to regress
o Angiokeratomas postpartum
o Venous malformations  Lymphangiomas
o Lymphangiomas o Similar to hemangiomas but they do not
have red blood cells in the vessels
STRAWBERRY AND CAVERNOUS HEMANGIOMA o There is absence of a muscular layer
 Congenital defects discovered in 60% of young around the vascular structures
children in the first years of life  Grossly:
 May increase in size until age 2 o They present as subdermal multiple, white
 Spontaneously regress in size by the time the child or gray, vascular nodules with epithelial
goes to school thickening
 Cavernous hemangiomas  Treatment: Surgical excision if symptoms develop
o Usually purple in color and vary in size
o The larger lesions extending deeply into the TREATMENT FOR HEMANGIOMAS
subcutaneous tissue  Rarely require therapy:
 Strawberry hemangioma o Asymptomatic hemangiomas
o Usually bright red to dark red and is o Hemangiomas in children
elevated  Subtotal resections in adults:
o Rarely increases in size after age 2 o Initial treatment of large symptomatic
hemangioimas
SENILE OR CHERRY ANGIOMAS that are
bleeding or
infected

 Cryosurgery or use of an argon laser to destroy


Hemangioma that is associated with troublesome
 Common small lesions that arise on the labia majora bleeding
 Usually in postmenopausal women  Cryosurgical treatment usually involves a single
 Most often < 3mm in diameter, multiple, and red- freeze-thaw cycle repeated three times at monthly
brown to dark blue intervals
ANGIOKERATOMA  Excisional biopsy
o When the differential diagnosis is
questionable
o Any bleeding vulvar mass

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GYNECOLOGY
BENIGN AND MALIGNANT LESION OF THE VULVA
o Performed for the definitive pathologic  Mesenchymal origin
diagnosis  Mostly in the labia majora, superficial in location
 Surgical removal of a large, cavernous  Softer and usually larger than fibromas
hemangioma or may be technically quite difficult  Grossly:
PYOGENIC GRANULOMA o Majority of lipomas in the vulva are < 3cm
o With the largest reported weight of 44
pounds
o On cut surface, substance is soft, yellow,
lobulated
 Histologically
o Lipomas are usually more homogenous than
fibromas
 Do not produce symptoms, unless extremely large
 Treatment:
 Grow under the hormonal influence of pregnancy, o Excision is usually performed to establish the
with similarities to oral lesions diagnosis
 Approximately 1cm in diameter o Smaller tumors may be followed
 Differential diagnosis, mistaken clinically for: conservatively.
o Malignant Melanomas
o Basal Cell Carcinomas SKENE’S DUCT CYST
o Vulvar Condylomas
o Nevi
 Treatment: Wide and deep excision to prevent
recurrence
FIBROMA

 Rare and small cysts


 Present with symptoms of discomfort, or found on
routine examination
 Secondary to infection & scarring of small ducts
(paraurethral glands)
 Location: near the urethra (paraurethral)
 Most common benign solid tumors of the vulva  Differential diagnosis
 Mesenchymal in origin o Urethral diverticula
 Has low grade potential for being malignant  Physical compression of the cyst should not produce
 More frequent than lipomas fluid from urethral meatus
 Occur in all age groups  In Skene’s duct cyst, the cyst produces fluid upon
 Commonly found in the labia majora compression
 Arise from deeper connective tissue  Treatment
o should be considered as dermatofibromas o If the patient is asymptomatic: none
 Smaller fibromas o If Symptomatic
o Firm, asymptomatic, discovered as  Excision with careful dissection to avoid urethral
subcutaneous nodules injury
o Becomes pedunculated as size and weight
increase HIDRADENOMA
 Larger tumors
o Become cystic after undergoing myxomatous
degeneration
 May produce chronic pressure symptoms or acute
pain when they degenerate
 Vulvar skin over a fibroma may be compromised by
pressure and ulcerates
 Grow slowly, size vary from 1-10 cm in diameter
o With a report of one gigantic vulvar fibroma
which weighed more than 250 pounds a
case report
 Grossly:  Rare, small, benign vulvar tumor
o Have a smooth surface and a distinct  Originates from apocrine sweat glands of the inner
contour surface of the labia majora (38%) and nearby
o On cut surface the tissue is gray-white perineum and labia minora (26%)
 Microscopically:  Occasionally, from eccrine sweat glands
o Has dense collagen fibers with fat or muscle  Discovered in white women between the ages of 30-
cells associated with the interlacing 70, most commonly in the fourth decade of life
fibroblasts  Not been reported prior to puberty
o Similar to leiomyomas  Grossly:
 Treatment o May be cystic (55%) or solid.
o Operative removal if symptomatic and if o With well-defined capsules
the lesion is large o Usually sessile, pinkish-gray nodules with
o Occasionally removed for cosmetic reasons white surface epithelium
LIPOMA o Not > 2cm in diameter
o Occasionally, necrosis of a central indented
area occurs, with a protrusion of reddish-
brown granulation tissue
 Confused with pyogenic granulomas
 Histologically:
o Because of its hyperplastic, adenomatous
pattern a hidradenoma may be mistaken at
first glance for an adenocarcinoma
 On close inspection, however, although there is
glandular hyperplasia with numerous tubular
ducts
 Second most frequent benign  There is paucity of mitotic figures and a lack of
 Slow-growing, circumscribed tumors of fat cells from significant cellular and nuclear pleomorphism
the subcutaneous tissue of the vulva

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GYNECOLOGY
BENIGN AND MALIGNANT LESION OF THE VULVA
 Generally asymptomatic but may cause pruritus or o Vulvar adenosis – occurs after laser therapy
bleeding if the tumor undergoes necrosis of condylomata acuminata
 Biopsy is the treatment of choice. GRANULAR CELL MYOBLASTOMA
SYRINGIOMA

 A very rare, cystic, asymptomatic, benign adenoma


of the eccrine sweat glands
 Grossly:  A rare, slow-growing, solid vulvar tumor originates
o Appear as small < 5 mm subcutaneous from neural sheath (Schwann) cells and sometimes
papules called a schwannoma
o Are either skin-colored or yellow  These tumors are found in connective tissues
o May coalesce to form cords of firm tissue throughout the body, most commonly in
o Usually located in the labia majora the tongue, and occur in any age group
 Differential Diagnosis: Fox-Fordyce disease  Approximately 7% are found in the subcutaneous
tissue of the vulva
 Twenty percent are located in the vulva usually in the
labia majora and occasionally involves the clitoris
 usually 1 to 5 cm in diameter
 benign but characteristically infiltrate the surrounding
local tissue
 slow growing, painless tumor but as they grow, they
may cause ulcerations in the skin.
 The overlying skin often has hyperplastic changes that
may look similar to invasive squamous cell
o The carcinoma.
most common differential diagnosis  Grossly
o Multiple retention cysts of apocrine glands o these tumors are not encapsulated
with inflammation of the skin o cut surface of the tumor is yellow
o Produces intense pruritus  Histologically
o Treated by oral or topical estrogens and o irregularly arranged bundles of large, round
topical retinoic acid cells with indistinct borders and pink-staining
 Treatment: Excisional biopsy or cryosurgery cytoplasm.
 Treatment involves wide excision to remove the
ENDOMETRIOSIS filamentous projections into the surrounding tissue.
o If the initial excisional biopsy is not adequate
and aggressive enough, these benign tumors
tend to recur.
o Recurrence occurs in approximately one in
five of these vulvar tumors.
o tumors are not radiosensitive
VON RECKLINGHAUSEN DISEASE
 Vulva is involved in 18% with the benign neural sheath
tumors of von Recklinghausen’s disease
 Generalized neurofibromatous and café-au-lait spots
 Lesions are fleshy, brownish red, polypoid
 Rare, only 1 in 500 women with endometriosis will  Treatment
present with vulvar lesions o Excision is the treatment of choice for
 Most common location: ovaries symptomatic tumors
 The firm, small nodules OTHER ABNORMAL TISSUES PRESENTING AS
 May be cystic or solid VULVAR MASSES
 Vary from a few millimeters to several centimeters in Differential diagnosis of vulvar masses
diameter  Leiomyomas
 The subcutaneous lesions are:  Squamous papillomas
o Blue, red, or purple, depending on their size,  Sebaceous adenomas
activity, and closeness to the surface of the  Dermoids
skin  Mullerian or wolffian duct remnants
 The gross and microscopic pathologic picture is  Epidermal inclusion cysts
similar to endometriosis of the pelvis  Sebaceous cysts
 Usually found at the  Mucous cysts
o site of an old, healed obstetric laceration  Skin diseases such as seborrheic keratosis,
o Episiotomy site condylomata acuminata, and molluscum
o An area of operative removal of a bartholin’s contagiosum
duct cvst,
 The pathophysiology of development of vulvar
HEMATOMA
endometriosis may be secondary to:
o Metaplasia
o Retrograde lymphatic spread
o Potential implantation of endometrial tissue
during operation
 Most common symptoms:
 Pain and introital dyspareunia
 Classic history:
o Cyclic discomfort and an enlargement of the
mass associated with menstrual periods.
 Treatment: wide excision or laser vaporization
 However, recurrences are common until the woman
reaches menopause
 Differential Diagnosis:

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GYNECOLOGY
BENIGN AND MALIGNANT LESION OF THE VULVA
 Allodynia – pain related to non-painful stimuli
 Treatment:
o Topical anesthetics, surgical removal of
skin

CONTACT DERMATITIS

 Usually secondary to blunt trauma such as


o A straddle injury from a fall
o An automobile accident
o A physical assault  Red, edematous, inflamed skin, weeping eczematoid
 Traumatic injuries are reported secondary to a wide vesicles
range of recreational activities  Intertriginous areas
o bicycle, motorcycle, skiing, amusement park  Treatment
rides o Withdrawal of offending substance
o Spontaneous hematomas are rare o Burrow’s solution
o Usually occur from rupture of a varicose vein o Hydrocortisone
during pregnancy or the postpartum period o Prednisone
 Management of non-obstetric vulvar hematomas o Petroleum jelly
o Usually conservative
 A pack is placed to promote hemostasis PSORIASIS
o Unless the hematoma is >10cm in diameter
or is rapidly expanding
o Usually venous in origin
o May be controlled by direct pressure
 Compression and application of an ice
pack to the area are appropriate therapy
o Operative therapy is indicated
 If hematoma continues to expand
 In an attempt to identify and ligate the
damaged vessel
 Common, generalized, unknown etiology
 During the operation careful inspection is performed
 Chronic and relapsing
to rule out injury to the urinary bladder and
rectosigmoid  Extremely variable and unpredictable course
 Marked by spontaneous remissions and
 The majority of small hematomas regress with
exacerbations
time
 Common areas of involvement are the scalp and
 Chronic expanding hematoma has underlying
fingernails
pathophysiology is the repetitive episodes of
 Vulvar psoriasis usually affects intertriginous areas
bleeding from capillaries in the granulation tissue of
 Manifested by red to red-yellow papules
the hematoma which results in a chronic, slowly
 Presence of classic silver scales and bleeding on
expanding vulvar mass
gentle scraping of the plaques may help to
o Treatment is Drainage and Debridement
establish the diagnosis
 the scales are less common in the vulva than on other
DERMATOLOGIC DISEASES
areas of the body
 Does not involve the vagina
 Most common skin diseases involving the vulva  May be the first clinical manifestation of HIV
include infection
o contact dermatitis  Treatment:
o neurodermatitis o 1% hydrocortisone cream
o psoriasis
o Initial treatment for mild disease
o seborrheic dermatitis
 4-week course of a fluorinated
o cutaneous candidiasis
corticosteroid cream ff the patient
o lichen planus
has pain secondary to chronic
 Majority of vulvar skin problems are red, scalelike
fissures or a more moderate
rashes
disease
 Primary complaint is usually pruritus
 If this treatment is not successful, a dermatologist
should be consulted
PRURITUS
 Intense itching, desire to scratch, “itch-scratch SEBORRHEIC DERMATITIS
cycle”  Rare, etiology unknown
 Management  Grossly:
 Establish diagnosis and treat offending cause o Pale to yellow-red erythematous, edematous
 Improve local hygiene lesions with oily scales
 Itch-scratch cycle must be interrupted before  Treatment: Hydrocortisone cream
condition becomes chronic
 Resulting in lichenification (lichen simplex chronicus) LICHEN PLANUS
VULVODYNIA
 Chronic vulvar discomfort, burning, stinging, and
rawness

VULVAR VESTIBULITIS
 Unknown etiology
 Pain and burning at introitus
 but not inflammation

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GYNECOLOGY
BENIGN AND MALIGNANT LESION OF THE VULVA

 VIN III – Severe (CIS)


 Paget’s disease
 Melanoma in situ
SQUAMOUS CELL HYPERPLASIA

 Chronic eruption of shiny, violaceous papules


 Involves inner aspects of the vulva
 Etiology: Local autoimmune cell mediated response
 Symptoms: Pruritus & pain, burning, scarring
 Diagnosis: small punch biopsy
 Treatment: topical steroid cream

VULVAR CANCER
 3-5% of female genital tract malignancies
 Fourth in ranking among Female Genital Tract
cancers
 Most common is cervix
 2nd most common: ovary
 3rd most common: uterus  Elongation and widening of the rete ridges
o Think, COUV  Hyperkeratotic surface layers
 90% of vulvar cancers are Squamous cell CA  Grossly: whitish or reddish
 Disease of older women (60 years)
 Increase in vulvar intraepithelial neoplasia (VIN) and LICHEN SCELROSUS
invasive vulvar CA (<50 years)
 Prognosis: good if found early
 Premalignant & malignant changes arise at
multifocal points on the vulva
o May arise from carcinoma in situ
 However, many cases develop in the absence of
premalignant changes
 Significant impact on sexuality
 Advances in management:
 More conservative surgery and improved
psychosexual outcomes  Whitish change in vulvar skin
 Early detection and biopsy of any abnormal vulvar  Epithelium becomes markedly thinned with loss or
lesions are imperative blunting of the rete ridges
 Diagnose early stages  “cigarette paper” appearance
 Improve subsequent morbidity and mortality  In some, thickening or hyperkeratosis of the surface
PREDISPOSING FACTOR layer
 Well defined predisposing factors are not identified  4.5% risk of developing into vulvar CA (HPV-negative)
 Many cases, develop in the absence of premalignant  Usually clitoral in location
lesions  Some developed malignancies in the cervix, colon,
 Occasionally, invasive CA arises from CA-in-situ breast, ovary, and endometrium
PREDISPOSING FACTORS FOR VULVAR CANCERS  “Itch-Scratch-Lichen Sclerosus Hypothesis”
o Severe pruritus leads to Squamous cell
o Human Papilloma Virus
hyperplasia progression of SCH leads to
 Evidence based link to Vulvar CA
atypia formation
 HPV DNA-associated CA were
o Atypia leads to VIN then to invasive
found in younger patients
squamous cell cancer
 HPV-positive tumors associated
 Aggressive evaluation and treatment has a dramatic
with VIN, warty of basaloid, and
impact on the incidence of vulvar cancer
good prognosis
 Treatment with topical steroids would prevent vulvar
 HPV-negative is associated with
cancer upon prevention of scratching
keratinized lesion, more like to recur
and lead to death and has poorer
prognosis
o Granulomatous disease of the vulva
o Hypertension
o Diabetes Mellitus
o Obesity – 25% of patients
 Recently, pre-malignant lesions have increased in
women in 20-30s Hyperkeratosis of the epithelium
o Multiple sexual contact VULVAR INTRAEPITHELIAL NEOPLASIA
o Venereal diseases (i.e. HPV)
o Immunosuppression
 There is also increasing frequency in those treated for
squamous cell CA of the cervix or vagina due to
increased carcinogenesis in the squamous epithelium
of the lower genital tract
 Age
>50% 65-75 y.o.
15% <40 y.o.
2% to 21% < 50 y.o
incidence has increased
from over the past 20
years
40-55 years old: carcinoma in situ

VULVAR ATYPIA
 Squamous cell hyperplasia Loss of maturation in squamous epithelium
 Lichen sclerosus  Microscopic features:
 Intraepithelial Neoplasia/ Dysplasia o Multinucleated cells
 VIN I – Mild o Abnormal mitoses
 VIN II – Moderate o Increased density in cells
o Increase NC ratio

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GYNECOLOGY
BENIGN AND MALIGNANT LESION OF THE VULVA
 Classification  Biopsy
o Mild (VIN I) involve lower third of the o Keyes dermal punch
epithelium o No gross features are diagnostic of
o Moderate (VIN II) ½ to 2/3 of epithelium is vulvar cancer
involved o Done on any suspicious lesions of
o Severe (VIN III) over 2/3 involved the vulva, asymptomatic or
 Carcinoma In Situ symptomatic
o Full thickness of epithelium o Full thickness of the skin is incised
 VIN is a premalignant finding o For 3-5mm lesions
 More than 1/3 of vulvar CA are associated with VIN  Done under local anesthesia
 VIN tends to be multifocal and regress in majority  Rotated downward to obtain a
 Risk of progression is higher in old and disk of entire thickness
immunosuppressed  Indications for biopsy (PIC-C)
 Risk of invasion is higher among raised lesions with  Persistent ulceration
irregular surface patterns  Itchy area
 Increased prevalence in young associated with HPV  Confluent, wart-like mass
subtype 16, 18  Change in the color, elevation, or
 A study evaluated tissue samples from 48 patients surface of a lesion
 Age: 45-88 y.o. MANAGEMENT OF VULVAR ATYPIAS
 48% - HPV DNA was detected by PCR  Relief of itching
 95% - HPV subtypes 16 and 18 o Topical corticosteroids
 HPV detection was not associated with age o 1% hydrocortisone
 71% - associated with coexisting severe (VIN III)  To avoid vulvar contraction due to long-term
corticosteroids
PAGET’S DISEASE o Cotton underclothes
o Avoid strong soaps and detergents
o Burrow’s solution
 Lichen Sclerosus
o Topical testosterone OD or BID
o Side effects: clitoral hypertrophy and
increase hair growth
o Local Progesterone
o Surgical excision :severe contracture in
the posterior fourchette
 Rare intraepithelial disorder HPV INFECTION
 Resemble Paget’s disease of the breast  HPV 6, 11 – benign warts
 Paget’s Cells  HPV 16, 11, 31, 33, 35, 45 – VIN
o Large pale cells that often occur in nests  80% of women 50 years old have acquired HPV at
and infiltrate upward through the some point in their life
epithelium  2/3 had pruritus and dyspareunia
 Major importance:  Complicated management because:
 Associated with invasive adenocarcinoma of the o It is extremely prevalent
vulva, vagina, anus, and distant sites (bladder, cervix, o Risk of progression to VIN is small
colon, stomach, and breast)  Best to restrict therapy to individuals with bothersome
 Tends to spread occulty symptoms such as warts and VIN
 Recurrences are frequent after treatment  Asymptomatic (koilocytosis) – no treatment but
 Usually seen in post-menopausal women requires follow up
 Diffuse erythematous eczematoid lesion VAGINAL INTRAEPITHELIAL NEOPLASIA
 Itching is a problem  Treating itching and dyspareunia
 Frequent association with carcinoma  VIN lesions tend to be posterior
o Squamous CA of vulva or cervix o predominantly in the perineal area
o Adenocarcinoma of sweat glands of  Wide local excision
vulva  Risk of recurrence 50% if margins were involved
o Bartholin’s gland CA  10% risk of recurrence if margins not involved
o Adenocarcinoma of breast and GIT  Recurrence may occur even if margins are clean, so
long term follow-up is mandatory
CO2 LASER
CLINICAL PRESENTATION OF VULVAR ATYPIAS
 Vulvar condyloma and VIN
 Irritation or itching most common  Vulvar CIS treated successfully
 Whitish change due to thickened keratin layer  Mandatory to do colposcopy and biopsy in the
 Lichen sclerosus diagnosis of the lesions
o Diffuse lesions  VIN lesions require slightly deeper ablation, about 1-3
o Skin appears thin, with scarring and mm deeper for areas with hair
contracture, fissuring, excoriation  Washing lesions with 5% acetic acid can aid in
o Cigarette paper appearance identifying the involved areas
 Squamous hyperplasia  Eradication of abnormal vulvar tissue and healing
o Focal or multifocal without scarring
o Vulva is thickened  Vaporization of skin that is too deep causes scarring
 VIN  Effective in a single treatment in 78.4% of cases
o white, red, or pigmented  96.8% cure with second laser treatment for multiple or
 Carcinoma In Situ large lesions and those with recurrent VIN
o 1/3 with CIS present with pigmented lesions  Healing is usually complete within 2 weeks
o Lesions are discrete and multifocal  Good result if used with other vulvar lesions
o Occur more frequently in those who have o Molluscum Contagiosum
had squamous cell neoplasia of the cervix o Lichen Sclerosis Atrophica
 Paget’s disease
o Chronic Vestibulitis
o Reddish eczematoid lesions o Other Dermatologic Lesions
DIAGNOSTICS  Postoperative pain following laser treatment can be
 Pap smear severe
o Not helpful because vulvar skin is thick o Local treatment with lidocaine gel
and keratinized o Stronger analgesics
o May help if there is ulceration o Local care with sitz baths
 Colposcopy
o Not used for routine vulvar examination PAGET’S DISEASE
o Used for those who are being followed
 Wide local excision if no malignancy found
for vulvar atypias
 Long follow-up
o 3% acetic acid highlights whitish areas
 Annual breast exam, Pap smear, screening for GIT
for biopsy
disease

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GYNECOLOGY
BENIGN AND MALIGNANT LESION OF THE VULVA
MALIGNANT LESIONS
SYMPTOMS
 Long history of pruritus – most common
 Vulvar bleeding
 Discharge
 Dysuria
 Pain
SIGNS
 Vulvar lump or mass – most common
 large, fungating mass – rare
CLINICAL FEATURES
 Lesion usually raised, fleshy, ulcerated, leukoplakic,
or warty in appearance
 Unifocal and occur on the labia majora
 5% - multifocal
 Labia minora, clitoris, or perineum may be primary
sites
SCREENING
 Any vulvar lesion must be thoroughly evaluated to
rule out malignancy
 Routine annual visual inspection of the external
genitalia even if the patient is no longer receiving
annual Pap smears
 Teaching female patients about vulvar self-
examination
HISTOLOGICAL TYPE

Squamous cell carcinoma -90% Malignant schwannoma


Sarcomas Melanoma -2-9%
o Leiomyosarcoma Bartholin’s gland carcinoma
o Epithelioid sarcoma Adenocarcinoma
o Rhabdomyosarcoma Basal cell carcinoma
Lymphoma Verrucous carcinoma -often
Endodermal sinus tumor misdiagnosed as condyloma TREATMENT
Merkel cell carcinoma acuminata  Microinvasive Carcinoma
Dermatofibrosarcoma o Wide excision with 1-2cm margin
protuberans o Hemivulvectomy
o Lymph node dissection may be deferred
 Surgical resection -gold standard
CANCER SPREAD  Should completely remove the cancer and identify the
extent of disease
Lymphatic embolization Direct extension  Determine the stage and the need for additional
o Inguinal lymph nodes Hematogenous spread therapy
o Femoral lymph nodes to distant sites  Radical vulvectomy with bilateral dissection of the groin
o Pelvic lymph nodes and pelvic nodes was recommended
o External iliac nodes  Presently, more individualized and conservative
approach

 Lymph node metastasis – 30% overall


 Middle of either labium drain initially to ipsilateral
femoral-inguinal nodes
 98% ipsilateral spread
 2% contralateral spread
 In clitoral or urethral areas spread to either side
 Risk increases as the stage of disease, size of the
lesion, and depth of invasion increase Radical vulvectomy or a radical local excision
 Pelvic node metastases are uncommon – 2-12% o Aim: to remove the primary lesion with a 2-3
 Usually not found in the absence of clinically cm margin
suspicious inguinal and femoral nodes
 25% with groin node metastases have positive
pelvic nodes
PROGNOSIS
 Related to stage, lesion size, lymph node status
 Lymph node involvement and size:
 42% if lesion > 2cm
 19% if lesion ≤ 2cm
 Five-year survival by stage of disease and lymph
node status
Stage I 98% Inguinal-femoral lymphadenectomy
Stage II 85% o Treat deep pelvic nodes with external radiation
Stage III 74% o If >2 groin nodules are positive or there are clinically
Stage IV 31% suspicious groin nodules
 Additional predictors of positive nodes: o Do postoperative groin and pelvic radiation therapy
o Tumor de-differentiation Recurrence in the groin
o Suspicious, fixed or ulcerated lymph
o Appears to represent persistent disease
nodes
o Occurring early and near the treated site among
o Capillary-lymphatic space involvement
patients treated conservatively
o Older age
 Risk of recurrent disease increases with the number
o Tumor thickness
of positive groin nodes
STAGING  Local recurrences
 Repeat surgical excision
 Radiation therapy with surgery
o To treat groin recurrence
 Chemotherapy
o For systemic metastasis
o Poor response rates
o Ineffective in the treatment of recurrent disease

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GYNECOLOGY
BENIGN AND MALIGNANT LESION OF THE VULVA
BARTHOLIN’S GLAND CARCINOMA o Flat or ulcerated, nodular surrounding the
 Adenocarcinoma primary lesions
 1-2% of vulvar carcinomas  Treatment: Wide Excision
 Sign:  Prognosis:
o Enlargement of the Bartholin’s gland in a o 5-year survival – 71%
postmenopausal patient o For nodular melanoma – 38%
 The American Joint Committee on Cancer (AJCC)

 Treatment: TNM classification system and the International


o Radical vulvectomy with bilateral Federation of Gynecology and Obstetrics (FIGO)
inguinal-femoral lymphadenectomy staging system for vulvar cancer
 Prognosis is good if nodes are negative
BASAL CELL CARCINOMA REVIEW QUESTIONS
 Can arise in the vulva and any skin on the body 1. What is the treatment for Bartholin’s cyst carcinoma?
 2% of vulvar carcinomas 2. What is the treatment for microinvasive carcinoma?
 Sign: ulcerated lesion 3. What is the classification of VIN if it involves more than
 Treatment: wide local excision 2/3 of the epithelium?
 If surgical resection margins are free of tumor, the 4. Slow-growing, circumscribed tumors usually larger
disease is cured than fibroma and does not present with pressure
VERRUCOUS CARCINOMA symptoms?
 Special variant of squamous cell cancer 5. There is already extension of tumor to the perineal
 Sign structures. What is the stage of the tumor?
o Large condylomatous mass on the vulva 6. What is the management for lichen sclerosus if there is
 Histopath: already severe contracture in the posterior fourchette?
o Mature squamous cells 7. What are the indications for biopsy of vulvar lesions?
o Extensive keratinization with nests that 8. What is the initial treatment for the cigarette paper
invade the underlying vulvar tissue appearance lesion of the vulva?
 Treatment: Wide excision 9. What is the most common symptom of malignant lesion
MELANOMA of the vulva?
10. This tumor originates from the neural sheath.
 Most frequent non-squamous cells malignancy of
the vulva
 5% of vulvar cancers
 Arise from junction or compound nevi
 Age: 50 years
 Grossly,
o Brown, black or blue-black masses

TRANSCRIBERS: GyneGirls (GG) - AGUIRRE, TAAN Page 10 of 11


BENIGN AND MALIGNANT LESIONS OF THE VULVA
4.05a
DR. MARIA CRISTINA ESTRELIA-SANTOS 02/22/2017

TRANSCRIBERS: GyneGirls (GG) - AGUIRRE, TAAN Page 11 of 11

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