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PRE-MALIGNANT AND
MALIGNANT CONDITIONS
OF VULVA
Dr. Ujjwal Khullar
Dr. Prachi Kushwaha(GUIDE)
• VIN (vulvar
intraepithelial
neoplasia) is the
premalignant condition of
the vulva where the
cellular changes are
limited to the epithelium
but do not extend beyond
the basement membrane.
• Also known as the
squamous
intraepithelial lesions
of the vulva
VIN
GRADING
• VIN 1 – atypical cells are found only in
deeper 1/3 of epithelium
• VIN 2 –intermediate state b/w 1 & 3
• VIN 3 – atypical epithelium is present
throughout the whole depth of
epithelium , k/a CARCINOMA in SITU.
 Basement membrane is intact in all.
• The vulva is the area
immediately external
to the vagina,
includes-
the mons pubis,
labia,
clitoris,
Bartholin glands,
perineum.
 Anatomically, the
vulva extends from-
• Anteriorly- Mons
pubis
• Laterally- Genito-
crural folds
• Posteriorly- Anus
The normal findings in
the vulva include-
• Micropapillomatosis,
• Sebaceous glands and
• Vestibular redness.
 It is important to be aware
of normal findings and not
mistake them for vulvar
lesions.
The anatomical structures in the vulva
CLASSIFICATION
• NON-NEOPLASTIC EPITHELIAL DISORDER OF SKIN AND MUCOSA
- Lichen sclerosis
- Squamous hyperplasia
- Other dermatosis
• MIXED NON-NEOPLASTIC AND NEOPLASTIC EPITHELIAL DISORDERS
• INTRAEPITHELIAL NEOPLASIA
-squamous intraepithelial neoplasia
 VIN 1
 VIN 2
 VIN 3
-non squamous intraepithelial neoplasia
paget`s disease
tumours of melanocytes, noninvasive
• High-grade squamous intraepithelial lesion
(HSIL) has replaced the term ‘VIN usual
type’ and includes the warty, basaloid and
mixed lesions.
• It is associated with high-risk HPV infection,
by HPV 16, 18 and 33.
• Other risk factors are smoking, HIV
infection and STDs.
• VIN usually associated with vulvar lichen
sclerosus and not associated with HPV and
occurring in older women.
PATHOLOGY
• Macroscopically-
the lesion may be a
warty, elevated plaque
that is white or red, an
ulcer or a nodular lesion
• The lesions may be
multifocal.
• Differentiated VIN is
usually associated with
lichen sclerosis and
squamous cell
hyperplasia.
Microscopically ,the
cells exhibit -
• Nuclear abnormalities
• Maturation
disturbance
• Increase in mitotic
activity
• Sometimes changes of
HPV infection.
DIAGNOSIS
Symptoms of VIN-
• Asymptomatic
• Pruritus vulva
• Ulcer
• Discomfort, pain
• Warty growth
• Bleeding, discharge
EXAMINATION
• VULVOSCOPY- with 3-
5% acetic acid is
helpful.
• Acetic acid enhances
– surface topography
and acetowhite areas.
• Helps to see – coarse
punctations
• LESSIONS SEEN ARE-
1. White hyperkeratotic
plaques
2. Hyper pigmented lesions
3. Areas of erythema
4. Raised / flat lesions
5. Bulky lesions
6. Condylomatous lesions
7. Multifocal lesions
In cases of DIFFERENTIATED VIN
(Dvin)-
• Lesion is single with
LICHEN SCLEROSES /
LICHEN SIMPLEX near
vulval skin.
• Lessions are-
- Ulcer
- Warty papule
- Hyperkeratotic
plaque
BIOPSY
• Which lessions to be
biopsied?
- Irregular
- Asymmetric
- Dark
- Large
- Elevated
- Rough
- Nodular
- Ulcerated lession with
induration
- Inguinal
lymphadenopathy
• KEYES PUNCH BIOPSY-
- Apply local anesthetic injection ,
apply acetic acid.
- Aceto-white area is biopsied.
- Alternatively biopsy can be taken
by cervical punch biopsy forceps /
knife
- About 6mm diameter
suspicious area is taken and
one or two haemostatic
sutures are taken with vicryl-
2-0.
- Lesions of CLITORIS – to be
done under GA.
MEDICAL TREATMENT FOR VIN
• Topical steroid therapy
(TST)
• Topical 5-fluorouracil
(TFT)
• Alfa interferon (under
trial)
• Even with medical
treatment routine
biopsy is essential.
TOPICAL STEROID
THERAPY (TST)
 CLOBETASOL
PROPROIONATE cream
 FLUOCINOLONE oint.
 HALCINONIDE cream.
 5-FLUOROURACIL cream
DOSE- TDS for 3-4 weeks.
Above is not definative
treatment.
MANAGEMENT OF
VIN -
• Local excision is done for
vulvar HSIL and VIN
differentiated.
• Disease-free margin of 5 mm
should be obtained.
• Large, confluent lesions
require more extensive
excision and rotational flap
for skin grafting.
• Young women with VIN
may be treated by CO2 laser.
• Immune response
modulators such as
imiquimod are under trial.
TREATMENT OF HIGH GRADE SIL OF
VULVA / VIN 2 AND 3
• High grade VIN is treated.
• VIN on skin surface tend to be deeper involving
pilosebaceous units in 66% cases and is limited to
2.5mm depth from epidermal surface
• Mucosal surface lessions are more superficial.
• Extensive excision is needed.
WIDE LOCAL
EXCISION
• Treatment of
choice- large
advanced VIN in
which probability of
vulvar cancer can`t
be excluded.
• A surgical margin of
at least 5 mm is
prefered
• Recurrence rate –
20%
LASER ABLATION
• CO2 gives good cosmetic
results and depth of tissue
distruction can be
controlled
• ADVANTAGES-
- It is less disfiguring as
compared to excision
- Less bleeding
• DISADVANTAGES
- Prolonged painful healing
- No tissue specimen
- More reurrence – 25%
CAVITATIONAL
ULTRASONIC
SURGICAL ASPIRATION
(CUSA)
- Done on non- hairy skin
- Ultrasound is used to
cause cavitation and
disruption of affected
tissue.
- Less pain and scaring
- Tissue obtained is in
fragments
- Recurrence -35%
TOPICAL MEDICAL
THERAPY
- Topical Imiquimod
cream applied over the
affected area has lower
toxicity than 5-
Fluorouracil and
Cidofovir.
- Efficacy- 77% cure rate
- Recurrence- 20%
- PREVENTION- HPV
vaccine.
- FOLLOW UP- after 6
months and then yearly.
BOWEN’S DISEASE
• It’s intraepithelial
carcinoma of Vulva.
• CLINICAL FEATURES-
slowly growing hard red
indurated patch on vulva
with intense itching.
• EXAMINATION-
- Well demarcated reddish
indurated lesion on vulva
with eczematous dry
surface.
- BIOPSY- pickle cells / giant
cells (BOWEN CELLS)
- TREATMENT- vulvectomy
- Prognosis- good
- Metastasis- rare
PAGET’S DISEASE
• Extra mammary disease on
vulva simulating intraductal
breast carcinoma with
involvement of apocrine sweat
glands
• May have associated
ADENOCARCINOMA in apocrine
and Bartholin gland
• May involve perianal and anal
skin.
• CLINICAL FEATURES-
- Pruritis
- Vulvar discomfort
- Pain
EXAMINATION-
• Elevated white indurated
eczematous lesions.
• Vulval biopsy- typical paget
cells in the epidermis
• These cells are large pale
vaculated rounded cells with
pale cytoplasm and vesicular
nuclei.
• Secreats- mucous
• May be asso. With
adenocarcinoma of Bartholin
glands in 20% cases.
TREATMENT-
- Simple vulvectomy
- Treat like vulval cancer – radical vulvectomy
with inguino- femoral lymphadenectomy.
- Radiaotherapy for elderly unfit women.
- Topical BLEOMYCIN or 5- fluorouracil
- Recurrence- 20-25%
CARCINOMA VULVA
• Vulvar cancer accounts for about 4% of cancers of
the female genital system in India.
• Most common age group - >65-70 yrs
• Median age 68 yrs.
• Most common variety of vulval cancer –
SQUAMOUS CELL (90-92%) followed by
melanoma (2-4%).
• Most common SITE for vulval carcinoma- HARTS
LINE f/b labia minora and L. majora.
ANATOMY
Possible signs of vulvar cancer include
bleeding or itching.
• -A lump or growth on the vulva.
• Changes in the vulvar skin, such as color
changes or growths that look like a wart or
ulcer.
• Itching in the vulvar area, that does not go
away.
• Bleeding and Tenderness in the vulvar area.
HISTOLOGICAL SUBTYPES
Most Common Histological types
• Squamous: >90%
• Melanoma: 5-10%
• Basal Cell: 2%
• Sarcoma: 1-2%
• Paget: <1%
• Bartholin gland: rare
TYPES OF SQUAMOUS CELL
CARCINOMA
TYPE 1 WARTY /
BASALOID TYPE-
• Young women <50 yrs
• Multifocal
• Related to HPV infection
, vulval intraepithelial
neoplasia , smoking ,
STD ,
immunosuppresion.
• PROGNOSIS- good
TYPE 2 KERATINIZING ,
DIFFERENTIATED /
SIMPLEX TYPE
• Elderly
• Unifocal
• Related to vulval ds-=
like- lichen sclerosis ,
squamous hyperplasia
• GENE mutation
responsible – p53
ETIOLOGY
• Older age
• Precancerous changes (dysplasia) in vulvar tissues
• Lichen sclerosus, which causes persistent
• itching and scarring of the vulva
• Human papillomavirus (HPV) infection
• Cancer of the vagina or cervix
• Heavy cigarette smoking
• Chronic granulomatous disease (a hereditary
disease that impairs the immune system)
HUMAN PAPILLOMA VIRUS
INFECTION (HPV)-
HPV mainly 16 and 18 , 31 , 33 are responsible
• HPV DNA is detected in 90% cases of VIN and
only 50-70% cases of vulval carcinoma ,i.e., only
basaloid and warty types
• RISK FACTORS- smoking and HPV genital warts
• HPV VACCINE reduces incidence of vulval
carcinoma.
• HERPES SIMPLEX VIRUS- is weakly associated
, along with co-factors like smoking.
• CHRONIC IMMUNOSUPPRESSION- transplant
patients on immunosuppressive drugs , HIV
women especially in younger age
- Hence vulval biopsy must be done for any
suspicious lesions specially in
immunocompromised patients.
• VIN-2 and VIN 3 can develop
into carcinoma with in 4
years
CLINICAL PRESENTATION
SYMPTOMS-
• Asymptomatic
• Vulvar pruritis of long
duration ( commonest
symptom)
• Vulvar irritation
• Vulvar pain
• Vulvar mass (Warty
growth)
• Non healing vulvar ulcer
• Vulvar bleeding
• Vulvar discharge
• Dysuria and difficult
micturition (involvement
of urethra)
• Rectal bleeding and
painful defecation
(involvement of anal
canal)
• Inguinal mass ( metastatic
lymph nodes)
SIGNS
Vulvar examination may
reveal-
• Irregular fungating mass
• Irregular ulcer
• Warty growth
• Plaque like lesion
• Red or white
pigmentation on vulvar
lesion
• Tenderness over lesion
(may or may not be
present)
• Unilateral or bilateral
inguino-femoral
lymphadenopathy
Parts involved-
Labia Majora or minora (60%), Clitoris ( 10%),
Perineum ( 10%)
PARTS INVOLVED ARE-
HISTORY
• Careful history taking for symptoms and their
duration.
• Past history of sexually transmitted disease ,
any condylomata or vulval disease (lichen
sclerosis)
• HISTORY- smoking and immunosuppressive
drugs.
EXAMINATION
• Careful examination of
vulva should be done ,
look for –
 Size
 Location
 Extent of lesion
 Warty growth or
 Ulcerative growth
 Single / multifocal
 Association with lichen
sclerosis
 Involvement of vagina
Urethra
Base of bladder /
anus
• PER SPECULAM EXAMINATION- to
look for extension to vagina and cervix
• Per vaginal and per rectal examination is also
important.
• Any fixity of vulval lession to bone is looked.
• Inguinal regions should be palpated for
inguinofemoral lymph nodes , their enlargement
,size , number and fixity to skin.
DIAGNOSIS
• If the lesion is not visible we can go for
VULVOSCOPY with 3% Acetic acid to look for
aceto- white areas.
• DEFINATIVE DIAGNOSIS- BIOPSY
TYPES OF BIOPSY
• Keyes punch biopsy :-
– Done under local anesthesia.Skin over lesion is made taut
with left hand.
– The keyes punch is put against the lesion firmly and rotated
with a constant firm pressure clockwise and then counter
clockwise for penetration into skin and to reach
subcutaneous fat.
– The raised circular tissue is grasped and cut at base with
scissors .
• Wedge biopsy :-
– Taken with knife.
– 2-0 chromic catgut or vicryl suture is applied for hemostasis.
INVESTIGATIONS
• PAP smear for cervial and vaginal cytology.
• Colposcopy of cervix and vagina.
• Vulvoscopy for inspection of other lesions.
• Imaging modalities:-
– Ultrasonography pelvis
– CT or MRI to see extent of disease and metastasis.
– Cytourethroscopy to rule out involvement of urethra and
bladder.
– Proctosigmoidoscopy.
– Intravenous urogram.
• Lymphography to detect smaller lymph node
metastasis.
Routine preoperative workup:
PAP smear
Serology
Complete hemogram
Blood gp (rh typing)
Urine routine and microscopy
Urine culture
LFT
KFT
BT/CT
X-ray chest
ECG
DEPTH OF INVASION
• Tumor shows classic
diagnostic features
of invasive
squamous cell
carcinoma that
include-
• a squamoid
appearance
• intercellular bridges
• brightly eosinophilic
keratin pearls
• Nests of invasive
tumor are
surrounded by
chronic
inflammation.
STAGING OF CARCINOMA VULVA
MODE OF SPREAD
• Direct extension :- To adjacent structures (vagina,
urethra , anus , clitoris )
• Lymphatic spread :- It is common and occurs by
embolization to regional lymph nodes.
Superficial inguinal lymph nodes
Deep inguinal and femoral nodes
Pelvic lymph nodes.
INITIAL SPREAD-
INGUINAL L.N (b/w Camper`s fascia ant. fascia
lata)
CLOQUET’S L.N / ROSENMULLER NODES
(SENTINAL LYMPH NODE of carcinoma vulva)
These are deep femoral nodes situated beneath
round ligament medial to femoral vein.
• Hematogenous spread : - Occurs rarely in the
late stages.
NORMAL LYMPHATIC DRAINAGE
• From VULVA+ Dist.3ed of
VAGINA.
• Drains into superficial
inguinal nodes.
• Lymph travels through
DEEP FEMORAL
Lymphatics and node of
CLONQUET to pelvic L.N
• Lymph can directly also
drain into DEEP
FEMORAL nodes from
CLITORIS and UPPER
LABIA
All lessions within 2cm of
midline may spread via
lymphatics.
DIFFERENTIAL DIAGNOSIS
• Syphilitic ulcer
• Tubercular ulcer
• Lymphogranuloma
venereum
• Granuloma inguinal
• Chancroid
• Vulvar elephantiasis
• Lichen sclerosis
TREATMENT
• SURGICAL TREATMENT :-
– Main treatment modality.
– Need to have adequate resection margin of 1 cm
and groin node dissection.
WIDE LOCAL EXCISION/ SIMPLE
PARTIAL VULVECTOMY
• For micro-invasive tumor (stage IA).
• 2cm tumour free surgical margin is
to obtained around the lesion
• Deep surgical margin should also
be 1 cm which roughly corresponds
to colles fascia.
• Lymph nodes are not removed.
RADICAL PARTIAL VULVECTOMY
• Partial vulvectomy is typically reserved for unifocal
lesions that are clinically connected to the labia majora,
labia minora , mons , vestibule, and/or perineum and
that have limited involvement to the adjacent urethra,
vagina, and/or anus.
• Complete removal of tumor containing portion of the
vulva wherever it is situated with 1 cm skin margins and
excision to the pelvic membrane.
• Combined with ipsilateral inguinofemoral
lymphadenectomy .
RADICAL PARTIAL VULVECTOMY
Its VARIATIONS are-
1. Right / left vulvectomy –
one side of L. majora and
minora.
2. Ant. Hemivulvectomy-
clitoris+ partial resection of
L. minora+ majora and mons
pubis
3. Post. Hemivulvectomy-
portion of L.majora ,
bartholin gland and upper
perineal body.
RADICAL TOTAL VULVECTOMY
• Removal of entire VULVA till
Perineal membrane + pubic
symphysis.
• Two elliptical incisions are made
on either sides of vulva.
• INNER INCISION- On vaginal
introitus and vestibule ant. To
urethral meatus post. to clitoris.
• OUTER INCISION- along
Labiocrural folds and Ant.across
mons pubis and post. Across the
perineal body.
• the en bloc incision,
also termed the
butterfly or longhorn
incision, made from one
ASIS to other.
• Its abandoned.
• Survival rates
equivalent to radical
complete vulvectomy
but carries signicantly
greater morbidity.
• After complete dissection ,
levator ani muscle are
approximated to prevent
RECTOCELE.
DISADVANTAGES-
 Complete loss of vulval
tissue with psychosexual
complications.
 Wound break down in 50%
 High rate of lymphedema
(65%)
NOT USED NOW A DAYS.
SENTINAL LYMPH NODE BIOPSY
• The FIRST LYMPH NODE to
receive tumour lymphatic
drainage is termed as
SENTINAL L.N
• If on biopsy sentinal node is
NEGATIVE , whole chain of
lymph nodes above it is also
likely to be NEGATIVE.
• NOT DONE – if groin
metastasis is suspected
• Done with
LYMPHOSCINTIGRAPHY with
Tc99m labelled nano colloid /
isosulphan blue injected into
skin around tumour.
• 5 mins late if TRACES of
dye +nt , excision under
microscope
INDICATIONS-
• Unifocal primary vulval
cancer
• <4cm diameter
• Tumour invasion <1mm
• Absence of obvious
metastasis on clinical
and radiology
• Absence of suspicious
inguino- femoral L.N
MANAGEMENT OF LYMPH NODES
 L.N DISECTION-
INGUINO-FEMORAL L.N
DISECTION
If carcinoma is within
2cm of midline
BILATERAL INGUINO-
FEMORAL LN DISECTION
If cancer is >2cm away
from midline
UNILATERAL INGUINO-
FEMORAL L.N DISECTION
APPROACH TO LYMPH NODE
DISECTION
5 YEAR SURVIVAL RATE
• SINGLE most important
factor for PROGNOSIS is
INVOLVEMENT OF
LYMPH NODES.
L.N INVOLVED
5 YRS survival rate – 85%
L.N NOT involved
5 yrs survival rate -50%
DEPENDING ON
DEPTH OF INVASION-
1. <1MM – L.N not
involved , no
disection needed
PROGNOSIS- GOOD
2. >1MM – need
disection
PROGNOSIS- POOR
LYMPH NODE METASTASIS
• OVER ALL L.N metastasis- 25% cases of vulval
cancer.
STAGE LYMPH NODE
METASIS
1 10%
2 30%
3 75%
4 98-100%
ROLE OF RADIOTHERAPY
INDICATION-
• PREOPERATIVELY in pt
with advanced disease
with fixed L.N
• POSTOP- pts with positive
inguinal L.N
• POSTOP- pts with close
surgical margins of <5mm
, to prevent recurrence.
• In young patients , to
prevent severe
psychological effects of
radical surgery
• Recurrent disease
• Women unfit for surgery
/ anaesthesia.
TREATMENT- usually
teletherapy of 45-55GY.
ROLE OF CHEMOTHERAPY
• Limited role
• Mostly used as
neoadjuvant therapy
with radiotherapy in
advanced cases.
• Done where surgery is
not possible.
DRUGS USED ARE-
 CRISPLATIN
 5-FLUROURACIL
 BLEOMYCIN
 MITOMYCIN C
5 YEAR SURVIVAL RATE
• STAGE 1 & 2 - 75-90%
• STAGE 3 – 54%
• STAGE 4B- 10%
• If INGUINAL lymph nodes are involved – 50%
FOLLOW-UP 6 monthly follow up after treatment is
completed and wound is healed till 5 yrs
• AIM- to detect recurrence , new primary lymph
node
• Patient should be looked for Ca vagina and cervix.
RECURRENCE
• About 30% vulval
cancer recur.
• 50% recurrence- if
margin is <8mm.
VULVA 70%
GROIN 24%
PELVIS 15%
DISTANT ORGAN
18%
MANAGEMENT OF
RECURRENCE-
VULVA- radiotherapy +/-
wide local excision
GROIN- radiotherapy/
radical groin dissection.
PELVIS- palliative
radiotherapy with
chemotherapy
DISTANT ORGAN-
palliative chemo. , poor-
prognosis. (<1 yr)
VULVAL MELANOMA
• 2nd MOST COMMON
vulval cancer (2-5%)
• Elderly
• 5 yrs survival – 10-50%
• Arises from L. majora ,
minora and clitoris.
• Hyperpigmented nodule
on vulva
• DIAGNOSIS- biopsy
TREATMENT-
• Radical Partial
vulvectomy with
inguino-femoral
lymphadenectomy.
• Prognosis- poor
• Chemotherapy –
alpha interferon,
interleukin-2 (new).
VERRUCOUS CARCINOMA OF VULVA
• Rare type of Squamous
cell Ca. of vulva
• Incidence- <1%
• Associations has been
seen with HPV genome
• Clinical features-
vulval mass , pain and
pluritis
• On examination –
cauliflower like tender
growth.
TREATMENT-
• Wide local excision with 1cm
surgical margin
• Usually no lymph node
involvement
• If needed FNAC of inflammed/
suspected L.N can be sent
• RADIOTHERAPY is
CONTRAINDICATED as it may
stimulate anaplastic
transformation , making it
more aggressive.
• PROGNOSIS- POOR
BASAL CELL CARCINOMA / RODENT
ULCER OF VULVA
• 2% of all vulval cancers
in elderly women.
• Site- labis majora
• Features- rolled out
edges with central
ulcer + pain +pruritus +
bleeding
• Rarely metastatic
• Biopsy - confirmatory
TREATMENT-
• Radical partial
vulvectomy with at least
1cm surgical margin
• If lymph node
involvement- inguino-
femoral
lymphadenectomy
BARTHOLIN GLAND CARCINOMA
• RARE tumour
HISTOLOGICAL TYPES
 ADENOCARCINOMA
 Squamous cell
carcinoma
 Transitional cell
carcinoma
• Age- 60-65 yrs
Clinical features-
• RECURRENT
BARTHOLIN’S ABSCESS
• Dyspareunia
SOLID TUMOUR OF BARTHOLIN
GLAND
 FNAC
 SPREAD- solid tumours
infiltrating into vulva and
ischiorectal fossa.
 Lymphatic spread- to pelvic LN
 Haematogenous spread- seen
TREATMENT
• Early stage- radical partial
vulvectomy with inguino-
femoral lymphadenectomy
• If tumour is pressing RECTUM-
preOP chemotherapy + radical
partial vulvectomy + ipsilateral/
B/L I.F lymphadenectomy.
VULVAL SARCOMA
• RARE
TYPES-
 Leiomyosarcoma
(commonest)
 Fibrous histiocytoma
• Develops as isolated
lesion on vulva
• Prognosis- poor
• TREATMENT-
Radical vulvectomy +
inguinofemoral
lymphadenectomy
Adjuvany chemotherapy
and chemotherapy given
for large tumours.
MANAGEMENT DURING PREGNANCY
• Squamous cell cancer of the vulva is rarely diagnosed and
surgically treated during pregnancy
• Incidence - 1 per 20,000
• Radical complete or partial vulvectomy and inguino femoral
lymphadenectomy can be performed if needed in 1st and
2nd trimester.
• During the 3ed trimester, there is increased genital
vasculature can increase surgical morbidity. Only local
excision is done
• Final surgery is done 2-3 weeks after delivery.
• If diagnosed at delivery, definitive surgery is performed
typically 2 to 3 weeks postpartum.
• MODE OF DELIVERY- VAGINAL DELIVERY.
• C.S is only done for obs. Indication or if tumour size is big
enough to cause hindrance in deliver
THANK YOU

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Carcinoma VULVA

  • 1. PRE-MALIGNANT AND MALIGNANT CONDITIONS OF VULVA Dr. Ujjwal Khullar Dr. Prachi Kushwaha(GUIDE)
  • 2. • VIN (vulvar intraepithelial neoplasia) is the premalignant condition of the vulva where the cellular changes are limited to the epithelium but do not extend beyond the basement membrane. • Also known as the squamous intraepithelial lesions of the vulva VIN
  • 3. GRADING • VIN 1 – atypical cells are found only in deeper 1/3 of epithelium • VIN 2 –intermediate state b/w 1 & 3 • VIN 3 – atypical epithelium is present throughout the whole depth of epithelium , k/a CARCINOMA in SITU.  Basement membrane is intact in all.
  • 4. • The vulva is the area immediately external to the vagina, includes- the mons pubis, labia, clitoris, Bartholin glands, perineum.
  • 5.  Anatomically, the vulva extends from- • Anteriorly- Mons pubis • Laterally- Genito- crural folds • Posteriorly- Anus The normal findings in the vulva include- • Micropapillomatosis, • Sebaceous glands and • Vestibular redness.  It is important to be aware of normal findings and not mistake them for vulvar lesions.
  • 7. CLASSIFICATION • NON-NEOPLASTIC EPITHELIAL DISORDER OF SKIN AND MUCOSA - Lichen sclerosis - Squamous hyperplasia - Other dermatosis • MIXED NON-NEOPLASTIC AND NEOPLASTIC EPITHELIAL DISORDERS • INTRAEPITHELIAL NEOPLASIA -squamous intraepithelial neoplasia  VIN 1  VIN 2  VIN 3 -non squamous intraepithelial neoplasia paget`s disease tumours of melanocytes, noninvasive
  • 8. • High-grade squamous intraepithelial lesion (HSIL) has replaced the term ‘VIN usual type’ and includes the warty, basaloid and mixed lesions. • It is associated with high-risk HPV infection, by HPV 16, 18 and 33. • Other risk factors are smoking, HIV infection and STDs. • VIN usually associated with vulvar lichen sclerosus and not associated with HPV and occurring in older women.
  • 9. PATHOLOGY • Macroscopically- the lesion may be a warty, elevated plaque that is white or red, an ulcer or a nodular lesion • The lesions may be multifocal. • Differentiated VIN is usually associated with lichen sclerosis and squamous cell hyperplasia.
  • 10. Microscopically ,the cells exhibit - • Nuclear abnormalities • Maturation disturbance • Increase in mitotic activity • Sometimes changes of HPV infection.
  • 11. DIAGNOSIS Symptoms of VIN- • Asymptomatic • Pruritus vulva • Ulcer • Discomfort, pain • Warty growth • Bleeding, discharge
  • 12. EXAMINATION • VULVOSCOPY- with 3- 5% acetic acid is helpful. • Acetic acid enhances – surface topography and acetowhite areas. • Helps to see – coarse punctations • LESSIONS SEEN ARE- 1. White hyperkeratotic plaques 2. Hyper pigmented lesions 3. Areas of erythema 4. Raised / flat lesions 5. Bulky lesions 6. Condylomatous lesions 7. Multifocal lesions
  • 13. In cases of DIFFERENTIATED VIN (Dvin)- • Lesion is single with LICHEN SCLEROSES / LICHEN SIMPLEX near vulval skin. • Lessions are- - Ulcer - Warty papule - Hyperkeratotic plaque
  • 14. BIOPSY • Which lessions to be biopsied? - Irregular - Asymmetric - Dark - Large - Elevated - Rough - Nodular - Ulcerated lession with induration - Inguinal lymphadenopathy • KEYES PUNCH BIOPSY- - Apply local anesthetic injection , apply acetic acid. - Aceto-white area is biopsied. - Alternatively biopsy can be taken by cervical punch biopsy forceps / knife - About 6mm diameter suspicious area is taken and one or two haemostatic sutures are taken with vicryl- 2-0. - Lesions of CLITORIS – to be done under GA.
  • 15. MEDICAL TREATMENT FOR VIN • Topical steroid therapy (TST) • Topical 5-fluorouracil (TFT) • Alfa interferon (under trial) • Even with medical treatment routine biopsy is essential. TOPICAL STEROID THERAPY (TST)  CLOBETASOL PROPROIONATE cream  FLUOCINOLONE oint.  HALCINONIDE cream.  5-FLUOROURACIL cream DOSE- TDS for 3-4 weeks. Above is not definative treatment.
  • 16. MANAGEMENT OF VIN - • Local excision is done for vulvar HSIL and VIN differentiated. • Disease-free margin of 5 mm should be obtained. • Large, confluent lesions require more extensive excision and rotational flap for skin grafting. • Young women with VIN may be treated by CO2 laser. • Immune response modulators such as imiquimod are under trial.
  • 17. TREATMENT OF HIGH GRADE SIL OF VULVA / VIN 2 AND 3 • High grade VIN is treated. • VIN on skin surface tend to be deeper involving pilosebaceous units in 66% cases and is limited to 2.5mm depth from epidermal surface • Mucosal surface lessions are more superficial. • Extensive excision is needed.
  • 18. WIDE LOCAL EXCISION • Treatment of choice- large advanced VIN in which probability of vulvar cancer can`t be excluded. • A surgical margin of at least 5 mm is prefered • Recurrence rate – 20% LASER ABLATION • CO2 gives good cosmetic results and depth of tissue distruction can be controlled • ADVANTAGES- - It is less disfiguring as compared to excision - Less bleeding • DISADVANTAGES - Prolonged painful healing - No tissue specimen - More reurrence – 25%
  • 19. CAVITATIONAL ULTRASONIC SURGICAL ASPIRATION (CUSA) - Done on non- hairy skin - Ultrasound is used to cause cavitation and disruption of affected tissue. - Less pain and scaring - Tissue obtained is in fragments - Recurrence -35% TOPICAL MEDICAL THERAPY - Topical Imiquimod cream applied over the affected area has lower toxicity than 5- Fluorouracil and Cidofovir. - Efficacy- 77% cure rate - Recurrence- 20% - PREVENTION- HPV vaccine. - FOLLOW UP- after 6 months and then yearly.
  • 20. BOWEN’S DISEASE • It’s intraepithelial carcinoma of Vulva. • CLINICAL FEATURES- slowly growing hard red indurated patch on vulva with intense itching. • EXAMINATION- - Well demarcated reddish indurated lesion on vulva with eczematous dry surface. - BIOPSY- pickle cells / giant cells (BOWEN CELLS) - TREATMENT- vulvectomy - Prognosis- good - Metastasis- rare
  • 21. PAGET’S DISEASE • Extra mammary disease on vulva simulating intraductal breast carcinoma with involvement of apocrine sweat glands • May have associated ADENOCARCINOMA in apocrine and Bartholin gland • May involve perianal and anal skin. • CLINICAL FEATURES- - Pruritis - Vulvar discomfort - Pain
  • 22. EXAMINATION- • Elevated white indurated eczematous lesions. • Vulval biopsy- typical paget cells in the epidermis • These cells are large pale vaculated rounded cells with pale cytoplasm and vesicular nuclei. • Secreats- mucous • May be asso. With adenocarcinoma of Bartholin glands in 20% cases.
  • 23. TREATMENT- - Simple vulvectomy - Treat like vulval cancer – radical vulvectomy with inguino- femoral lymphadenectomy. - Radiaotherapy for elderly unfit women. - Topical BLEOMYCIN or 5- fluorouracil - Recurrence- 20-25%
  • 24. CARCINOMA VULVA • Vulvar cancer accounts for about 4% of cancers of the female genital system in India. • Most common age group - >65-70 yrs • Median age 68 yrs. • Most common variety of vulval cancer – SQUAMOUS CELL (90-92%) followed by melanoma (2-4%). • Most common SITE for vulval carcinoma- HARTS LINE f/b labia minora and L. majora.
  • 26. Possible signs of vulvar cancer include bleeding or itching. • -A lump or growth on the vulva. • Changes in the vulvar skin, such as color changes or growths that look like a wart or ulcer. • Itching in the vulvar area, that does not go away. • Bleeding and Tenderness in the vulvar area.
  • 28. Most Common Histological types • Squamous: >90% • Melanoma: 5-10% • Basal Cell: 2% • Sarcoma: 1-2% • Paget: <1% • Bartholin gland: rare
  • 29. TYPES OF SQUAMOUS CELL CARCINOMA TYPE 1 WARTY / BASALOID TYPE- • Young women <50 yrs • Multifocal • Related to HPV infection , vulval intraepithelial neoplasia , smoking , STD , immunosuppresion. • PROGNOSIS- good TYPE 2 KERATINIZING , DIFFERENTIATED / SIMPLEX TYPE • Elderly • Unifocal • Related to vulval ds-= like- lichen sclerosis , squamous hyperplasia • GENE mutation responsible – p53
  • 30. ETIOLOGY • Older age • Precancerous changes (dysplasia) in vulvar tissues • Lichen sclerosus, which causes persistent • itching and scarring of the vulva • Human papillomavirus (HPV) infection • Cancer of the vagina or cervix • Heavy cigarette smoking • Chronic granulomatous disease (a hereditary disease that impairs the immune system)
  • 31. HUMAN PAPILLOMA VIRUS INFECTION (HPV)- HPV mainly 16 and 18 , 31 , 33 are responsible • HPV DNA is detected in 90% cases of VIN and only 50-70% cases of vulval carcinoma ,i.e., only basaloid and warty types • RISK FACTORS- smoking and HPV genital warts • HPV VACCINE reduces incidence of vulval carcinoma.
  • 32. • HERPES SIMPLEX VIRUS- is weakly associated , along with co-factors like smoking. • CHRONIC IMMUNOSUPPRESSION- transplant patients on immunosuppressive drugs , HIV women especially in younger age - Hence vulval biopsy must be done for any suspicious lesions specially in immunocompromised patients. • VIN-2 and VIN 3 can develop into carcinoma with in 4 years
  • 33. CLINICAL PRESENTATION SYMPTOMS- • Asymptomatic • Vulvar pruritis of long duration ( commonest symptom) • Vulvar irritation • Vulvar pain • Vulvar mass (Warty growth) • Non healing vulvar ulcer • Vulvar bleeding • Vulvar discharge • Dysuria and difficult micturition (involvement of urethra) • Rectal bleeding and painful defecation (involvement of anal canal) • Inguinal mass ( metastatic lymph nodes)
  • 34. SIGNS Vulvar examination may reveal- • Irregular fungating mass • Irregular ulcer • Warty growth • Plaque like lesion • Red or white pigmentation on vulvar lesion • Tenderness over lesion (may or may not be present) • Unilateral or bilateral inguino-femoral lymphadenopathy Parts involved- Labia Majora or minora (60%), Clitoris ( 10%), Perineum ( 10%)
  • 36. HISTORY • Careful history taking for symptoms and their duration. • Past history of sexually transmitted disease , any condylomata or vulval disease (lichen sclerosis) • HISTORY- smoking and immunosuppressive drugs.
  • 37. EXAMINATION • Careful examination of vulva should be done , look for –  Size  Location  Extent of lesion  Warty growth or  Ulcerative growth  Single / multifocal  Association with lichen sclerosis  Involvement of vagina Urethra Base of bladder / anus
  • 38. • PER SPECULAM EXAMINATION- to look for extension to vagina and cervix • Per vaginal and per rectal examination is also important. • Any fixity of vulval lession to bone is looked. • Inguinal regions should be palpated for inguinofemoral lymph nodes , their enlargement ,size , number and fixity to skin.
  • 39. DIAGNOSIS • If the lesion is not visible we can go for VULVOSCOPY with 3% Acetic acid to look for aceto- white areas. • DEFINATIVE DIAGNOSIS- BIOPSY
  • 40. TYPES OF BIOPSY • Keyes punch biopsy :- – Done under local anesthesia.Skin over lesion is made taut with left hand. – The keyes punch is put against the lesion firmly and rotated with a constant firm pressure clockwise and then counter clockwise for penetration into skin and to reach subcutaneous fat. – The raised circular tissue is grasped and cut at base with scissors . • Wedge biopsy :- – Taken with knife. – 2-0 chromic catgut or vicryl suture is applied for hemostasis.
  • 41. INVESTIGATIONS • PAP smear for cervial and vaginal cytology. • Colposcopy of cervix and vagina. • Vulvoscopy for inspection of other lesions. • Imaging modalities:- – Ultrasonography pelvis – CT or MRI to see extent of disease and metastasis. – Cytourethroscopy to rule out involvement of urethra and bladder. – Proctosigmoidoscopy. – Intravenous urogram. • Lymphography to detect smaller lymph node metastasis.
  • 42. Routine preoperative workup: PAP smear Serology Complete hemogram Blood gp (rh typing) Urine routine and microscopy Urine culture LFT KFT BT/CT X-ray chest ECG
  • 44. • Tumor shows classic diagnostic features of invasive squamous cell carcinoma that include- • a squamoid appearance • intercellular bridges • brightly eosinophilic keratin pearls • Nests of invasive tumor are surrounded by chronic inflammation.
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  • 48. MODE OF SPREAD • Direct extension :- To adjacent structures (vagina, urethra , anus , clitoris ) • Lymphatic spread :- It is common and occurs by embolization to regional lymph nodes. Superficial inguinal lymph nodes Deep inguinal and femoral nodes Pelvic lymph nodes.
  • 49. INITIAL SPREAD- INGUINAL L.N (b/w Camper`s fascia ant. fascia lata) CLOQUET’S L.N / ROSENMULLER NODES (SENTINAL LYMPH NODE of carcinoma vulva) These are deep femoral nodes situated beneath round ligament medial to femoral vein. • Hematogenous spread : - Occurs rarely in the late stages.
  • 50. NORMAL LYMPHATIC DRAINAGE • From VULVA+ Dist.3ed of VAGINA. • Drains into superficial inguinal nodes. • Lymph travels through DEEP FEMORAL Lymphatics and node of CLONQUET to pelvic L.N • Lymph can directly also drain into DEEP FEMORAL nodes from CLITORIS and UPPER LABIA All lessions within 2cm of midline may spread via lymphatics.
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  • 52. DIFFERENTIAL DIAGNOSIS • Syphilitic ulcer • Tubercular ulcer • Lymphogranuloma venereum • Granuloma inguinal • Chancroid • Vulvar elephantiasis • Lichen sclerosis
  • 53. TREATMENT • SURGICAL TREATMENT :- – Main treatment modality. – Need to have adequate resection margin of 1 cm and groin node dissection.
  • 54. WIDE LOCAL EXCISION/ SIMPLE PARTIAL VULVECTOMY • For micro-invasive tumor (stage IA). • 2cm tumour free surgical margin is to obtained around the lesion • Deep surgical margin should also be 1 cm which roughly corresponds to colles fascia. • Lymph nodes are not removed.
  • 55. RADICAL PARTIAL VULVECTOMY • Partial vulvectomy is typically reserved for unifocal lesions that are clinically connected to the labia majora, labia minora , mons , vestibule, and/or perineum and that have limited involvement to the adjacent urethra, vagina, and/or anus. • Complete removal of tumor containing portion of the vulva wherever it is situated with 1 cm skin margins and excision to the pelvic membrane. • Combined with ipsilateral inguinofemoral lymphadenectomy .
  • 56. RADICAL PARTIAL VULVECTOMY Its VARIATIONS are- 1. Right / left vulvectomy – one side of L. majora and minora. 2. Ant. Hemivulvectomy- clitoris+ partial resection of L. minora+ majora and mons pubis 3. Post. Hemivulvectomy- portion of L.majora , bartholin gland and upper perineal body.
  • 57. RADICAL TOTAL VULVECTOMY • Removal of entire VULVA till Perineal membrane + pubic symphysis. • Two elliptical incisions are made on either sides of vulva. • INNER INCISION- On vaginal introitus and vestibule ant. To urethral meatus post. to clitoris. • OUTER INCISION- along Labiocrural folds and Ant.across mons pubis and post. Across the perineal body.
  • 58. • the en bloc incision, also termed the butterfly or longhorn incision, made from one ASIS to other. • Its abandoned. • Survival rates equivalent to radical complete vulvectomy but carries signicantly greater morbidity. • After complete dissection , levator ani muscle are approximated to prevent RECTOCELE. DISADVANTAGES-  Complete loss of vulval tissue with psychosexual complications.  Wound break down in 50%  High rate of lymphedema (65%) NOT USED NOW A DAYS.
  • 59. SENTINAL LYMPH NODE BIOPSY • The FIRST LYMPH NODE to receive tumour lymphatic drainage is termed as SENTINAL L.N • If on biopsy sentinal node is NEGATIVE , whole chain of lymph nodes above it is also likely to be NEGATIVE. • NOT DONE – if groin metastasis is suspected • Done with LYMPHOSCINTIGRAPHY with Tc99m labelled nano colloid / isosulphan blue injected into skin around tumour. • 5 mins late if TRACES of dye +nt , excision under microscope INDICATIONS- • Unifocal primary vulval cancer • <4cm diameter • Tumour invasion <1mm • Absence of obvious metastasis on clinical and radiology • Absence of suspicious inguino- femoral L.N
  • 60. MANAGEMENT OF LYMPH NODES  L.N DISECTION- INGUINO-FEMORAL L.N DISECTION If carcinoma is within 2cm of midline BILATERAL INGUINO- FEMORAL LN DISECTION If cancer is >2cm away from midline UNILATERAL INGUINO- FEMORAL L.N DISECTION
  • 61. APPROACH TO LYMPH NODE DISECTION
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  • 66. 5 YEAR SURVIVAL RATE • SINGLE most important factor for PROGNOSIS is INVOLVEMENT OF LYMPH NODES. L.N INVOLVED 5 YRS survival rate – 85% L.N NOT involved 5 yrs survival rate -50% DEPENDING ON DEPTH OF INVASION- 1. <1MM – L.N not involved , no disection needed PROGNOSIS- GOOD 2. >1MM – need disection PROGNOSIS- POOR
  • 67. LYMPH NODE METASTASIS • OVER ALL L.N metastasis- 25% cases of vulval cancer. STAGE LYMPH NODE METASIS 1 10% 2 30% 3 75% 4 98-100%
  • 68. ROLE OF RADIOTHERAPY INDICATION- • PREOPERATIVELY in pt with advanced disease with fixed L.N • POSTOP- pts with positive inguinal L.N • POSTOP- pts with close surgical margins of <5mm , to prevent recurrence. • In young patients , to prevent severe psychological effects of radical surgery • Recurrent disease • Women unfit for surgery / anaesthesia. TREATMENT- usually teletherapy of 45-55GY.
  • 69. ROLE OF CHEMOTHERAPY • Limited role • Mostly used as neoadjuvant therapy with radiotherapy in advanced cases. • Done where surgery is not possible. DRUGS USED ARE-  CRISPLATIN  5-FLUROURACIL  BLEOMYCIN  MITOMYCIN C
  • 70. 5 YEAR SURVIVAL RATE • STAGE 1 & 2 - 75-90% • STAGE 3 – 54% • STAGE 4B- 10% • If INGUINAL lymph nodes are involved – 50% FOLLOW-UP 6 monthly follow up after treatment is completed and wound is healed till 5 yrs • AIM- to detect recurrence , new primary lymph node • Patient should be looked for Ca vagina and cervix.
  • 71. RECURRENCE • About 30% vulval cancer recur. • 50% recurrence- if margin is <8mm. VULVA 70% GROIN 24% PELVIS 15% DISTANT ORGAN 18% MANAGEMENT OF RECURRENCE- VULVA- radiotherapy +/- wide local excision GROIN- radiotherapy/ radical groin dissection. PELVIS- palliative radiotherapy with chemotherapy DISTANT ORGAN- palliative chemo. , poor- prognosis. (<1 yr)
  • 72. VULVAL MELANOMA • 2nd MOST COMMON vulval cancer (2-5%) • Elderly • 5 yrs survival – 10-50% • Arises from L. majora , minora and clitoris. • Hyperpigmented nodule on vulva • DIAGNOSIS- biopsy TREATMENT- • Radical Partial vulvectomy with inguino-femoral lymphadenectomy. • Prognosis- poor • Chemotherapy – alpha interferon, interleukin-2 (new).
  • 73. VERRUCOUS CARCINOMA OF VULVA • Rare type of Squamous cell Ca. of vulva • Incidence- <1% • Associations has been seen with HPV genome • Clinical features- vulval mass , pain and pluritis • On examination – cauliflower like tender growth. TREATMENT- • Wide local excision with 1cm surgical margin • Usually no lymph node involvement • If needed FNAC of inflammed/ suspected L.N can be sent • RADIOTHERAPY is CONTRAINDICATED as it may stimulate anaplastic transformation , making it more aggressive. • PROGNOSIS- POOR
  • 74. BASAL CELL CARCINOMA / RODENT ULCER OF VULVA • 2% of all vulval cancers in elderly women. • Site- labis majora • Features- rolled out edges with central ulcer + pain +pruritus + bleeding • Rarely metastatic • Biopsy - confirmatory TREATMENT- • Radical partial vulvectomy with at least 1cm surgical margin • If lymph node involvement- inguino- femoral lymphadenectomy
  • 75. BARTHOLIN GLAND CARCINOMA • RARE tumour HISTOLOGICAL TYPES  ADENOCARCINOMA  Squamous cell carcinoma  Transitional cell carcinoma • Age- 60-65 yrs Clinical features- • RECURRENT BARTHOLIN’S ABSCESS • Dyspareunia SOLID TUMOUR OF BARTHOLIN GLAND  FNAC  SPREAD- solid tumours infiltrating into vulva and ischiorectal fossa.  Lymphatic spread- to pelvic LN  Haematogenous spread- seen TREATMENT • Early stage- radical partial vulvectomy with inguino- femoral lymphadenectomy • If tumour is pressing RECTUM- preOP chemotherapy + radical partial vulvectomy + ipsilateral/ B/L I.F lymphadenectomy.
  • 76. VULVAL SARCOMA • RARE TYPES-  Leiomyosarcoma (commonest)  Fibrous histiocytoma • Develops as isolated lesion on vulva • Prognosis- poor • TREATMENT- Radical vulvectomy + inguinofemoral lymphadenectomy Adjuvany chemotherapy and chemotherapy given for large tumours.
  • 77. MANAGEMENT DURING PREGNANCY • Squamous cell cancer of the vulva is rarely diagnosed and surgically treated during pregnancy • Incidence - 1 per 20,000 • Radical complete or partial vulvectomy and inguino femoral lymphadenectomy can be performed if needed in 1st and 2nd trimester. • During the 3ed trimester, there is increased genital vasculature can increase surgical morbidity. Only local excision is done • Final surgery is done 2-3 weeks after delivery. • If diagnosed at delivery, definitive surgery is performed typically 2 to 3 weeks postpartum. • MODE OF DELIVERY- VAGINAL DELIVERY. • C.S is only done for obs. Indication or if tumour size is big enough to cause hindrance in deliver