By senior resident ,obgy,AIIMS RAIPUR

1. PRESENTED BY:
DR.SAGARIKA MAJUMDAR
SENIOR RESIDENT
AIIMS RAIPUR

2. VULVAL ANATOMY

3. ANATOMICAL CONSIDERATION
 Vulva includes mons veneris,
labia majora, labia minora,
clitoris, vestibule and
conventionally the perineum
 Bounded anteriorly by the
mons veneris, laterally by
the labia majora and
posteriorly by the perineum

4. BLOOD SUPPLY OF VULVA
 ARTERIAL SUPPLY
Branches of internal pudendal artery
Branches of femoral artery
 VEINOUS SUPPLY
Internal pudendal vein
Vesical or vaginal venous plexus, and
Long saphenous vein

5. VULVAL ANATOMY: INNERVATION
 Anterosuperior part is supplied by
the cutaneous branches from the
ilioinguinal and genital branch of
genitofemoral nerve (L1 and L2)
 Posteroinferior part by the
pudendal branches from the
posterior cutaneous nerve of thigh
(S2,3,4)

7. BENIGN DISORDERS OF THE
VULVA:

8. BENIGN VULVAL LUMPS

9. BARTHOLIN’S CYST
Bartholin’s glands are the two pea sized (2 cm)
glands, located in the groove between the hymen and
the labia minora at 5 O’Clock and 7 O’Clock position

10. Swollen inflamed Vulva: Underlying
Bartholin’s abscess with inflammation of
the Vulva
•Painful swollen gland
•Fluctuant tender
•May have expressible
purulent discharge
•Located at the 5 and 7
o’clock position of the
vestibule.
•2% have adenocarcinoma

11. Treatment Bartholin’s Cyst or
Abscess
 Consider antibiotics if extensive inflammation vulval tissues.
 If small may settle, alternately for persistent cysts and or larger
cysts/absecess 2 options.-
1. First consider insertion of Word Catheter in OPD and prescribe
antibiotics. Allow free drainage remove catheter when settled
.Send swab for C&S
2. Marsupialization of Bartholin’s abscess or cyst alternative option
 In Recurrent abscess or cyst consider excision Bartholin’s cyst/gland
 In Older females tissue for histology sent as 2% risk adenocarcinoma
 Antibiotics: Chlamydia cover with Doxycycline 100mg BD for 10 days
and broad spectrum cover with a Cephalopsporin 500mg TDS for 5-7
days or Metronidazole 400mg TDS for 5-7 days

12. SEBACEOUS CYST/EPIDERMAL INCLUSION CYST —
 Common vulvar cyst
 Location-anterior half of labia majora
 multiple
 formed by accumulation of the sebaceous material due to occlusion of the ducts.
 If infected, treatment is done by antibiotics and surgical drainage/excision
CYST OF CANAL OF NUCK
 Part of the processus vaginalis, which accompanied the round ligament and got
obliterated prior to birth may persist to form a cyst.
 occupies the anterior part of the labium majus.
INGUINOLABIAL HERNIA
 When the entire processus vaginalis remains patent-herniation of the abdominal
contents along the tract.
 The hernia may be limited to the inguinal canal or may extend up to the anterior part
of the labium majus.
 The contents of the sac - intestine or omentum. The swelling is reducible and impulse on
straining can be elicited. One should be conscious of the entity as casual surgical incision
on labial swelling may cause inadvertent injury to the gut.

13. VULVAL VARICOSITIES
 predominantly seen during pregnancy and subside following
delivery.
 Intolerable aching on standing.
 Support pads and tights or T-bandage may be tried, If these
fails, treatment by injecting sclerosing fluids or high ligature of
the long saphenous vein may be of help.
ELEPHANTIASIS VULVAE
 It is mainly due to consequence of lymphatic obstruction by
microfilaria (filariasis).
 Plastic surgery may be tried to restore the normal anatomy
along with antifilarial treatment.

14. BENIGN TUMORS OF VULVA
FIBROMA,LIPOMA
,NEUROFIBROMA
 Fibroma is the most common
benign solid tumor of the vulva
Vulval fibroma grow slowly.
 malignant change is very low.
 Surgical removal is necessary
as they produce discomfort
HYDRAADENOMA
 arises from the sweat gland
in the vulva, usually located
in the anterior part of the
labia majora (38%).
 benign lesion but its reddish
look and complex
adenomatous pattern on
histology ,may be confused
with adenocarcinoma.
 Simple excision and biopsy is
adequate.

15. VULVAL EPITHELIAL DISORDERS

16. WHITE LESIONS OF VULVA RED LESIONS OF VULVA
 The melanocytes loose their
ability to manufacture
melanin resulting in
depigmentation and
subsequent
white lesions
Lichen sclerosus
Squamous cell hyperplasia
Vitiligo
Albinism—enzyme deficiency
preventing normal formation
of melanin
Leukoderma
Intertrigo.
 Due to infection
 Includes-
 Candidiasis
 Allergic dermatitis
 Melanosis

17. Non- Neoplastic Epithelial Disorders
 CLASSIFICATION OF VULVAL DERMATOSES (ISSVD-2006)

18. ETIOLOGY –
 Traumatic (scratching)
 autoimmune allergic (atopic)
 nutritional (deficiency of folic acid, vitamin B12,riboflavine
achlorhydria, etc.)
 infection (fungus)
 metabolic or systemic (hepatic, hematological)
 Autoimmune disorders like thyroid disease, pernicious
anemia . (asthma, eczema, hay fever)
 Drugs:β-blockers, ACE inhibitors
 Common allergens are: cosmetics, synthetic underwears
and fragrances.

19. Vulval assessment:
 DETAILED HISTORY;
 -Itching or pruritus
 -Pain/ soreness (character, severity, radiation, relieving/exacerbating factors)
 -Erythema (redness), Localised lesions, Any discharge
 -Onset and progression of any pain tenderness or itching
 -Sexual history including any previous STIs, sexual pain
 -Hygiene/cleansing routine (check specific products ?allergy)
 -Menopausal status
 -Contraception including use of condoms or lubricants (impact on pain or itching)
 -Cervical screening history and vaccination
 -Medical, psychological (anxiety/depression), surgical and social history (e.g.
cigarettes)
 -Psychosexual symptoms (e.g. vaginismus, loss of libido)
 -Any lesions elsewhere on the body or history dermatoses (e.g. dermatitis, psoriasis)
 -Any history atopy, chest infection, sore throat etc
 -Current or recent medications

20. Vulval examination
 Detailed vulvovaginal and general examination;
 Inspect external/internal vagina then exam including bi-manual
 Qualify the rash lesion(s); (erythema, petechiae, ulcer, type lesion, colour)
 Tender non tender
 Assess the quality of the surrounding skin
 Hygiene
 Identify any vaginal discharge
 Cusco speculum exam; visualise cervix and vaginal walls (document tenderness)
 Consider microscopy, wet mount (LM assess) and/or HVS and/or PID screen
 Palpation vulva using swab; any pain tenderness, character, radiation
 Check the anus and peri-anal skin
 Consider vulvoscopy
 General medical exam of the body skin remembering to look at the scalp and behind
the ears. Oral exam using tongue depressor identify and oral mucosal /gingival
lesions and tongue. Check elbows and behind the knees.

21. PROCEDURE-VULVAL BIOPSY
 INDICATION -Biopsy is required if the woman
fails to respond to treatment or there is clinical
suspicion of VIN or cancer
 The sites for biopsy are from the margins of
cracks and fissures and the sky blue areas left
behind after applying 1 percent aqueous toluidine
blue to the vulva and washing it off after 1 minute
with 1 percent acetic acid.

22. Vulval biopsy procedure:
 Keyes Biopsy
 Different sizes
 LA cover
 Only every biopsy the edge of
a lesion
 Always take a clinical photo
were possible
 Review histology and adjust
treatment as required
 Refer to specialist
dermatologist if unclear
 Discuss histology at MDT if
fails to respond to therapy

23. Review of Benign Vulval Skin
Disorders

24. Lichen Sclerosus
 Aetiology: All 1 in 300 females of all ages and 1 in 30 elderly women. Ratio F:M is 5:1
 Location - The entire vulva is involved. Lesion encircles the vestibule. It is usually bilateral and
symmetrical .It does not involve the vestibule or extend into the vagina or anal canal
 Presentation: White patch (leukoplakia) which coalesce leading to a paper-thin-like skin, stiff
labia with a constricted vaginal orifice and loss of architecture,Figure of ‘8’ appearance
 Pathogenesis: Unknown (autoimmune?), autoantibodies to ECM protein.
 Histology: Benign thin, flat squamous epidermal cells with fibrosis, degeneration of basal
epidermal cells . Hallmark at histology: Band of Fibrosis.
 Symptoms: None (rare), itching (worse at night), sore, pain, dyspareunia (vulval stenosis),
constipation -peri-anal involvement.
 Differential skin lesions: Can co-exist with LP and must exclude neoplastic lesions or VIN.(risk of
malignancy =1-4%

25. Lichen sclerosus

26. Management Lichen Sclerosus:
INIVESTIGATIONS;
1. Biopsy: mandatory if diagnosis
uncertain, atypical features
coexistent VIN or SCC is suspected
2. Investigation autoimmune disease:
If clinically indicated (e.g. T4 TSH)
is often asymptomatic
3. Skin swab:exclude co-existing
infection
4. Consider referral to dermatologist
or Vulval Clinic if chronic,
complicated or diagnosis unclear.
;
TREATMENT :
1. Recommended regimen: Ultra-potent
topical steroids (e.g. Clobetasol
proprionate (level evidence I A).
Various regimens are used. The most
common is daily use for 1-month,
alternate days 1-month, x2 weekly
for 1-month , review at three
months. Then use as required
2. Alternative regimens: Ultra-potent
topical steroid with antibacterial
/antifungal(Clobetasol with
neomycin/nystatin), alternative
preparation that combats secondary
infection (e.g. Fucibet cream)

27. Refractory LS: New and novel
therapies
•Topical calcineurin inhibitors (e.g topical tacrolimus 0.1%)
•Avocado and soya beans extracts
•Methyl aminolevulinate photodynamic therapy (MAL-PDT)
•Cyclopsporin
•Methotrexate (dosing e.g. Methylprednisolone 1g for 3 days
then 15mg once weekly for 6 months)
•Retinoids (e.g. elretinate)
•Surgical excision,CO2 laser vaporisation
 Cryotherapy and laser ablation –rarely needed

28. LICHEN PLANUS

30. LICHEN PLANUS

31. Lichen Simplex Chronicus (squamous
hyperplasia)
 Presentation: White patches (leukoplakia)
with thick leathery vulval skin.
 Symptoms:itching (intractable pruritus –
night )
 Pathogenesis: Benign hyperplasia of the
vulvar epithelium secondary to rubbing
and scratching due to pruritus.
 Histology: Hyperkeratosis, no nuclear
atypia.
 Hallmark at histology: hyperkeratosis
 Differential skin lesions: Can co-exist must
exclude neoplastic lesions or VIN.
 Biopsy: If fails to respond to therapy or
suspicious lesions.
 The initial stimulus itch may be due to;
-Underlying seborrrhoeic dermatitis
-Intertrigo
-Tinea
-Psoriasis
 Any itching disease of the vulva may
become secondarily lichenified
 Psychological factors may play a role -some
specialists may use the term
neurodermatitis
 Main stay of treatment is to avoid any
irritants, antihistaminics,ultrapotent topical
steroids (clobetasol) is helpful to break the
itch-scratch cycle.

32. Vulval Psoriasis
 Psoriasis is a chronic, inflammatory
epidermal skin disease
 It is the 3rd most common
dermatoses of genital skin where it
may affect the pubic area, vulva, skin
folds and buttocks.
 The appearance of vulvar psoriasis is
often symmetrical and can vary from
silvery, scaling patches adjacent to
the outer parts of the labia majora to
moist greyish plaques or glossy red
plaques without scaling in the skin
folds.
 Most genital psoriatic lesions
represent plaque-type
 The diagnosis of psoriasis is based on
appearance
 Treatment: Topical preparations
and UV light are most often used.
- Moisturize
- Emollients can cover and
protect the skin
- Low strength topical steroids
-Topical Vitamin D preparations
- Coal tar preparations
- UV light (risk burns with excess)
 Refractory psoriasis may require
systemic medications: Calcineurin
inhibitors: Tacrolimus and
Pimecrolimus
 Topical cyclosporin may also be
considered 3rd line.

33. Vulvo-Vaginal Candidiasis (VVC)
 Differential diagnoses includes: BV,
dermatitis, allergic reactions, HS
infection and lichen sclerosis
 Investigations: empirical treatment
can be considered based on the
history. Vaginal vulval swabs should be
taken if symptoms are persistent or
recurrent
 TREATMENT-
-TOPICAL -clotrimazole ,fenticonazole
,miconazle –cream ,pessary
intravaginal cream
-ORAL –Fluconazole 150mg single
dose and itraconazole 200 mg twice
daily x 1 day

34. Irritant Contact Dermatitis (ICD)
 Irritant Contact Dermatitis (ICD) is
a common problem and vulvar
irritation is a frequent complaint
among women
 It occurs after exposure/contact
with exogenous irritants
 Unlike ACD, irritant (ICD) reactions
are not typically vesicular or
bullous
 There is often sparing of the
inguinal creases where the
offending agent is not as capable
of contacting the skin

 ETIOLOGY-Local irritants as
perfumed soap ,deodorant
,bubble bath ,tight cothin,urie
,faeces
 Sodium lauryl sulfate (SLS) is an
anionic detergent and surfactant
used as a foaming agent in many
soaps and shampoos –been
implicated in induction of
dermatitis
 Differential diagnosis-vulvar
candidiasis
 TREATMENT
-Avoid local cause
-Oral antihistamincs
-Topical steroids

35. Allergic Contact Dermatitis (ACD):
 ACD a type IV delayed hypersensitivity reaction
 Itching is a prominent feature of ACD, which can be somewhat
helpful in distinguishing from ICD, in which pain is often a
primary
 Characteristic appearance of ACD - erythema, oedema, and
possible vesicles or bullae with weeping
 contact irritants predisposing to ACD-some antibiotics
,antifungals,all corticosteroids ,nail polish ,body fluids
 Because topical corticosteroids are commonly used to treat a
variety of vulvar dermatoses, it’s important to keep in mind
their potential for inducing comorbid ACD

36. VULVAL ULCERS

37. Extra-mammary Pagets (risk of
adenocarcinoma)
 Age-seen in postmenopausal women
 Presentation: florid eczematous with erythema and
excoriation
 Symptoms: None, itching, soreness
 Differential skin lesions: Can co-exist must exclude
neoplastic lesions or VIN.
 Biopsy: Biopsy from edge of lesion
 Pathogenesis: Arises from sweat glands and 10% patients
have underlying sweat gland adenocarcinoma.
 Histology:paget’s cell-IHC shows, PAS+ (mucin secreting
cells), Keratin + (intermediate fillaments), S100-.
 The gastrointestinal, urinary tract and the breasts should
be checked
 TREATMENT-Surgical excision to exclude adenocarcinoma
,photodynamic therapy and topical imiquimod

38. CONDYLOMA ACUMINATUM (GENITAL WARTS )
 Presentation: Numerous often large white warty
neoplasms of vulvar skin
 Symptoms: None, itching
 The disease is transmitted sexually
 Pathogenesis: Usually benign genital warts caused
by HPV (subtypes 6,11).
 Histology: Koilocytosis (clear halo around raisin
like nuclei, a viral effect) in papillomatosis
formations . Usually benign but rarely progress to
squamous cell cancer.
 Differential skin lesions: Can co-exist ,must
exclude neoplastic lesions or VIN.
 Biopsy: If fails to respond to therapy or suspicious
lesions.
 Treatment-25% TCA,podophyllin ,cryosurgery
,electrodiathermy ,CO2 laser,interferon

39. VULVAL PAIN DISORDERS:

40. ISSVD 2015 CLASSIFICATION OF VULVODYNIA

41. VULVODYNIA
 Chronic pain syndrome that affects the vulvar area and
occurs without an identifiable cause affecting up to
16% women
 Symptoms typically include a feeling of burning or
irritation. For diagnosis symptoms must last at least 3
months. (BURNING VULVA SYNDROME )
 The exact cause - unknown but is believed to be
multifactorial- including genetics, immunology, and
possibly diet.
 Diagnosis is by ruling out other possible causes that
may include biopsy of the area.

42. POTENTIAL FACTORS ASSOCIATED WITH
VULVODYNIA

43. TREATMENT OF VVD (VULVODYNIA )
 General measures: Minimizing exposure to contact irritants
 Topical agents: Local Anaesthetic (LA): Topical lidocaine gels or ointments-
for provoked VVD making penetrative sex possible, applied 15–20 mins
prior to sex.
 Oral analgesics: Paracetamol, NSAIDs
 TCADs: Effective particularly for unprovoked VVD.
Dosage: Amitriptyline -Start 10 mg OD increasing weekly until pain
controlled . Average dose 60 mg divided daily up to 100 mg-For 3-6 months
 Other drugs:Gabapentin and Pre-gabalin at increasing dosages.
 Combining medication with Psychosexual counselling, physiotherapy and
dietary advice significantly improves pain free outcome rates.
 However, The optimal drug treatment for VVD remains unclear due to a
lack of well-conducted trials
 Surgical removal of the vestibule also has a better outcome when other
measures have been tried
 Only a minority of patients may be suitable for surgery

44. PREMALIGNANT VULVAL LESIONS
♦♦ Vulval intraepithelial neoplasia
(VIN)
♦♦ Paget’s disease
♦♦ Lichen sclerosus
♦♦ Squamous cell hyperplasia
♦♦ Condyloma accuminata

45. VULVAL INTRAEPITHELIAL
NEOPLASIA

46. INTRODUCTION -
 Vulvar squamous intraepithelial lesions (SIL), previously referred to as vulvar intraepithelial neoplasia (VIN)
 Group of premalignant conditions of the vulva
 No routine screening methods available
 The prevalence of vulvar SIL is higher in premenopausal women –Avg age of diagnosis-46 years
 There is increased prevalence of associated CIN (10–80%)
 It is often related with STD such as condyloma accuminata, herpes simplex virus II, gonorrhea, syphilis or
Gardnerella vaginalis
 HPV 16, 18, 31, 35 have been found to be associated with VIN lesions
 Location-The interlabial grooves, posterior fourchette, and perineum

49.  Vulvar LSIL is a benign lesion and is not considered a premalignant lesion.
 HSILs can be subdivided based on their morphologic and histologic features
as-BASALOID SUBTYPE and WARTY (CONDYLOMATOUS) SUBTYPE
 dVIN includes lesions that are not associated with HPV but are associated
with vulvar dermatoses, mainly lichen sclerosus
 dVIN and HSIL are neoplastic (premalignant) changes, with dVIN accounting
for 2 to 29 percent of such changes and HSIL comprising the rest
 HSIL occurs much more frequently than dVIN
 dVIN is more likely to progress to invasive carcinoma-80 percent of
keratinizing vulvar cancers

50. RISK FACTORS AND PREVENTION
RISK –
 Human papillomavirus (HPV):high-risk types (16, 18, 31)
can be found to be a/w HSIL
 Cigarette smoking
 Immunodeficiencies –more common in women with HIV
infection
 Vulvar dermatosis –such as Lichen sclerosis
PREVENTION –
 The quadrivalent and 9-valent HPV vaccines
 Encourage to stop using tobacco
 Earlier detection and proactive management of vulvar
dermatosis

51. CLINICAL PRESENTATION -
 Asymptomatic
 Vulvar pruritus –Most common ,Other potential
symptoms are vulvar pain, burning,or dysuria,bleeding
from vulvar ulcer
 Vulvar lesion/lump
 Persistent abnormal cervical cytology with no
abnormality identified on cervical biopsy- multicentric
origin, which is actually representative of disease in other
nearby genital tract sites (such as the vulva, vagina, and
anus)

52. DIAGNOSTIC EVALUATION
 HISTORY –Risk factors assessment
 LOCAL EXAMINATION reveals a lesion with white, grey, pink or dull red color.
Lesions look rough, raised from the surface and often multifocal
 APPLICATION OF 5 PERCENT ACETIC ACID turns VIN lesions white with
punctuation and mosaic patterns,best seen with a colposcope.
 Cytologic screening of the vulva is not useful and unreliable.
 Confirmation of diagnosis is done by biopsy.-Usually 3–5 mm diameter
dermal punch is taken under LA . Larger biopsy when required may be taken
using a scalpel. Multiple site biopsies are useful.
 A complete pelvic examination is to be done
 To exclude vaginal or cervical neoplasia, cytologic evaluation has to be
performed.

53. SURGERY-
 Local excision—Wide local excision with 1 cm margin is reserved in young
patient with localized lesion
 Laser therapy—CO2 laser vaporization -It gives better cosmetic results
with lower recurrence rate.
 Skinning vulvectomy is less commonly done.
 Simple vulvectomy—It is employed in diffuse type especially in
postmenopausal women - Long-term follow-up is needed as the risk of
recurrence is high (40–70%)
TREATMENT
Differentiated VIN — For women with dVIN, we recommend surgical
excision rather than ablation or pharmacologic therapy

54. Alternatives treatment
TOPICAL THERAPIES-
IMIQUIMOD
Imiquimod cream (Aldara) is a topical immune response modifier –
applied to individual lesions
 Dosing-thin layer of cream 3-5 times per week (alternate days
)for 16 weeks
 INDICATION-
 initial treatment for recurrent vulvar HSIL
 patients with clitoral lesions who prefer to avoid excision and
ablation, provided that they are able to comply with a long
treatment course (typically 16 weeks)
TOPICAL FLUOROURACIL -only rarely used and as a last resort
when other therapies have failed

55. POSTTREATMENT SURVEILLANCE
 GYNECOLOGIC EXAMINATION
every six months for five years and then annually
 COLPOSCOPY AND BIOPSIES -if the patient
exhibits symptoms and/or examination findings
concerning for additional disease

56. VULVAL CARCINOMA

57. INTRODUCTION
 Vulvar cancer is
uncommon, accounting
for only 2%–5% of
gynecologic malignancies
 Squamous cell carcinoma
(SCC) of the vulva, the
most common subtype
 Median age 68yrs

58. PATHOGENESIS
Two independent pathways of vulvar carcinogenesis
are:-
Mucosal HPV (Human Papilloma Virus) infection
&
Chronic inflammatory (vulvar dystrophy)

59. The risk of developing vulvar cancer is increased by
the following:-
 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)

60. PATHOLOGY
 Sites: The commonest site is labium majus followed by
clitoris and labium minus. Anterior two-third are
commonly affected.
 Naked Eye
1. Ulcerative: The features are raised everted edges,
sloughing base with surrounding induration. This is
common.
2. Hypertrophic: The overlying skin may be intact or it
ulcerates sooner or later. This is rare.

61. SPREAD-
DIRECT:
 Occurs to the urethra, vagina, rectum and even to pelvic
bones.
LYMPHATICS:
 It is the commonest method of spread
 About 50 percent of the lymph glands are involved by the
time of presentation
HEMATOGENOUS: This is rare but may occur in advanced
cases.

62. LYMPHATIC SPREAD
 Primarily by embolization and only at a late stage-by permeation to fill the lymphatic channels
 Contralateral metastases -(25%) as the lymphatics of the vulva cross the midline
 Lymphatics of the clitoris, anus and rectovaginal septum may drain directly into the pelvic lymph nodes
 When the ipsilateral nodes are not involved -contralateral groin node spread is very unlikely.
 Sequential pattern. The lymphatics of labia → superficial inguinal lymph nodes → deep inguinal lymph
nodes → pelvic nodes
 Pelvic nodes are secondarily involved in about 20 percent--obturator, external iliac, hypogastric and
common iliac.
 Involvement of pelvic nodes, bypassing the inguinal lymph nodes, is less than 3 percent.
 BILATERAL LYMPH NODE INVOLVEMENT :Directly related to the site(Midline structure) , size of the lesion and
the depth of stromal invasion
 Regional lymph nodes are assessed clinically , by using MRI , sentinel node lymphoscintigraphy ,
ultrasound and PET

63. DEPTH OF STROMAL INVASION AND GROIN LYMPH
NODE INVOLVEMENT IN SQUAMOUS CELL CARCINOMA
OF VULVA:
DEPTH OF INVASION
(MM)
PERCENT OF POSITIVE
NODES
<1 0
1-2 7.5
2.1-3 10
3.1-5 30

64. HISTOLOGICAL SUBTYPES OF VULVAL CANCERS
„
•Squamous cell carcinoma–
90%
•Melanoma -5%
•Adenocarcinoma (bartholin’s
gland)-Rare
•„basal cell carcinoma-2%
•„Sarcoma-1-2 %

65. CLINICAL FEATURES
SYMPTOMS
 Asymptomatic
 Pruritus vulvae
 Swelling with or without offensive
discharge
 Difficulty in urination
 Vulval ulceration
 Bleeding
 Inguinal mass
 Pain
SIGNS
 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.The ulcer has a
sloughing base with raised, everted
and irregular edges and bleeds on
touch
 Tenderness in the vulvar area
 „Inguinal lymph nodes of one or
both the sides may be enlarged and
palpable„
 Clinical examination of the pelvic
organs,including the cervix, vagina,
urethra and rectum

66. DIAGNOSIS
 Basic blood tests –CBC ,LFT/RFT
 When a definite growth is present, the biopsy is to be taken from the
margin
 Chest x ray, chest CT,Cystourethroscopy, Proctoscopy CT/MRI scan (for
nodes) may be needed
 Whole body PET/CT if recurrence/metastasis is suspected
 In cases of vulval dystrophy-multiple areas usually from the persistent red
areas or from stained areas following toluidine blue test are biopsied
 Consider HPV testing
 Consider HIV testing

67. DIFFERENTIAL DIAGNOSIS
 Condyloma accuminata
 Syphilitic ulcer
 Tubercular ulcer
 Lymphogranuloma venereum
 Soft sore

68. PROGNOSIS
STAGE SURVIVAL
I 90-100%
II 65-75%
III 35-45%
IV 20-30%
SURVIVAL BY NODE STATUS
(5 YEARS)
Negative nodes 80-100%
Positive inguinal
femoral lymph
nodes
30-50%
Positive pelvic
lymph nodes
10-20 %

69. CAUSES OF DEATH
 Uremia—from ureteric obstruction due to enlarged
common iliac and paraaortic nodes
 „Rupture of the femoral vessels by the overlying involved
inguinal lymph glands
 „Sepsis

70. MANAGEMENT
PROPHYLACTIC
 Adequate therapy for non-neoplastic epithelial disorders of
the vulva
 Adequate therapy for persistent pruritus vulvae in
postmenopausal women
 Frequent use of multiple biopsies in conservative treatment of
VIN
 Liberal use of simple vulvectomy in postmenopausal women
with VIN where follow-up facilities are not available

71. DEFINITIVE TREATMENT
 SURGERY (wide local excision,Modified radical
vulvectomy with or without B/L inguinal
Lymph dissection or sentinel lymph node
biopsy)
 RADIOTHERAPY
 CHEMORADIATION
 NEOADJUVANT CHEMOTHERAPY

72. TYPES OF SURGERY
 EXCISION-The cancer and an edge (margin) of normal, healthy skin (usually at least ½ inch) around it and a
thin layer of fat below it are excised . This is sometimes called wide local excision. If extensive (a lot of
tissue is removed), it may be called a simple partial vulvectomy.
 SKINNING VULVECTOMY—removes the top layer of skin .This is an option for treating extensive VIN, but
this operation is rarely done.
 SIMPLE VULVECTOMY—removes multiple layers of skin and superficial subcutaneous tissue
 PARTIAL VULVECTOMY/MODIFIED RADICAL HYSTERECTOMY —removes a part of the vulva, as well as
deep subcutaneous tissue and lymph nodes
 COMPLETE RADICAL VULVECTOMY--the entire vulva and deep tissues, including the clitoris, are removed.
A complete radical vulvectomy rarely needed.
 VULVAR RECONSTRUCTION-secondary intention ,split skin grafts and flap coverage
 PELVIC EXENTERATION

73. Modified radical vulvectomy
 THREE INCISION TECHNIQUE is preferred -(i) Vulval
incision, (ii) Groin incision one on either side
 Groin incision is a crescent-shaped one, starting about
2–4 cm medial and about 2 cm below the
anteriorsuperior iliac spine. The incision curves
graduallydownwards above the inguinal ligament
medially to the superficial inguinal ring or about 2 cm
below and 2 cm medial to the pubic tubercle. A strip of
skin (2–4 cm) width is excised
 Vulval incision –
Outer incision—an elliptical incision is made commencing
anteriorly on the mons pubis → encircling
laterally along the medial side of labiocrural
fold → posteriorly across the mid-line of
perineum
Inner incision — passes around the introitus and
anterior to urethra

74. COMPLICATIONS ASSOCIATED WITH VULVAL AND
INGUINAL LYMPH NODE SURGERY
 wound breakdown
 wound infection
 deep vein thrombosis and pulmonary embolism
 pressure sores
 introital stenosis
 urinary incontinence
 rectocele
 faecal incontinence
 inguinal lymphocyst
 lymphoedema
 hernia
 psychosexual complications

75. FIGO STAGING OF CARCINOMA OF VULVA-
The depth of invasion is defined as the measurement of the tumor from the epithelial–stromal
junction of the adjacent most superficial dermal papilla to the deepest point of invasion

76. TNM staging vs FIGO staging

77. PRINCIPLES OF SURGERY: SURGICAL STAGING
 Staged using the American Joint Committee on Cancer (AJCC) and (FIGO) staging systems
 Involves complete surgical resection of the primary vulvar tumor(s) with at least 1-cm
margins and either a unilateral or bilateral inguinofemoral lymphadenectomy, or an SLN
biopsy in selected patients
 Inguinofemoral lymphadenectomy removes the LNs superficial to the inguinal ligament,
within the proximal femoral triangle, and deep to the cribriform fascia
 LN status is the most important determinant of survival
 The current standard involves resection of the vulvar tumor and LNs through 3 separate
incisions
 The choice of vulvar tumor resection technique depends on the size and extent of the primary
lesion and may include radical local excision and modified radical vulvectomy
(NCCN-2018)

78. PRINCIPLES OF SURGERY (NCCN-2018)
 For a primary vulvar tumor located within 2 cm from or crossing the vulvar midline,
a bilateral inguinofemoral lymphadenectomy or SLN biopsy is recommended
 Locally advanced disease- neoadjuvant radiation with concurrent platinum-based
radiosensitizing chemotherapy
 If a complete response is not achieved, surgical resection of the residual disease is
recommended
 The management of bulky inguinofemoral LNs in the setting of an unresectable or
T3 primary vulvar lesion –
1) primary cytoreductive surgery followed by platinum-based chemosensitizing
radiation to the bilateral groins and primary vulvar tumor
2) platinum-based chemosensitizing radiation to the bilateral groins and primary
vulvar tumor alone

79. INGUINOFEMORAL SENTINEL LYMPH NODE BIOPSY
 Alternative standard-of-care approach to lymphadenectomy in select women with SCC of the
vulva
 Eligibility criteria for SLNB
● Tumor diameter <4 cm
● >1 mm depth of invasion
● No palpable groin lymph node
● Unifocal disease
 Inguinal lymphadenectomy is a/w a high rate of postoperative morbidity; 20%–40% have wound
complications and 30%–70% -risk for lymphedema
 The radiocolloid most commonly injected is technetium-99m sulfur colloid-2 hr prior to
procedure
 Dye most commonly used is Isosulfan Blue 1%-intradermally in the operating room within 15–30
minutes of initiating the procedure.
 Performed prior to the excision of the vulvar tumor, so as not to disrupt the lymphatic network
 A complete inguinofemoral lymphadenectomy is recommended if an ipsilateral SLN is not
identified
 If ipsilateral SLN is positive, the contralateral groin should be evaluated surgically and/or treated
with EBRT

80. PRINCIPLES OF RADIATION
THERAPY
INDICATION
 As adjuvant therapy following initial surgery,
 As part of Primary therapy in
1. locally advanced disease,
2. for secondary therapy/palliation in recurrent/metastatic
diseases
Tumor-directed EBRT is directed to the vulva and/or
inguinofemoral, external, and internal iliac nodal regions.
Brachytherapy can sometimes be used as a boost to
anatomically amenable primary tumors

81. POST-OPERATIVE RADIATION
INDICATIONS
 Lympho-vascular invasion
 depth of invasion > 5mm
 positive surgical margins
 more than one + node
 node with extracapsular invasion

82. CHEMORADIATION
INDICATIONS
 Anorectal, urethral, or bladder involvement (in an
effort to avoid colostomy and urostomy)
 Disease that is fixed to the bone
 Gross inguinal or femoral node involvement
(regardless of whether a debulking lymphadenectomy
was performed)

84. PRIMARY TREATMENT- STAGE WISE
 EARLY-STAGE (STAGE I/II)
 LOCALLY ADVANCED (STAGE III/IVA/IVB WITH
PELVIC CONFINED DISEASE)
 DISTANT METASTATIC DISEASE (EXTRAPELVIC
STAGE IVB) NCCN-2018
National Comprehensive Cancer
Network

85. NCCN GUIDELINES 2018 VULVAR CANCER (SQUAMOUS
CELL CARCINOMA)-EARLY STAGE
IF WIDE LOCAL RESECTION PATHOLOGY REVEALS TUMOR IN AGGREGATE
OF ≥1 MM INVASION, THEN ADDITIONAL SURGERY MAY BE WARRANTED.

86. primary risk factors include: close tumor margins, lymphovascular
invasion, tumor size, depth of invasion, and pattern of invasion
(spray or diffuse

87. IF IPSILATERAL GROIN IS POSITIVE, THE CONTRALATERAL GROIN SHOULD EVALUATED
SURGICALLY AND/OR TREATED WITH EBRT

88. LOCALLY ADVANCED (STAGE III/IVA/IVB WITH PELVIC CONFINED DISEASE)
LARGER T2 TUMORS: >4 CM
AND/OR INVOLVEMENT OF THE
URETHRA, VAGINA, OR ANUS.

90. DISTANT METASTATIC DISEASE
(EXTRAPELVIC STAGE IVB)

91. SURVEILLANCE

92. RECURRENCE

94. THANK YOU