Vulvar cancer is an uncommon gynecological cancer that mainly affects elderly women. Squamous cell carcinoma is the most common type, comprising 92% of cases. Lymphatic drainage occurs to ipsilateral lymph nodes but some areas can drain to bilateral nodes, facilitating spread. Diagnosis is by biopsy and treatment involves wide local excision or radical vulvectomy with lymph node dissection. Prognosis depends on stage, with 5-year survival rates of 90% for stage I but only 11% for stage IVB.

1. Vulvar cancer
-DR. DIVYA SHIVANAND SHANBHAG

2. INTRODUCTION
 It is a uncommon cancer comprising 4% of gynecological malignancies
and 0.6% of all cancers in women.
 mainly a disease of elderly women(mean age 65 yrs) but 15% cases occur
in young age .
 The incidence is rising due to HPV infection and HIV infection and
increased life expectancy .

3.  Lymphatics of vulva donot cross midline and drain into ipsilateral
superficial inguinal and femoral lymph nodes.
 However lymphatics from clitoris and fourchette cross the midline causing
bilateral inguino-femoral lymph node metastasis, and from inguinofemoral
nodes, they spread to external iliac , pelvic and para-aortic lymph nodes .

4. Histological subtypes of vulvar cancer
 Squamous cell carcinoma – commonest 92% . Can be keratinizing and non-
keratinizing types.
 Vulvar malignant melanoma- 3-4% second most common
 Basal cell carcinoma- 2-3%
 Bartholin gland carcinoma – 1% ( adenocarcinoma, squamous carcinoma,
transitional cell carcinoma)
 Vulvar Paget’s disease

5.  Verrucous carcinoma<%
 Vulvar sarcoma <1%
 Vulvar metastatic tumor %
 Undifferentiated tumors

6. Types of squamous cell carcinoma
 1. Type 1 warty or basaloid type: It occurs in younger patients (<50 years of
age). It is multifocal and is related to HPV infection , vulvar intraepithelial
neoplasia (VIN) , smoking , immunosuppression and sexually transmitted
diseases.
 2. Type 2 keratinizing , differentiated or simplex type: it is usually unifocal (
single lesion ) found in elderly patients , found in elderly patients , found
in areas adjacent to vulvar diseases like lichen sclerosis and squamous
hyperplasia and can be due gene mutation p53 . It is not related to HPV
infection.

7. Etiology and risk factors
 Human papilloma virus(HPV) infection- high risk serotypes (mainly 16, but
18, 31,33 can also cause) are implicated in etiology of both VIN and vulvar
cancer. Hence prophylactic HPV vaccine against high risk HPV strains can
reduce incidence of vulvar cancer .
 Herpes simplex with smoking as a cofactor but never alone
 Chronic immunosuppression in conditions like transplant patients on
immunosuppressive drugs, HIV positive women are at an increased risk

8.  Lichen sclerosis
 VIN 2 and VIN 3 can develop into vulval cancer in 4 years in young women.
 Alcohol consumption
 Smoking

10. Clinical presentation
Symptoms
 1. asymptomatic
 2. vulval pruritus of long duration (commonest symptom)
 3. vulvar irritation
 4. vulvar pain
 5. vulvar mass

11.  6. Non-healing vulvar cancer
 7.vulvar bleeding
 8. vulvar discharge
 9.dysuria and difficult micturition
 10. rectal bleeding and painful defecation
 11. inguinal mass

12. Signs
 Irregular fungating mass
 Irregular ulcer
 Warty growth
 Plaque like lesion
 Red or white pigmentation on the vulvar lesion
 Tenderness over the lesion may or may not be present
 Unilateral or bilateral inguino- femoral lymphadenopathy

13. History taking
 Careful history taking for symptoms and their duration
 Any past history of sexually transmitted disease, any condylomata or vulvar
disease should be elicited
 History of smoking or any immunosuppressive drugs or HIV infection
should be asked

14. Physical , local and vaginal
examination
 Careful evaluation of the vulva should be done for size , location, extent of
lesion , whether warty or ulcerative growth , single or multi focal lesion.
 Any involvement of vagina, urethra, base of bladder or anus
 Speculum , vaginal and per rectal examination are performed
 Look for lesion in cervix and vagina
 The inguinal region is palpated for inguino femoral lymph nodes, their
size, number, any fixity to the skin

15. Vulvar biopsy-
 Diagnosis is made by directed biopsy from the suspected area . Biopsy is taken either
under local anesthesia with 1% xylocaine infiltration or under regional or general
anesthesia . It is of two types
 A) keyes punch biopsy-
-skin over the lesion is made taut with left hand
-keyes punch is put against the lesion firmly and rotated with a constant firm pressure
clockwise and then counter clockwise for penetration into the skin and to reach
subcutaneous fat (loss of resistance)
-the keyes punch is removed, the circular tissue is grasped and biopsied, the biopsy is about
4-6 mm and has both dermis and epidermis and is sent for HPE

16. Keyes punch biopsy

17.  Wedge biopsy- when large tissue is needed or keyes punch biopsy is not
available, wedge biopsy is taken with knife
-a 2-0 chromic catgut or vicryl suture suture is applied for hemostasis and
cosmetic results

18. Other investigations
 Pap smear for cervical and vaginal cytology to rule out CIN and VAIN
 Colposcopy of cervix and vagina to rule out CIN and VAIN
 Vulvoscopy for inspection of other lesions on vulva. Toluidine blue can be
used to localize sites for biopsy
IMAGING MODALITIES
- USG pelvis
- CT, MRI , PET
- cystourethroscopy
- proctosigmoidoscopy

19. - Intravenous urogram
- lymphography- to detect smaller lymph node metastasis (2-5mm)
- Routine preoperative investigations for fitness for surgery
a) Complete hemogram
b) urine routine , microscopy and culture
c) LFTs
d) KFTs
e) Xray chest PA view
f) ECG

20. Staging of carcinoma vulva

21. Mode of spread of vulvar cancer
 Direct extension to adjacent structures
 Lymphatic spread is common and occurs by embolization to regional
lymph nodes , even in early stage.

22.  Superficial inguinal
deep inguinal and femoral lymph nodes
pelvic lymph nodes
Inguinal lymph nodes is the sentinel lymph node of carcinoma vulva.
Over all, lymph node metastasis is seen in 25% cases, of vulvar cancer being
10% in stage 1, 30% in stage 2, 75% in stage 3, 98-100% in stage 4.

23.  Hematogenous spread- it occurs rarely in the late stages and can occur
even without inguinofemoral lymphnode involvement

24. Differential diagnosis
 Syphilitic ulcer
 Tubercular ulcer
 Lymphogranuloma venerum
 granuloma inguinale
 Chancroid
 vulvar elephantiasis
 lichen sclerosis
Vulvar biopsy can differentiate between different conditions

25. Treatment
 Surgical treatment is the main treatment modality for carcinoma vulva. There is
need to have adequate resection margin of 1 cm and groin node dissection in
most cases.
1) Wide local excision or simple partial vulvectomy- excision is with 2 cm
surgical margin around the lesion and depth of 1 cm upto colles fascia
2) Radical partial vulvectomy- usually combined with ipsilateral
lymphadenectomy-

26. a) right or left hemivulvectomy – one sided labium majus and minus
depending upon the site of lesion are removed.
B) anterior hemivulvectomy- removal of clitoris and partial resection of labia
minora, majora and mons pubis.
C)posterior hemivulvectomy- removal of portion of labia majora, bartholian
glands and upper perineal body. Suitable of posterior lesion.

27. 3) radical total vulvectomy- removal of all the vulva to the level of perineal
membrane and the periosteum of the pubic rami .
 For adequate margins two elliptical incisions are made on the vulva.
 The inner incision is on vaginal introitus and vestibule
 The outer incision is made on the labiocrural folds
 The dissection is carried out to deep down to the deep perineal fascia.
 There should atleast 1 cm free margin around the tumor.
 All intervening subcutaneous tissue is excised.

28.  The enbloc incision also called butterfly or longhorn incision in which a
single incision is made from one anterior iliac spine to another.
DISADVANTAGES OF ENBLOC DISSECTION
 Extensive loss of vulvar tissue with distortion with psychosexual sequelae
 High incidence of wound break down
 High incidence of lymphedema especially in lower limbs

29. Triple incision technique
 General preparation ( consent, counselling, iv antibiotics, arrangement of
blood , anesthesia , spinal or genral ), lithotomy position
 The outer incision is given starting from the mons pubis,inguinocrural folds
on either side and to meet on perineum.
 The inner incision is given on the introitus and on vestibule anterior to urethra.
 The lateral incisions are deepened upyo perineal membrane and dissected
medially to the inner incision and thus removing labia majora and minora with
their fat pads

31. Ultra radical vulvectomy
 For resectable tumor but with the involvement of distal urethra , vagina,
and anus radical vulvectomy with partial resection of theses structures with
b/l inguinofemoral lymphadenectomy.
 ADVANTAGES
1)Easier to do
2)Significant reduction in wound morbidity
3)Cure rates are almost similar as metastasis rarely occurs in the retained skin
bridge.

32. Inguino femoral lymphadenectomy
 It is integral for all vulvar squamous cancers with size >2 cm and invasion
>1mm.
 Traditionally both superficial inguinal and deep femoral lymph nodes are
removed on both side for evaluation of metastatic disease.
COMPLICATIONS
Early complications
1) Hemorrhage
2) Infection
3) Wound break down
4) Lymphocyst formation

33.  Femoral nerve injury
 Urinary tract infections
 Thromboembolism
 Osteitis pubis
Late complications
1)Chronic lymphedema
2)Cellulitis of leg
3)Urinary incontinence

34.  Fecal incontinence
 Rectocele formation
 Femoral hernia
 Dyspareunia
 Rectovaginal fistula
 Pubic osteomyelitis
 Vulvar disfigurement
 Depression of psychosexual complications

35. Sentinel lymph node biopsy
 The first lymph node to receive tumor lymphatic drainage
 If this node is negative it is most likely that the whole chain is negative ,
and lymphadenectomy can be avoided
 The tracer and dye is taken up by the sentinel node which is blue in color
 If biopsy is positive, b/l inguinofemoral lymphadenectomy is done.
INDICATIONS
1) Unifocal primary vulvar cancer
2) <4cm diameter
3) tumor invasion <1mm
4) Absence of obvious metastasis.

36. Role of radiotherapy
 Preoperatively in patients with advanced disease
 Postoperatively in patients with positive inguinal nodes
 Postoperatively in patients with closed surgical margins < 5mm
 As primary therapy for young women with tumor involving clitoris
 Recurrent disease
 Patient should receive postoperative groin and pelvic irradiation (usually
teletherapy of 45-55Gy)

37. Role of chemotherapy
 Has a very limited role. As a neoadjuvant therapy with radiotherapy.
 The drugs used are
1) Cisplatin
2) 5-flurouracil
3) Bleomycin
4) Mitomycin

38. Stagewise treatment of vulvar cancer
Sl no stage Surgery(treatment)
1 Stage IA Wide local excision with 1 cm normaltissue
margin. No lymphadenectomy (except for
poorlydifferentiated tumor)
2 Stage IB (1) Lateral lesions: wide local excision or radical
partial vulvectomy with ipsilateral
inguinofemoral lymphadenectomy(ii) Midline
lesions (clitoris, posterior fourchette): wide
local excision (radical partial vulvectomy with
bilateral inguinofemoral lymphadenectomy)
For older patients: Radical vulvectomy with
bilateral inguinofemoral lymphadenectomy
(triple incision) is preferred

39. SL NO STAGE TREATMENT
3 Stage II Radical vulvectomy with
bilateral inguinofemoral
lymphadenectomy.
Resection of involved lower
third of urethra, vagina or
anal canal can be
performed along with.They
can also be managed by
partial radical vulvectomy
with 1 cm surgical margin
with bilateral inguino
femoral lymphadenectomy
with similar results.

40. SL NO STAGE TREATMENT
4 ) STAGE III IIIA Radical or partial radical
vulvectomy with bilateral
inguinofemoral
lymphadenectomy.
IIIB Radical vulvectomy with
bilateral inguinofemoral
lymphadenectomy with
postoperative radiotherapy
IIIC Preoperative radiotherapy
followed by surgery
(limited resection) after 6
weeks. Sometimes pelvic
exenteration can be done.

41. STAGE III

42. SL NO STAGE TREATMENT
5 Stage IV (i) Chemoradiation
(chemotherapy with
radiotherapy) is preferred.
It may be followed by
limited resection of residual
disease.(ii) Pelvic
exenteration can be tried in
stage IVA.(iii) For stage IVB:
palliative chemoradiation is
given.

43. Prognosis
 Stage I- 90%
 Stage II-invasive cancer with negative nodes-80%
 Stage III-positive groin nodes-50%
 Stage IVA-15%
 Stage IVb- 11%

44. Recurrence
 About 30% cancers recur.
 Management of recurrent disease
1) In vulvar recurrence- wide local excision
2)In groin recurrence-radiotherapy
3)In pelvic recurrence-pelvic irradition
4)Distant recurrence- palliative chemotherapy

45. Vulvar melanoma
 It is second most common vulvar cancer(5-10%)
 It usually arises from the labia majora, minora and clitoris and may arise in
pre-existing pigmented nevi.
 Diagnosis is by biopsy of vulvar lesion
 TREATMENT
1) Chemotherapy and radiotherapy(limited response)
2) Surgery is the best definitive therapy – radical partial vulvectomy
3) Adjuvant with alpha interferon or radiotherapy
4) Prognosis is very poor (<20%)

46. Vulvar melanoma

47. Verrucous carcinoma of vulva
 Rare type of squamous cell carcinoma of vulva
 Exact etiology unknown, HPV genome has been found in some tumors.
 They are locally invasive tumors
 Metastais is rare
 Surgery is the mode of Treatment.
 Usually lymphadenectomy is not needed.
 Radiotherapy is contraindicated in verrucous carcinomas it may stimulate
anaplastic transformation.
 Recurrence is also treated by surgery.

48. Verrucous carcinoma of vulva

49. Basal cell carcinoma (rodent ulcer) of
vulva
 It accounts for about 2% vulvar cancers in elderly women
 It is usually present in labia majora as an ulcerv with rolled edges and
central ulcer with poor pigmentation with pruritus,pain and bleeding and
may be confused with eczema or psoriasis
 Treatment is by radical partial vulvectomy
 If surgery is conintaindicated then radiotherapy or local
immunomodulator( imiquimod) can be given.

50. BASAL CELL CARCINOMA (RODENT ULCER) OF VULVA

51. Vulvar sarcoma
 Rare tumor and can be leiomyosarcoma, arising from the smooth muscles
of the round ligaments.
 It develops as an isolated mass on labia.
 Outcome depends on the size, invasion and grading of the tumor.
 Prognosis is poor
 Treatment is by radical vulvectomy with inguinofemoral
lymophadenectomy.
 For unresectable sarcoma treatment is by chemotherapy and radiotherapy.

52. Bartholin gland carcinoma
 Rare tumor arising from the Bartholin gland and can be adenocarcinoma.
 Clinical features
1) Dyspareunia
2) Enlargement and recureent abscesses
3) Solid tumor-FNAC or biopsy should be done
TREATMENT
1) For early stage cancer- radical partial vulvectomy with inguinofemoral
lymphadectomy
2) Postoperative radiation can be given to avoid the local recurrence

53. Bartholin gland carcinoma

54. Secondary tumors ( metastasis) of
vulva
 Metastasic tumors can affect vulva in 5-8% cases and can be from adjacent
organs like bladder , urethra or distant organs like breasts , kidneys ,
lungs.
 Treatment is of primary disease and is usually chemo and radiotherapy.