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Carcinoma Vulva
1. CARCINOMA VULVA
Prof. M.C.Bansal
MBBS,MS,MICOG,FICOG
Professor OBGY
Ex-Principal & Controller
Jhalawar Medical College & Hospital
Mahatma Gandhi Medical College, Jaipur.
2. Varieties of vulval
carcinoma
Cancer of vulva accounts for 1 to 5% of all genital cancers.
Malignant tumours of vulva are groupe as follows---
1.Preinvasive lesions— VIN I,II,III.
Bowens disease
Pagets disease } Intraepethelial ca.
Micro invasive
2.Invasive Lesions – Sqamous cell carcinoma 90% most common
Melanoma - 3-5%
Adenocarcinoma - 1%
Sarcoma - 2%
Rodent ulcer or Basal cell carcinoma - 1%
3. Metastatic tumours
In 5% cases the lesions are multifocal , and are seen in young women
below 40 years.
3. Preinvasive Lesions
Definition Intra epithelial vulval cancer is defined as
Cellular abnormality limited to the epithelium of vulval
skin excluding the keratinized layer. Presence of
acanthosis, intraepithelial pearl formation and
inflammatory reaction in dermis are the other
characteristics of this lesion.
Classification
VIN I - cellular abnormality is mild , limited to the basal
layer.
VIN II- cellular abnormality is limited to lower 1/3rd of
the vulval epithelium , basal & intermediate layers.
VIN III- entire thickness is involved and shows cellular
abnormality.
4. VIN
Incidence Increasing because of increased awareness,
early detection, increased age < 70yrs.
Aetiology Chronic vulval irritation ,Immunosuppressive
conditions ,HIV ,Human Papilloma virus infection , smoking
STD , dystrophies , poor nutrition , poor hygiene and local
moisture are also contributing factors.
50% VIN cases have sequential or concomitant neoplasia in
the lower genital tract.
VIN lesions are reported in young women <40 years.
Obesity , Diabetes , Chronic puritus and dermatitis are
often linked to this disease.
HPV DNA detection combined with cytology improves the
detection test to 95%.
5. Histology A loss of polarity and stratification , dystrophic changes are
confined to epidermis ( epithelium only). Basement membrane remains intact.
Clinical Features
Asymptomatic for long time.
Purities is common symptom usually being treated as
fungal infection in early cases.
Later on soreness , dysuria , dyspareunia develop.
Pre existing leucoplakia , condyloma and dystrophy may
show — white/red flat or papular lesions –single/multiple.
Multiple wide spread lesions are more common in young women
and occurs in5-25 % .
Some develop Pigmentation.
Purities -- Scratch /cuts – secondary infected.
.
9. Investigations
-Difficult to diagnose and differentiate from
dystrophies – Biopsy .
-Exfoliative cytology / colposcopy Are off no
use because of poor exfoliation and
keratinization.
-5% acetic acid application , 1% Toludine blue
stains abnormal areas . Excisional biopsy of a
localized lesion picks up VIN.
10. Management Of VIN
Purpose of Rx---
1.To relieve symptoms of prurius and
soreness.
2.To prevent development of invasive cancer.
3. To avoid mutilating surgery and sexual
dysfunction in young woman . Redical
vulvectomy causes disfigurement and
dyspariunia.
11. VIN Rx
Rx depends on age , sexual activity , site and extent of VIN
and its grading.
Conservative management in place of radical is giving 90-
94 % success.
Modern Management VIN I &II with multiple lesion
showing euploidy can be watched for 6 months to regress
spontaneously in young woman having
Immunocompromised state like pregnancy , following
recent viral infection.
With unifocal lesion local excision including 2cm healthy
area all around . Closure of wound by stitching and
approximating the margins. Local recurrence is to be watch
for.
12. VIN Rx
Persistent / recurrent lesions of early grades VIN II /VIN III need
skinning vulvectomy with split thickness skin graft to avoid
dyspareunia.
Laser excision—Co2 Laser.
Cryosurgery —up to depth of 2cm.
Local application of Dinitrochlorobenzene , 5% testosteron e and
corticosteroid cream .
Flouracil (5Fu) 5% cream applied locally for 6-8 weeks . It causes
sloughing and burning.
Prophylactic -- HPV vaccination.
Photodynamic therapy -- tumour photo sensitizer-5-amino-
leuvinic acid application combinned with non thermal light of
appropriate wave length causes nascent oxygen induced cell
death.
Elderly woman -- simple vulvectomy.
13.
14. Follow up
Reccurance around excised lesion or fresh
lesion - 20-30 % cases.
5-10% progress to invasive cancer in 8-10 yrs.
Regular follow up at 6-12 monthly interval.
15. Bowen’s Disease
It is intra epithelial carcinoma of vulva.
A slow growing hard excematous surface.
Pruritus is the main symptom.
It rarely metastastises.
Biopsy reveals typical prickle cells invading the
epidermis and presence of giant cells.
The vagina & Cx both may be involved
simultaneously.
Simple vulvectomy is the treatment of choice.
16. Paget’s Disease
A rare extra mammary disease.
Apocrine sweat glands are involved.
Slighted elevated , sharply demarcated , white
indurated /excezmatous lesion found in
postmenopausal woman c/o pruritis.
Biopsy reveals typical large pale vacuolated cells
in epidermis . These Paget’s cells are adeno
carcinomatous mucus secreting round cells with
vesicular nucleus and pale cytoplasm.
18. Paget’s disease
20 % cases may have associated
adenocarcinoma of Bartholin gland.
30% cases of perianal lesion may have cancer
anus.
Rx is vulvectomy if no underlying cancer is
detected.
Radiotherapy.
Local and systemic 5Fu and Bleomycin is also
tried.
The tumour recurrence is 20%.
19. Paget’s Disease
A rare extra mammary disease ,comperable to intraductal carcinoma of breast.,
apocrine sweat glands are involved.
It occurs in postmenopausal women.
It is a sharply demarcated and slightly elevated white indurated or eczematous
lesion.
Pruritus is presenting symptom.
HPR –reveals large pale vacuolated cells in epidermis .
Paget cells are adenocarcimatous, mucus secreting round cells with pale
cytoplasm and vesicular nucleus.
20% cases may have underlying carcinoma of Bartholin glands
Perianal lesion though rare ,but may have anal adenocarcinoma in 30% cases.
Local excision or vulvectomy if no underlying cancer is present.
With underlying lesion case is treated as invasive carcinoma Radiotherapy ,local
or systemic 5 Fu and bleomycine are also tried.
The tumor recurs in 20% cases.
20. Verrucous carcinomas
Most commonly found in the oral cavity, but may be found on any moist
membrane composed of squamous epithelium . They are a distinct
entity, with no association with human papillomavirus infection, and a
peculiar distribution pattern of cytokeratins AE1 and AE3 on
immunohistochemical staining .
Grossly, the tumors have a cauliflower-like appearance, and the
diameter of reported lesions ranges from 1 to 15 cm . Microscopically,
they contain multiple papillary fronds that lack the central connective
tissue core that characterizes condylomata acuminata. The gross and
microscopic features of a verrucous carcinoma are very similar to those
of the giant condyloma of Buschke-Loewenstein, and they probably
represent the same disease entity . Adequate biopsy from the base of
the lesion is required to differentiate a verrucous carcinoma from a
benign condyloma acuminatum or a squamous cell carcinoma with a
verrucous growth pattern.
.
21. Verrucous carcinomas (contd)
Usually occur in postmenopausal women, and they are slowly growing
but locally destructive lesions. Even bone may be invaded. Metastasis to
regional lymph nodes is rare.
Radical local excision is the basic treatment, palpably suspicious groin
nodes, should be evaluated with fine-needle aspiration cytologic testing
or excisional biopsy. If the nodes contain metastases, radical local
excision and at least an ipsilateral inguinofemoral lymphadenectomy is
indicated.
Vulvar intraepithelial neoplasia or invasive squamous cell carcinoma
may be seen in association with verrucous carcinoma.
Radiation therapy is contraindicated as it may induce anaplastic
transformation with subsequent regional and distant metastasis . 5-year
survival rate of 94% treated with surgery alone, compared with 42%
treated with surgery and radiation. If there is a recurrence, further
surgical excision is the treatment of choice. This may occasionally
necessitate some type of exenteration
23. Microinvasive Cancer
Microinvasive sqamous epithelioma is common,
melanoma is very rare and detected hystologically.
It is a single lesion measuring 2cm or less with a
depth of invasion not more than 1cm.
It is stage 1 A of figo staging .
Multiple foci even depth < 1cm are not included in
this classification.
Simple excision with sentinel lymph node sampling
is best mode of treatment.
When lymph node is involved radical surgery is
better than radiotherapy.
24. Invasive Carcinoma Of Vulva
Epidemiology
1.Accounts for 2-4% of genital cancers.
2. Common in elderly women 6-7th
decade(60%).Rest before 60yrs of age.
3.Now it is being reported in young patients-
smoking,STD,HIVand HPV infection.
4. vulval cancer is associated with Ca Cx and
ovarian cancer IN 20% cases., may be due to
infection in forma and advancing age in later
case.
5.Nulliparous and woman of low parity are more
disposed to vulval cancer.
25. Invasive Cancer Of Vulva
Aetiology
- Causes are same as that of VIN.
The lesion associated with VIN or
dystrophy progress to invasive
carcinoma of vulva.
-However VIN always does not preceed
invasive carcinoma as seen in cervical
cancer.
26. Invasive Cancer Vulva
80% complain of pruritus ,lump ,swelling or ulcer.
Lump may be raised papular area with pigmentation.
Ulcer has everted margins and idurated base.Surronding skin
may be fissured, cracked and inflamed.
Leucoplakic or dystrophic area may also be present.,may be
single or multiple foci .
Labia major is commonly involved but clitoris,perineal area may
also be involved.
Anterior 2/3rd area is involved more.
Ulcerative lesions bleed and offensively smell.
Pain is late feature when nerve are infiltrated.
Inguinal L.N. may be palpable.
Urethral, rectal symptoms may be preset when cancer spread to
them.
32. Invasive carcinoma of Vulva
Differential Diagnosis
Tubercular ulcer.
Syphilitic condiloma/ulcer
Elephantiasis of vulva.
Soft sore and Lymhogranuloma
Excision biopsy will help .
33. Staging Of carcinoma
Vulva(FIGO)
Stage 0--Intra epthelial,Bowe’s,Paget’s disease
and dystrophy with atypia.
Stage IA— single microinvasive cancer lesion
<1cm invasion and no lymphatic
infiltration.
Stage IB– Lesion < 2cm with No suspicious
lymph glands.(15% +ve lymph node)
Stage II– Lesion > 2cm ,no suspicious lymph
nodes(30%+ve lymph node)
34. Stages of Cancer Vulva(FIGO)
Stage III– 1.lesion of any kind extending
beyond vulva.
2.Unilateral lymph node ivolment
Stage IV A---lesion ivading any of these
structures-urethra, bladder,rectal
mucosa, pelvic bone and/ Bilateral
lymph nodes.
Stage IV B—Distant metastesis , pelvic lymph
nodes.
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36.
37.
38. Spread of Vulvar Cancer
1. Direct spread to the adjacent organs.
2.By lymphatics.
Blood born metastesis are rare.
At first superficial inguinal LN are invaded by
embolism. later lymphatic channel permeation
occurs causing blockade and lymph edema of vulva
& leg.
Deep L N are involved via Cloquet -to the
external iliac, internal iliac, obturator and common
iliac deep pelvic LN.
Centrally located lesion usually invade contra
lateral nodes in 25%cases.
39. Spread of cancer of vulva---
-
Lymph Nodes not clinically suspicious may
have +ve metastasis in 25% cases.
Inguinal L N are involved in---
Stage I—10%
Stage II– 30%
Stage III—70%
Stage IV– 100%
40. Investigations
Diagnostic investigation includes---
Punch or excision biopsy.
cystoscopy.
PR, Anoscopy,Proctoscopy.
X Ray Chest and log bones.
CT and MRI for Lymph Node’s metastasis.
Lymphangiography---superior to CT/MRI.
Mapping is done by Intra operative intra dermal injection of
blue dye around the lesion, detection rate –100%
-Labeling tissue with radio active tracer and localization with
handheld detector.
- Lymphoscintigraphy—100% detection rate.
41. Treatment Of Cancer Vulva
Stage I Lateral lesion <2cm can be delt by
partial vulvectomy with the margins 3cm
beyond the growth-or Unilateral vulvctomy,
accompanied by ipsilateral
lymphadenectomy.If frozen section reveals
no metastasis ., nothing more is needed. if LN
+ve for cancer cells contra lateral node
dissection is must. Pelvic node dissection is
required if cloquet LN is involved .,or pelvic
gland’s radiotherapy may be given post
operatively.
42. Rx of Ca vulva-----
Stage IITumour between2-4cm will require
total vulvectomy and Lymph node dissection/
pelvic radiotherapy.
Tumour <4cm poorly diffrentiated
/melanoma/ adenocarcinoma –nothing less than
Radical vulvectomy ,bilateral inguinal LN and
pelvic LN dissection is needed. Separate Incision
for vulvectomy and inguinal dissection on either
side are used ,so primary closure of wound is
possible or ½ thickness skin graft is used.
43. Rx of Ca Vulva
Stage III Mega voltage Radiotherapy 4000- 5000 rads over a
period of 5 weeks.
Shrunken lesion can locally excised. Local
recurrence can be treated by chemotherapy.
Stage IV It is treated by chemotherapy and/radiotherapy.
Anal involvement is treated by infusion of 5-Fu and
Mytomycin-C Followed by radiotherapy 300rads over 3
weeks.
- 5Fu 750 mg / m 2 is given DAILY FOR 3-5 days and
Mytomycin-C 10mg/m2 bolous given on 1st day.Cisplatinum
is also given now a days with good out come.
Chemotherapy and radiotherapy avoids exentration which
carry high mortality and morbidity,.
-Local excision of residual tumor may be required.
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51. Results of treatment and 5 year
survival rates
Figo Staging 5 years survival rate
Stage I 90%
Stage II 80%
Stage III About 50%
Stage IV About 15%
Total About 60%
52. Way & Taussig’s Radical
vulvectomy 1935
Extensively wide incision for radical vulvectomy
+ bilateral inguinal and pelvic lymphadectomy---
has undergone radical modification in present
era.
Radical surgery can cause---wound
infection,slouhging,haemohrrage.lymph loss,
lymph edema, thromboembolism and High
primary Mortality.
Late sequels are lymph edema, scarring,
disfigurement, dyspareunia,lymphoma, edema
legs. Urinary incontinence.
53. Bartholin Gland’s tumor
It is a rare unilateral tumor.
It is commonly Adeno carcinoma
Carries a poor prognosis.
Redical vulvectomy is the treatment
treatment.
Chemotherapy and radiotherapy can also be
added.
54. Vulval Sarcoma
Rare tumor .
Occurs in reatively young woman.
Treatment is local excision.
Distant metastasis are common.
Chemotherapy is also helpful
Prognosis is very poor.
55. Vulvar Sarcomas
Sarcomas represent 1% to 2% of vulvar malignancies and comprise a
heterogenous group of tumors. Leiomyosarcomas are the most common, and
other histologic types include fibrosarcomas, neurofibrosarcomas, liposarcomas,
rhabdomyosarcomas, angiosarcomas, epithelioid sarcomas, and malignant
schwannomas . The primary treatment is wide surgical excision . Adjuvant
radiation may be helpful for high-grade tumors and locally recurrent low-grade
lesions . The overall survival rate is approximately 70%. Leiomyosarcomas
usually appear as enlarging, often painful masses, usually in the labium majus.
Recurrence was associated with three main determinants: diameter greater than
5 cm, infiltrating margins, and five or more mitotic figures per 10 high-power
fields..
Vulvar smooth muscle tumor should be considered a sarcoma when three or all
of the following four features were present: (i) over 5 cm in greatest dimension;
(ii) infiltrate margins; (iii) 5 mitoses/10 HPF; and (iv) moderate to severe cytologic
atypia .
Lymphatic metastases are uncommon, and radical local excision is the usual
treatment.
56. Vulvar Sarcomas
+ Epithelioid sarcomas characteristically develop in the soft tissues of the
extremities of young adults but may rarely occur on the vulva epithelioid
sarcomas behave more aggressively than their extragenital counterparts.
Epithelioid sarcomas in general have a propensity for extensive local disease at
presentation, local recurrence, lymph node metastasis, and distant metastasis .
Treatment consists of radical excision of the tumor, and at least ipsilateral groin
dissection. Systemic therapy is ineffective.
+ Rhabdomyosarcomas are the most common soft tissue sarcomas in childhood,
and 20% involve the pelvis or genitourinary tract. A multimodality approach has
evolved, principally as a result of four successful protocols organized by the
Intergroup Rhabdomyosarcoma Study Group (IRSG), and survival rates have
improved significantly, with a corresponding decrease in morbidity. Arndt et al.
summarized the results of these four protocols in 2001. All were managed with
chemotherapy (vincristine, dactinomycin ± cyclophosphamide ± doxorubicin),
with or without radiation therapy. Wide local excision of the tumor, with or
without inguinofemoral lymphadenectomy, was carried out before or after the
chemotherapy.
Patients with local and/or regional rhabdomyosarcoma of the female genital
tract have an excellent prognosis, with an estimated 5-year overall survival of
87% .
57. Rodent ulcer
This common lesion presents as ulcer which
keeps invading deeper tissues.
Biopsy reveals Basal carcinoma.
It is locally malignant.
It respond well to wide excision.
Persistent cancer
Persistent cancer is one which develop
within 6 months of primary treatment.
58. Vulval MELANOMA
Accounts for 3-5% of all cases of vulval tumors.
Can occur in any age and may develop ina mole
or de novo.
It is pigmented nodule or superficial spreading
tumor. Its margins are irregular and frequently
ulcerate and bleed.
Valvectomy and bilateral lymphadectomyis the
treatment of choice.
Post operative radiotherapy may be required.
Prognosis is very poor.
59.
60. Secondary growth of the
Vulva.
It occurs following metastasis from chorio
carcinoma, Endometrial and Ovarian cancer.
Treated by radiotherapy and chemotherapy .
50% recurrent growths are seen after 2years of
Rx of primary lesion. More common when
primary lesion was large and Lymph Nodes were
involved.
RX– exenteration ,radiotherapy and
chemotherapy.
Recurrent growth occur due to incmplete
excision.
61. Persistent Residual Cancer
Persistent residual cancer is one which
develops with in 6 months of primary
treatment.
Local excision with wide margin is required.
62.
63. Prognostic factors
Prognosis depends over size of tumor,
grading, staging, how much excision is being
done , inguinal and/ pelvic LN positivity.
Immune status of woman
Age and operability of patient.
Hystolgical type of tumor.