Cancer vulva recapitulating the FACTS

Dr. Sunaina wadhwa Dr. Pratima mittal Department of obstetrics &

gynecology Vmmc & sjh.

Objectives of this presentation

To know the out lines of etiology,diagnosis and mangment of cancer vulva.

To understand the importance of early dectection of cancer vulva to improve prognosis and survival rate

Introduction Vulval cancer is uncommon & accounts for

approximately 1-4% of all gynecological cancer 4th most common malignancy of the female

genital tract

Incidence : 1.8 /100.000, It is predominantly seen in postmenopausal and old women (mean age 65 years ) ,and only 2% were less than 30 years.

As the 6th , 7th decade of life and does increase with increasing age.

- The incidence increases with age.

- Recently there was a rise in the incidence, due to

Longevity (Long standing preinvasive stage)

Increased HPV infections. Increased smoking habits

Risk factors for carcinoma of the vulva

1- Human papillomavirus infection. Genital condylomas: these are detected in 5 % of vulvar cancer. Vulvar intraepithelial neoplasia (VIN) and also CIN. 2- Medical history of: Vulvar dystrophy. Chronic vulvar pruritus. 3- Patients with a history of squamous cell ca of cervix or vagina. 4- Chronic immunosuppression.

5- Smoking

2 Types / Variants

(15%) (85%)

(90%)

(2-3%)

(5%)

PATHOLOGY

Primary Tumor90% of lesions are of squamous in origin.3-5 of lesions are melanoma.2% of lesions is basal cell carcinoma.Less than 1% is sarcoma.

Secondary TumorsIt is occasionly found in vulva

Most commonly the primary lesion is from the cervix or the endometrium .

Essentials of DiagnosisTypically occurs in postmenopausal

women. Long history of vulvar irritation with pruritus,

local discomfort, and bloody discharge. Appearance of early lesions like that of

chronic vulvar dermatitis. Appearance of late lesions like that of a

large cauliflower, or a hard ulcerated area in the vulva.

Biopsy necessary for diagnosis.

Clinical Features & Diagnosis

Most patients with invasive disease complain of:

Irritation or purities in 70% of cases

Vulvar mass or ulcer in 55% of cases

Bleeding in 28% of cases

Discharge in 2-3% of cases

The major problem in invasive vulvar cancer is delay between the first appearance of the symptoms and referral to the gynecological opinion due to :

1. The doctor fails to recognize the gravity of the lesion and prescribes topical therapy.

2. Older women are often embarrassed and shy.

Diagnosis1) Local examination of the relevant areas: early lesions appears as

chronic vulvar dermatitis. 2) Clinical assessment of the lymph nodes is to be performed in the

relevant regions. 3) Biopsy: 1- From the suspected lesions: a) Dermal punch biopsy using a local anesthetic: Lesions

< 1 cm b) Excisional biopsy under general anesthesia: Lesions

> 1 cm: 2- From the lymph nodes in the relevant regions when

suspected for metastasis. Differential diagnosis: 1- Venereal diseases: syphilis, chancroid, lymphogranuloma venereum,

granuloma inguinale.2- VIN. An association between invasive and noninvasive lesions is a

possibility. 3- Condyloma acuminatum.

In order to make a diagnosis

you need to get tissue, and wedge biopsy, excisional biopsies, colposcopy,

It’s very important to remember that you have to examine the remainder of the genital tract looking for vaginal lesions and also for cervical dysplasia or early invasive cancer because often times these can be metastatic from another site,

Squamous Cell carcinoma

65% arises in labia majora and minora25% percent in clitoris or perineum Appearance varies from a large,

exophytic, cauliflowerlike lesion to a small ulcer crater superimposed on a dystrophic lesion of the vulvar skin

primary determinant of nodal metastases is tumor size.

SITES.The most frequent sites are on the labia

majus, followed by the labium minorum, and then some patient’s will have combined lesions about 15%.

SQUAMOUS CELL CARCINOMAAre usually seen in the anterior part

of the vulva.2/3 of cases in the labia majora.1/3 of cases in the clitoris ,labia minora,fourchitte, and perineum.

Spread:-1. LYMPHATIC > 50%2. Direct spread occurs in 25% to the

urethra, vagina and rectum3. Hematogenous spread to bone or

lung is rare The lymph nodes are arranged in 5

groups in each groin:

Lt sided lesion will spread to the Lt groin Lymph node.Rt sided lesion will spread to the Rt Groin Lymph node.Bilateral nodes involvement is seen in 14% of cases.Contralateral node involvement without ipsilateral disease is seen in 5% of cases.

Never found pelvic nodes to be involved in the absence of inguinal nodes metastases.

External Iliac Nodes

Common Iliac Nodes

Para Aortic L.N

Thoracic Duct

STAGING:FIGO suggest clinical staging in 1969 based on TNM (Tumor node metastasis) classification taking into consideration:The size of the local lesion.Groin node involvement.Metastases.

A new FIGO staging based on surgical findings in 1988, which underwent revision in 2009 to provide better prognostic discrimination b/w stages and less hetrogeneityThe main changes are:While stage 1a remains unchanged as s only group of patients with negligible risk of ln involvment , former stage 1 and 11 have been combined to 1b

The new stage 11 segregates pts whose tumors involve the lower adjacent perineal structure from those with positive ln

For stages iii and iv the no. and morphology of the involved nodes are taken into account and the bilaterality of lymphnodes is discounted

T N M STAGING T-0 pre-malignant change T-1

A a cancer less than 2.0cm in diameter and less than 1.0mm in depth of invasion

B a cancer less than 2.0cm in diameter but greater than 1.0mm in invasion

T-2 greater than 2.0 centimeters in diameter T-3 involves vagina, urethra or anus T-4 involves bladder, rectum or pelvic bone N-0 no lymph

nodes involved N-1 lymph node metastases to one groin

N-2 lymph node metastases to both groins M-1 any distant metastases M-0 no distant metastases

Stage I and II Stage III

Stage IV

PROGNOSISThe overall 5 years survival rate for

vulval cancer is 70% for all operable cases,

This depends on:1. L.N Involvement:

This is the most prognostic factorMetstatic involvement of groin nodes decreases the 5 years survival rate to below 50% as opposed to the 90% when L.N are not involved.Once pelvic nodes are involved the 5 years survival rate is 15%.

2. The number of groin nodes involvement:microscopical involvement of N.regardless of stage has a good prognosis.2 or more positive nodes have a worse prognosis.

3. Stage:The 5 years survival rate decreases with advancing stage from >90% in stage 1 to < 10% in stage 4.

4. Differentiation:A well diff.tumor has a better prognosis than poor diff.

5.Depth of Invasion:A-invasion of 1 mm no risk of nodal metastases.B-invasion of 1-3 mm 6-8% incidence of metastases.C-invasion of 5 mm 22-37% incidence of metastases.6.Surgical Margin:Surgical excision margin of more than 1 cm in all diameters results in a low local recurrence rate.

Prophylaxis…A high index of suspicion

Detection and management of VIN. Proper management of all cases with

pruiritus vulvae. All vulval lesions should be diagnosed

accurately especially those arising after menopause.

All pigmented vulvar lesions should be removed for biopsy.

Advances in managment of vulvar ca

Individualization of treatment for all patients with invasive disease

Vulvar conservation for patients with unifocal tumor and an otherwise normal vula

Omission of groin dissection for patients with microinvasive tumor(t1a<2cm diameter and <1mm stromal invasion

Elimination of routine pelvic lymphedenectomy Investigation of role of sentinal ln procedure to

eliminate complete inguinofemoral lyphedenectomy

Use of separate incision for groin dissection to improve wound healing

Omission of contralateral groin dissection in pts with lateral t1 lesion and negative ipsilateral lnodes

Use of preoperative radiotherapy to obviate the need for exenteration in pts with advanced disease

Use of post operative rt to decrese the incidence of groin recurrence with multiple groin nodes

SURGURY:The standard surgery is enblock radical vulvectomy and bilateral groin nodes dissection as described by Taussing and way (three separate incision). This associated with:High incidence of morbidity (wound infection, necrosis and break down , pul. Embolism, and lymphoedema).Problems with body image and sexual function.

The recent trend in management is not to cure patients but to preserve body image and sexual function by performing less radical surgery .The individualization of the treatment depends on:Size and position of tumor.Depth of invasion.The age and performance status of the patient.

Treatment Options by Stage

Treatment Option StagePartial Vulvectomy excision of the tumor, with a 1 cm safe margins. No need for node removal.

Ia

IbModified radical vulvectomy with either of the following: 1) Ipsilateral groin lymph node dissection: in cases of lateralized lesion 2) Bilateral groin node dissection: in cases of centralized lesions

Modified radical vulvectomy with bilateral groin node dissection. II- Combined approach: 1- Preoperative external beam radiation therapy. 2- Chemotherapy (e.g. 5-fluorouracil, cisplatin). 3- Radical excision with bilateral inguinal & femoral node dissection. 4- Preoperative RT, then surgical excision of the tumor. - Pelvic exenteration.

III

Individualized IV

HISTORICAL.

The surgical treatment, back in the early 1900s Basset from France who adopted a Hallstedian concept to the treatment of vulvar cancer very similar what Dr. Hallstead had adopted for breast cancer, felt that wide surgical excision was the best.

The standard treatment( Hallstedian concept )

, was block radical vulvectomy with bilateral inguinal femoral lymphadenectomies and we did selective pelvic lymphadenectomies through separate extra peritoneal incisions and this basically is what has been called the butterfly incision or the Texas longhorn incision.

Why conservative surgery?

The rationale for conservative surgery is that most of the metastases occur by embolization and the early advocates of the more conservative procedures in their series found no metastatic lesions in the skin bridge between the vulva and the groin,

Vulvectomy There are several operations in which part of the

vulva or all of the vulva is removed: A skinning vulvectomy means only the top layer of skin

affected by the cancer is removed. Although this is an option for treating extensive VIN3, this operation is rarely done.

Simple vulvectomy, the entire vulva is removed. Radical vulvectomy can be complete or partial.

When part of the vulva, including the deep tissue, is removed, the operation is called a partial vulvectomy.

In a complete radical vulvectomy, the entire vulva and deep tissues, including the clitoris, are removed.

An operation to remove the lymph nodes near the vulva is called a en block dissection. It is important to remove these lymph nodes if they contain cancer.

Skinning / Simple Vulvectomy

Radiotherapy Malignant diseases of the vulva are not commonly managed

by RT because of the intolerance of surrounding normal tissues. Chemotherapy as radiation sensitizer can improve response of the

malignant tissues. Indications of RT in malignant diseases of the vulva:

Preoperative RT in stage III and IV: The lesion shrunk and it limits the need for pelvic exenteration. It also improves surgical respectability of tumors.

Postoperative RT: can reduce regional recurrences and inguinal lymph node metastases.

Multiple positive groin nodes: It decreases the incidence of recurrence. Positive surgical margins as seen on microscopic examination. Multiple focal recurrences. When the tumor size is > 4 cm

metastasis

Primarily lymphatics to the superficial inguinal lymph nodes

Direct extension to vagina, urethra and anus

Malignant Melanoma

Accounts for 5% of vulvar cancersmost commonly arises in the labia minora

and clitorissuperficial spread toward the urethra and

vaginanonpigmented melanoma may closely

resembles squamous cell carcinomadarkly pigmented, raised lesion is a

characteristic finding

Colposcopy images after application of acetowhite 5% and toluidine blue test

FACT: Current place of pelvic lymphadenectomy?

In the past pelvic lymphedenectomy was routinely done now it has been established that patients with negative groin nodes rarely have positive pelvic nodes.

pts with >3 positive groin nodes are prone to pelvic ln involvement

We omitted routine pelvic lymphadenectomy,

patient’s who have positive nodes, , end up getting radiation therapy to the whole pelvis anywhay.

Role of adjuvent radiotherapy?

Review of recurrence studies of in Homesley,s study suggests that adjuvent RT is more effective largely because groin recurrences are reduced.

Should we do separate groin incisions ?

Understanding that the mode ofmetastatic spread is embolic rather than by contiguous grouth allowed for three-incision technique..

Less morbidity.No impact on survival.(54% BREAKDOWN RATE WITH BUTTERFLY TECH.)

Is there a place for unilateral inguinofemoral lymphadenectomy?May be indicated in well lateralised early

tumors.No lymph-capillary space involvement.Negative groin nodes by frozen section.

What is the place of superficial inguinal lymphadenectomy?

Above the cribriform fascia , mainly those associated with great saphenous and superficial epigastric veins.

ONLY with low risk for LN metastasis.Tumors confined to labia majora.Negative superficial nodes on frozen

section.

Can we omit groin node dissection in superficial diseases?

Stage 1a have <1% for groin node metastasis.

we do give postoperative radiation for groin nodal metastases

Is there a place for preoperative radiotherapy?

we give preop radiation therapy for advanced disease.

Conclusions. Now to run through management,

again

for stage I, it’s pretty much radical local excision, and try to maintain at least a 1 cm margin and if it’s truly a small lesion with less than a mm invasion, it is felt that most of those patient’s do not need to have lymph nodes removed.

CONCLUSIONS.

For large stage II lesions, again, depending on where it’s located, we do a radical vulvectomy and bilateral inguinal femoral lymphadenectomy, if there are more than two lymph nodes positive, the patient’s will get postoperative whole pelvic radiation.

For stage III tumors, it depends on what’s involved, you can do a radical excision which often times becomes extended and you have to take the distal vagina and even sometimes the distal urethra and if you are going to treat it surgically it needs to be combined with the bilateral inguinal femoral lymphadenectomy and again, if there is lymph node involvement POST OPERATIVE RADIOTHERAPY.

Conclusions.

For advanced disease, again you have to individualize, add up with surgical clearance for disease sometimes involves the anus, rectum, proximal urethra and requires an exenterative procedure with radical vulvectomy and bilateral groin nodes and that particular circumstance is very important that patient’s are evaluated either with MRI, CAT scans and possibly even a PET scan for metastatic disease prior to undertaking such a large procedure. The operative mortality is about 5 to 10%.

Conclusions.

Survival is also determined whether or not the nodes are positive or negative, and by which nodes are involved.

If patient’s have negative groin nodes, the five year survival is 90% and that’s for stage I and stage II.

If they have positive groin nodes, survival drops about 57%.

If they have positive pelvic lymph nodes, it drops to 20%. Unilateral positive groin nodes is about 70% five year survival, bilateral positive groin nodes, however, drops down to 25% five year survival, and then the increasing number of positive nodes.

CONCLUSIONS.

and also the tumor diameter affects nodal involvement, lymphatic vascular space involvement and then overall survival.

Conclusions. . Less extensive surgery for vulvar cancer appears safe and

limits mutilation

Vulvar cancer is rare, affecting mainly older women. Until the 1980s, affected women underwent extensive, mutilating surgery. Groin nodes on both sides as well as all vulvar tissue were removed. Recently surgeons have carried out a smaller operation, leaving as much vulvar tissue as possible behind. No randomized controlled trials have been conducted on the safety of this reduced surgery, but from the available evidence it appears to be safe to perform this smaller operation in most patients.

The Cochrane Database of Systematic Reviews 2006 Issue 1Copyright © 2006 The Cochrane Collaboration. Published by John Wiley & Sons, LtdSurgical interventions for early squamous cell carcinoma of the vulva

Ansink A, van der Velden J, Collingwood M

What is the place of modified radical vulvectomy?

main morbidity of radical vulvectomy is sexual dysfunction and compromised function of the anus and urethra.

The main fear of about the modified operation is the multicentricity of the tumor.(20-30%).

So reservethe operation to well localised tumors,with 2 cm free margin.

How should we treat vulvar carcinoma with perianal

involvement?The main problem in these cases is to do

adequate resection with maintaining sphincteric function.sometimes

we may need to do more radical resection and colostomy or

preoperative radiotherapy.

what is the place of ultraradical surgery?

Only in patients with clearly resectable lesions and negative or one or two microscopicaly positive nodes.

what is the place of neoadjuvent chemotherapy?

Resuts are not encouraging for time being.

CONCLUSION

1.Standard radical vulvectomy and bilateral lymphadenectomy(Hallstedian concept.)has compromised the life of many women with cancer vulva.

2.In many well selected patients wide excision with 2 cm margin with or without node selection may suffice.

3.modified radical vulvectomy with bilateral groin node dissection will give equaly good results in the majority of cases

4.Pelvic lymphadenectomy should be abondoned except in a minority of selected cases.

5.Radiotherapy should be given to the groins and pelvis postoperatively only if

more than one groin nodesis positive for metestatic disease.

6.ultraradical surgery selective

7.In situ stage is almost 100% curable.and FIGO stage 1 disease is 90% curable and 5 year survival rate.

Take home messageAny patient with persistence

itching or vulval lesion not responds to simple treatment , you should take multiple biopsies from vulva to exclude malignancy.

In management of cancer vulva, age group, psychology of patient, and the appearance of the vulva should be taken in account as this will change the plan of management of cancer.

Plastic surgery should play role in the future.

In future infrared , and laser therapy under microscopy will play role in the management of premalignant lesions.