Cancer vulvaCancer vulva

MOUNIR M F ELHAO,MOUNIR M F ELHAO,PROF OF OB &GYN. AIN SHAMS PROF OF OB &GYN. AIN SHAMS

UNIVERSITY,GYNEONCOLOGY UNIT.UNIVERSITY,GYNEONCOLOGY UNIT.

HISTORICAL.HISTORICAL.

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

INCIDENCE.INCIDENCE.

Relatively rare, accounting for Relatively rare, accounting for about about 3 to 3 to 5%5% of all gynecologic malignancies. of all gynecologic malignancies.

INCIDENCE.INCIDENCE.

FourthFourth most common malignancy of most common malignancy of the female genital tractthe female genital tract

INCIDENCE.INCIDENCE.

As the As the 6th , 7th decade6th , 7th decade of life of life and does and does increase with increasing age.increase with increasing age.

INCIDENCE.INCIDENCE.

very high association with the high risk very high association with the high risk HPV serotypesHPV serotypes, specifically type , specifically type 16 16 and and 1818..

PREDISPOSING FACTORS.PREDISPOSING FACTORS.

any chronic inflammatory conditionany chronic inflammatory condition, , herpes has been implicated, obesity, herpes has been implicated, obesity, diabetes, hypertension, prior diabetes, hypertension, prior squamous cell carcinoma of the cervix, squamous cell carcinoma of the cervix, vagina or the anal rectal area as well, vagina or the anal rectal area as well, and then vulvar dystrophy probably and then vulvar dystrophy probably increases a woman’s risk.increases a woman’s risk.

ASSOCIATIONS.ASSOCIATIONS.

associated vulvar dystrophies, they may associated vulvar dystrophies, they may even have vulvar intraepithelial neoplasia, even have vulvar intraepithelial neoplasia, they often times are incontinent they often times are incontinent there is there is often a often a delay of 2 to16delay of 2 to16 months between months between the onset of symptoms and the initial the onset of symptoms and the initial presentation to the physicianpresentation to the physician

SYMPTOMS.SYMPTOMS.

Symptoms include chronicSymptoms include chronic pruritus pruritus, a , a lump or masslump or mass, pain, bleeding , pain, bleeding ulceration, dysuria and leg edemaulceration, dysuria and leg edema

FACT:SURVIVALFACT:SURVIVAL

Survival rates in most series relates to Survival rates in most series relates to nodal involvementnodal involvement..

Intraepithelial lesions.Intraepithelial lesions.

are treated a number of different ways, are treated a number of different ways, it depends on the site, the age of the it depends on the site, the age of the patient and the size of the lesion. It is patient and the size of the lesion. It is proper to excise with a 3 to 4 mm proper to excise with a 3 to 4 mm margin and then primarily close the margin and then primarily close the area.area.

In order to make a diagnosisIn order to make a diagnosis

you you need to need to get tissue, and wedge get tissue, and wedge biopsy,biopsy, excisional biopsies, excisional biopsies, colposcopycolposcopy,, it’s very important to it’s very important to remember that you have to examine the remember that you have to examine the remainder of the genital tract looking for remainder of the genital tract looking for vaginal lesions and also for cervical vaginal lesions and also for cervical dysplasia or early invasive cancer dysplasia or early invasive cancer because often times these can be because often times these can be metastatic from another site, metastatic from another site,

Squamous cell carcinomaSquamous cell carcinoma

is the most common followed by is the most common followed by melanomasmelanomas, about just under 6% of the , about just under 6% of the time, time, BartholinBartholin gland cancers gland cancers are third are third, , basal cell carcinomabasal cell carcinoma, you see some , you see some sarcomassarcomas, you can very rarely see , you can very rarely see invasive invasive Paget’s diseasePaget’s disease..

SITES.SITES.

The most frequent sites are on the The most frequent sites are on the labialabia majusmajus, followed by the , followed by the labium labium minorumminorum, and then some patient’s will , and then some patient’s will have have combined combined lesions about 15%.lesions about 15%.

FACT.FACT.

the demarcation for micro invasion is the demarcation for micro invasion is actually 1 mm or less.actually 1 mm or less.

AnatomyAnatomy

The The superficial inguinal lymph nodessuperficial inguinal lymph nodes lie along the lie along the saphenous vein, deep to Camper's fascia and superficial saphenous vein, deep to Camper's fascia and superficial to the cribriform fascia which overlies the to the cribriform fascia which overlies the femoral vessels. They are found in the triangle bounded . They are found in the triangle bounded by the inguinal ligament superiorly, the border of the by the inguinal ligament superiorly, the border of the sartorius muscle laterally, and the adductor longus laterally, and the adductor longus muscle medially. There are appoximately 10 superficial muscle medially. There are appoximately 10 superficial lymph nodes. lymph nodes.

The The deep inguinal lymph nodesdeep inguinal lymph nodes are located medial to are located medial to the femoral vein and under the cribriform fascia. There the femoral vein and under the cribriform fascia. There are approximately 3 to 5 deep nodes. The superior-most are approximately 3 to 5 deep nodes. The superior-most node is located under the inguinal ligament and is called node is located under the inguinal ligament and is called Cloquet's node

DrainageDrainage

The The superficial inguinal lymph nodessuperficial inguinal lymph nodes receive drainage from the receive drainage from the vulva and and anus. The superficial nodes drain to . The superficial nodes drain to the the deep inguinaldeep inguinal lymph nodes, which lymph nodes, which then drain superiorly to the then drain superiorly to the external external iliaciliac lymph nodes, then to the lymph nodes, then to the pelvic pelvic lymph nodeslymph nodes and to the and to the paraaorticparaaortic lymph nodes.lymph nodes.

The TNM staging system is used.The TNM staging system is used.

T-0 T-0 Pre-malignant change. Pre-malignant change. T-1A T-1A A cancer less than 2.0cm in diameter and less than 1.0mm in A cancer less than 2.0cm in diameter and less than 1.0mm in

depth of invasion. depth of invasion. T-1BT-1B A cancer less than 2.0cm in diameter but greater than 1.0mm in A cancer less than 2.0cm in diameter but greater than 1.0mm in

invasion. invasion. T-2 T-2 Greater than 2.0cm in diameter. Greater than 2.0cm in diameter. T-3T-3 Involves vagina, urethra or anus. Involves vagina, urethra or anus. T-4 T-4 Involves bladder, rectum or pelvic bone. Involves bladder, rectum or pelvic bone. N-0 N-0 No lymph nodes involved No lymph nodes involved. N-1 . N-1 Lymph node metastases to one groin. Lymph node metastases to one groin. N-2 N-2 Lymph node metastases to both groins. Lymph node metastases to both groins. M-0 M-0 No distant metastases. No distant metastases. M-1 M-1 Any distant metastases. Any distant metastases.

The standard treatmentThe standard treatment( ( Hallstedian conceptHallstedian concept ) )

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

Why conservative surgery?Why conservative surgery?

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

FACT: FACT: Current place of pelvic Current place of pelvic lymphadenectomy?lymphadenectomy?

No patients with negative groin nodesNo patients with negative groin nodes

Had positive pelvic nodes.Had positive pelvic nodes.

Positive bilateral groin nodes five year Positive bilateral groin nodes five year survival <20 %survival <20 %

We omitted routine We omitted routine pelvic lymphadenectomypelvic lymphadenectomy,,

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

Role of adjuvent radiotherapy?Role of adjuvent radiotherapy?

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

Should we do separate groin Should we do separate groin incisionsincisions ??

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

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

Is there a place for unilateral Is there a place for unilateral inguinofemoral lymphadenectomy?inguinofemoral lymphadenectomy?

May be indicated in well lateralised early May be indicated in well lateralised early tumors.tumors.

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

What is the place of superficial What is the place of superficial inguinal lymphadenectomy?inguinal lymphadenectomy?

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

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

sectionsection..

Can we omit groin node dissection Can we omit groin node dissection in superficial diseasesin superficial diseases??

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

we do give postoperative radiation for we do give postoperative radiation for groin nodal metastasesgroin nodal metastases

Is there a place for preoperative Is there a place for preoperative radiotherapy?radiotherapy?

we give preop radiation therapy for we give preop radiation therapy for advanced disease.advanced disease.

Conclusions. Conclusions. Now to run through management, Now to run through management,

againagain

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

CONCLUSIONS.CONCLUSIONS.

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

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

Conclusions.Conclusions.

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

Conclusions.Conclusions.

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

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

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

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

CONCLUSIONS.CONCLUSIONS.

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

Conclusions.Conclusions. The Cochrane Database of Systematic ReviewsThe Cochrane Database of Systematic Reviews 2006 Issue 1 2006 Issue 1

Copyright © Copyright © 2006 The Cochrane Collaboration. Published by John Wiley & 2006 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.Sons, Ltd.

Surgical interventions for early squamous cell carcinoma of the vulvaSurgical interventions for early squamous cell carcinoma of the vulva Ansink A, van der Velden J, Collingwood MAnsink A, van der Velden J, Collingwood M Plain language summaryPlain language summary Less extensive surgery for vulvar cancer appears Less extensive surgery for vulvar cancer appears

safe and limits mutilationsafe and limits mutilation Vulvar cancer is rare, affecting mainly older women. Until the 1980s, Vulvar cancer is rare, affecting mainly older women. Until the 1980s,

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

CONCLUSIONS.CONCLUSIONS. TheCochraneLibrary Cochrane review abstract and Cochrane review abstract and

plain language summaryplain language summary This is an abstract and plain language summary of a regularly updated, systematic review This is an abstract and plain language summary of a regularly updated, systematic review

prepared and maintained by The Cochrane Collaboration. The full text of the review is available prepared and maintained by The Cochrane Collaboration. The full text of the review is available in in The Cochrane Library (ISSN 1464-780X). (ISSN 1464-780X).

The Cochrane Database of Systematic ReviewsThe Cochrane Database of Systematic Reviews 2006 Issue 1 2006 Issue 1Copyright © 2006 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd. Copyright © 2006 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.

Primary groin irradiation vs primary groin surgery for early Primary groin irradiation vs primary groin surgery for early vulvar cancervulvar cancer

van der Velden J, Ansink Avan der Velden J, Ansink A Plain language summaryPlain language summary Insufficient evidence that radiotherapy works as well as surgery for vulvar cancer. Insufficient evidence that radiotherapy works as well as surgery for vulvar cancer. Cancer of the vulva is mainly a disease of elderly women. Surgery involves removal of the Cancer of the vulva is mainly a disease of elderly women. Surgery involves removal of the

tumour and surrounding lymph nodes, occasionally followed by radiotherapy. Although survival tumour and surrounding lymph nodes, occasionally followed by radiotherapy. Although survival rates are high if the tumour is found early enough, removal of the lymph nodes causes swelling, rates are high if the tumour is found early enough, removal of the lymph nodes causes swelling, particularly in the legs. Wound healing and sexual problems are also common. particularly in the legs. Wound healing and sexual problems are also common. While While radiotherapy is effective in the short term, there is not enough evidence from radiotherapy is effective in the short term, there is not enough evidence from trials to show that it is as effective as surgery in preventing tumour regrowth trials to show that it is as effective as surgery in preventing tumour regrowth in the groins.in the groins.

What is the place of modified What is the place of modified radical vulvectomy?radical vulvectomy?

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

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

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

How should we treat vulvar How should we treat vulvar carcinoma with perianal carcinoma with perianal

involvement?involvement?The main problem in these cases is to do The main problem in these cases is to do

adequate resection with maintaining adequate resection with maintaining sphincteric function.sometimessphincteric function.sometimes

we may need to do more radical resection we may need to do more radical resection and colostomy orand colostomy or

preoperative radiotherapy.preoperative radiotherapy.

what is the place of ultraradical what is the place of ultraradical surgery?surgery?

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

what is the place of neoadjuvent what is the place of neoadjuvent chemotherapy?chemotherapy?

Resuts are not encouraging for time being.Resuts are not encouraging for time being.

CONCLUSION.CONCLUSION.

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

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

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

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

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

more than one groin nodesis positive for more than one groin nodesis positive for metestatic disease.metestatic disease.

6.6.ultraradical surgery selective .ultraradical surgery selective .

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