MANAGEMENT OF MANAGEMENT OF CARCINOMA OF THE CARCINOMA OF THE

VULVAVULVABY DR. D.O ALLAGOABY DR. D.O ALLAGOA

CLINICAL FEATURESCLINICAL FEATURES

INVESTIGATIONSINVESTIGATIONS

TREATMENTTREATMENT

COMPLICATIONSCOMPLICATIONS

CONCLUSIONCONCLUSION

CLINICAL FEATURESCLINICAL FEATURESPatients usually > 60 years old, may be youngerPatients usually > 60 years old, may be youngerSmall lesions may be asymptomaticSmall lesions may be asymptomaticMost patients present late with 1/3 of the women Most patients present late with 1/3 of the women presenting with stage III or IV disease.presenting with stage III or IV disease.Patients present an average of 6-12 monthsPatients present an average of 6-12 months+ from onset of symptoms. They present late due to + from onset of symptoms. They present late due to embarrassment, fear or ignorance.embarrassment, fear or ignorance.Delay in diagnosis may be due to Delay in diagnosis may be due to the non-specific nature of symptoms,the non-specific nature of symptoms, rarity of vulvar Ca compared with non-rarity of vulvar Ca compared with non-

malignant lesions malignant lesions delay in clinical examination by primary carers delay in clinical examination by primary carers

due to other medical problems the patients due to other medical problems the patients may havemay have

PRESENTING SYMPTOMSPRESENTING SYMPTOMS

PruritusPruritus 71%71%

Vulvar lump or swellingVulvar lump or swelling 58%58%

Vulvar ulcerationVulvar ulceration 28%28%

(Typical raised ulcer with rolled edges)(Typical raised ulcer with rolled edges)

BleedingBleeding 26%26%

Pain or SorenessPain or Soreness 23%23%

Urinary tract symptomsUrinary tract symptoms 14%14%

DischargeDischarge 13%13%

PRESENTING SYMPTOMSPRESENTING SYMPTOMSThe lesions may be:The lesions may be:-Ulcerated.-Ulcerated.-Raised.-Raised.-Pigmented.-Pigmented.- warty appearance.- warty appearance.IN ORDER OF APPEARANCE IN ORDER OF APPEARANCE the lesions appear mainly on ;the lesions appear mainly on ;- labia majora. (67%)- labia majora. (67%)- labia minora.- labia minora.- clitoris.- clitoris.- perineum.- perineum.- posterior fourchette.- posterior fourchette.The definitive diagnosis depends on biopsy under The definitive diagnosis depends on biopsy under anaesthesia.anaesthesia.

INVESTIGATIONSINVESTIGATIONS

1.1. Confirmation of diagnosisConfirmation of diagnosis

2.2. Staging the diseaseStaging the disease

3.3. Assess the patients fitnessAssess the patients fitness

4.4. Assess the possibility of concurrent Assess the possibility of concurrent disease that might influence disease that might influence management.management.

INVESTIGATIONS TO CONFIRM INVESTIGATIONS TO CONFIRM DIAGNOSIS/STAGE THE DISEASEDIAGNOSIS/STAGE THE DISEASEIncisional biopsyIncisional biopsyExcisional biopsy (for local lesions)Excisional biopsy (for local lesions)Colposcopy is useful for diagnosis of smaller Colposcopy is useful for diagnosis of smaller

superficial lesions which are clinically difficult to superficial lesions which are clinically difficult to recognize. Colposcopy helps map out areas of recognize. Colposcopy helps map out areas of invasive disease & plan management. invasive disease & plan management.

-5% Acetic acid will help to identify areas to biopsy -5% Acetic acid will help to identify areas to biopsy although subtle changes may be difficult to although subtle changes may be difficult to recognizerecognize

-Toluidine blue can also be used.-Toluidine blue can also be used.Cystoscopy/Proctoscopy (bladder/rectal mucosa Cystoscopy/Proctoscopy (bladder/rectal mucosa

involvement)involvement)

INVESTIGATIONS TO STAGE THE DISEASE (CONT.)INVESTIGATIONS TO STAGE THE DISEASE (CONT.)

Accurate assessment of inguinofemoral lymph node status is the Accurate assessment of inguinofemoral lymph node status is the most important feature for staging and management planning. most important feature for staging and management planning.

There are limitations to clinical assessment of nodal status. It is There are limitations to clinical assessment of nodal status. It is notoriously unreliable. Clinical suspicion of nodal involvement notoriously unreliable. Clinical suspicion of nodal involvement may be evaluated with may be evaluated with

Fine-needle aspiration – Fine-needle aspiration – • ––ve result does not exclude need for node dissection. ve result does not exclude need for node dissection. • +ve result may prompt neoadjuvant radiation+ve result may prompt neoadjuvant radiation

LymphangiographyLymphangiography Ultrasonography (including Doppler flow study)Ultrasonography (including Doppler flow study) Magnetic resonance imaging (MRI)Magnetic resonance imaging (MRI) Computed tomography (CT)Computed tomography (CT)Combination of imaging techniques & fine-needle aspiration Combination of imaging techniques & fine-needle aspiration

improves sensitivityimproves sensitivity

INVESTIGATIONS TO ASSESS FITNESS & INVESTIGATIONS TO ASSESS FITNESS & POSSIBLE CONCURRENT DISEASEPOSSIBLE CONCURRENT DISEASE

Full blood countFull blood count

UrinalysisUrinalysis

Serum electrolytes/ureaSerum electrolytes/urea

Chest radiograph, Intravenous UrographyChest radiograph, Intravenous Urography

ECG ECG

Assessment and possible biopsy of vagina Assessment and possible biopsy of vagina & cervix to detect co-existing VAIN & CIN& cervix to detect co-existing VAIN & CIN

Investigation- contInvestigation- cont

VulvoscopyVulvoscopy

CystoscopyCystoscopy

ProctoscopyProctoscopy

PyelographyPyelography

Barium enemaBarium enema

TREATMENTTREATMENT

It is a rare tumour hence current guidelines It is a rare tumour hence current guidelines recommend that it is managed in a cancer centre recommend that it is managed in a cancer centre within the context of a multidisciplinary team of within the context of a multidisciplinary team of experts led by a specialist gynaecological experts led by a specialist gynaecological oncologistoncologist

-SURGERY-SURGERY-CHEMOTHERAPY-CHEMOTHERAPY

Neoadjuvant chemotherapyNeoadjuvant chemotherapy-RADIOTHERAPY-RADIOTHERAPY

SURGICAL MANAGEMENTSURGICAL MANAGEMENT

The objective of surgery is to remove sufficient The objective of surgery is to remove sufficient tissue to prevent local recurrence as well as tissue to prevent local recurrence as well as excise groin nodes both to stage the disease excise groin nodes both to stage the disease and to prevent groin recurrenceand to prevent groin recurrenceTraditional surgery has been a large en bloc resection of the vulva with the superficial and deep inguinal nodes through a single incision with at least 2-cm margins around the tumor and deep resection to the genitourinary diaphragmThe morbidity associated with radical The morbidity associated with radical vulvectomy has led to changes in management vulvectomy has led to changes in management

REFINEMENTS TO SURGERYREFINEMENTS TO SURGERY

(1)(1) Ipsilateral inguinal node dissection in early tumorsIpsilateral inguinal node dissection in early tumors(2)(2) Using a triple-incision technique instead of an en bloc Using a triple-incision technique instead of an en bloc

approachapproach(3)(3) Using radical local resection with 1-cm margins instead Using radical local resection with 1-cm margins instead

of complete vulvectomyof complete vulvectomy(4)(4) Sparing the saphenous vein in an attempt to prevent Sparing the saphenous vein in an attempt to prevent

lymphedemalymphedema(5)(5) Omitting deep node dissectionOmitting deep node dissection(6)(6) Radiation alone for nodal involvement Vs groin node Radiation alone for nodal involvement Vs groin node

dissectiondissection(7)(7) Sentinel lymph node dissectionSentinel lymph node dissection

PRINCIPLES OF SURGERYPRINCIPLES OF SURGERY

Maximum individualisationMaximum individualisationCautious conservationCautious conservationRestoration by plastic reconstructionRestoration by plastic reconstructionChoice of surgery will depend onChoice of surgery will depend onAge & general state of the patientAge & general state of the patientSize, site and stage of the lesionSize, site and stage of the lesionHistology of the lesionHistology of the lesionClinical status & histology of groin lymph nodesClinical status & histology of groin lymph nodesCondition of uninvolved vulvar/anal skin Condition of uninvolved vulvar/anal skin

Disease categorizationDisease categorization

EARLY DISEASEEARLY DISEASE

- Small unifocal lesions with no clinical - Small unifocal lesions with no clinical evidence of nodal involvementevidence of nodal involvement

LATE DISEASELATE DISEASE

-Advanced vulvar disease and/ or clinical -Advanced vulvar disease and/ or clinical evidence of nodal involvementevidence of nodal involvement

SURGERYSURGERY

Wide local excision for Stage I & IIWide local excision for Stage I & IIHemivulvectomy & ipsilateral lymph node excision Hemivulvectomy & ipsilateral lymph node excision for lateral lesions.for lateral lesions.- if lesion 2cm or less- ipsilateral lymph - if lesion 2cm or less- ipsilateral lymph adenectomy is appropriateadenectomy is appropriate-contralateral dissection done once ipsilateral -contralateral dissection done once ipsilateral node is positive.node is positive.-lesion > 2cm bilateral node dissection is advised-lesion > 2cm bilateral node dissection is advisedRadical vulvectomy - triple incision or enbloc Radical vulvectomy - triple incision or enbloc dissection for midline lesionsdissection for midline lesionsPelvic exenteration for advanced cancer.Pelvic exenteration for advanced cancer.(dehumanising)(dehumanising)

Radical vulvectomyRadical vulvectomyEn blocEn bloc

significant morbiditysignificant morbidity

Triple incision Triple incision

- better cosmetic outcome- better cosmetic outcome

-better survival rate-better survival rate

- reduced local relapse- reduced local relapse

-improvement in morbidity-improvement in morbidity

GROIN NODE STATUSGROIN NODE STATUS

Groin node negative – no further treatmentGroin node negative – no further treatment

Groin nodes positive after surgeryGroin nodes positive after surgery

- one node only involved- observation only- one node only involved- observation only

- two or more nodes involved inguinal and - two or more nodes involved inguinal and

pelvic radiationpelvic radiation

- nodes clinically positive before surgery: - nodes clinically positive before surgery:

resection followed by irradiation or irradiation resection followed by irradiation or irradiation

ffed by resection or radiation only ffed by resection or radiation only

Role of plastic surgeryRole of plastic surgery

Can be done primarily or as a second stage Can be done primarily or as a second stage procedure.procedure.- insertion of bilateral myocutaneous - insertion of bilateral myocutaneous

tensor fascia lata flaptensor fascia lata flap- rectus abdominis myocutaneous flap- rectus abdominis myocutaneous flap- gracilis muscle myocutaneous flap- gracilis muscle myocutaneous flap- rotational full thickness skin flap taken from the - rotational full thickness skin flap taken from the

inner thigh or buttocksinner thigh or buttocks

POST-OPERATIVE CAREPOST-OPERATIVE CARE

Frequent sitz baths. Patients should dry the vulva completely Frequent sitz baths. Patients should dry the vulva completely after each sitz bath. after each sitz bath. A Foley catheter may be needed for a prolonged period after A Foley catheter may be needed for a prolonged period after surgery around the urethra. surgery around the urethra. Early mobilizationEarly mobilizationHeparin or pneumatic compression stockings should be used in Heparin or pneumatic compression stockings should be used in all women to prevent postoperative venous thrombosis. all women to prevent postoperative venous thrombosis. Place drains at the time of lymphadenectomy because of the Place drains at the time of lymphadenectomy because of the flow through the groin lymphatics. Leave these drains in place flow through the groin lymphatics. Leave these drains in place until drainage is approximately 25 mL or less per day. In many until drainage is approximately 25 mL or less per day. In many cases, this may take more than 2 weeks.cases, this may take more than 2 weeks.Antibiotics /analgesicsAntibiotics /analgesicsHospitalization : 17-33 days on the averageHospitalization : 17-33 days on the average

RADIOTHERAPYRADIOTHERAPY--External beam megavoltageExternal beam megavoltage

-brachytherapy-brachytherapy

INDICATIONSINDICATIONS Primary tumorsPrimary tumors

-squamous vulva cancer-squamous vulva cancer

-basal cell carcinoma-basal cell carcinoma

N.B: verrucous cancers (worsened), melanomas not responsiveN.B: verrucous cancers (worsened), melanomas not responsive -if surgery will lead to significant functional compromise-if surgery will lead to significant functional compromise -cosmesis-cosmesis -frail patients unsuitable for surgery-frail patients unsuitable for surgery Groin nodal involvement- second line/ recurrenceGroin nodal involvement- second line/ recurrence Young women with clitoral cancerYoung women with clitoral cancer Failed local controlFailed local control

chemotherapychemotherapy

No reliable data supports effectiveness of No reliable data supports effectiveness of chemotherapychemotherapy

Its enhances tissue sensitivity to radiationIts enhances tissue sensitivity to radiation

RegimenRegimen

-Cisplatin 100mg /m-Cisplatin 100mg /m2 2 iv on day 1 iv on day 1

-Bleomycin 15mg iv on day 1 and 8-Bleomycin 15mg iv on day 1 and 8

-Methotrexate 300mg/m-Methotrexate 300mg/m22 with citrovarum with citrovarum factor rescue on day 8 factor rescue on day 8

contcont

This must be repeated every 21 days for This must be repeated every 21 days for 2-3 cycles if patient condition and 2-3 cycles if patient condition and laboratory results permitlaboratory results permit

Normal:Normal:

- liver fxn test, - liver fxn test,

-blood urea and electrolyte-blood urea and electrolyte

- HB > 10 g/dl- HB > 10 g/dl

-WBC- > 3x 10-WBC- > 3x 1099/l, platelet > 100 x 10/l, platelet > 100 x 1099/l/l

contcont

Topical 5 fluoro-uracilTopical 5 fluoro-uracil

CryotherapyCryotherapy

Photodynamic therapyPhotodynamic therapy

Done for stage 0Done for stage 0

Immunotherapy- futuristic mgtImmunotherapy- futuristic mgt

Vaccine for HPVVaccine for HPV

Use of immuno-modulating agent – topical Use of immuno-modulating agent – topical imiquimodimiquimod

FOLLOW-UP CAREFOLLOW-UP CARE

Watch out for Psychosexual disordersWatch out for Psychosexual disorders3 monthly visits for the 13 monthly visits for the 1stst 2 years is 2 years is recommended because 80% of recurrence are recommended because 80% of recurrence are in this time period.in this time period.Every 6 months for detection of recurrent dx or Every 6 months for detection of recurrent dx or 2200 primary cancer. primary cancer. At each visit, look for evidence of recurrence of At each visit, look for evidence of recurrence of metastasis & possibility of further neoplastic metastasis & possibility of further neoplastic alteration in adjacent skinalteration in adjacent skinLocal recurrence can be treated by radical Local recurrence can be treated by radical surgical excision or radiotherapysurgical excision or radiotherapy

COMPLICATIONSCOMPLICATIONS

SHORT TERMSHORT TERMHaemorrhage, HaematomaHaemorrhage, HaematomaWound infection and breakdownWound infection and breakdownDeep-vein thrombosisDeep-vein thrombosisPressure soresPressure soresLONG TERMLONG TERMIntroital stenosis (dyspareunia, Apareunia)Introital stenosis (dyspareunia, Apareunia)Urinary & faecal incontinenceUrinary & faecal incontinenceChronic lymphoedema/Lymphocyst formationChronic lymphoedema/Lymphocyst formationCellulitis and LymphangitisCellulitis and LymphangitisPsychological and psychosexual problemsPsychological and psychosexual problems

Differential diagnosisDifferential diagnosis

Vulvar dystrophies- lichen sclerosus, squamous Vulvar dystrophies- lichen sclerosus, squamous cell hyperplasiacell hyperplasiaPaget’s dxPaget’s dxVulval intraepithelia neoplasiaVulval intraepithelia neoplasiaAphthous ulcersAphthous ulcersHerpes genitalisHerpes genitalisLyphogranuloma venerumLyphogranuloma venerumTuberculosisTuberculosisChancroidChancroid primary syphilisprimary syphilis

PROGNOSISPROGNOSIS

Early stage disease carries good prognosisEarly stage disease carries good prognosisLocal recurrence at sites other than the vulva Local recurrence at sites other than the vulva carries poor prognosiscarries poor prognosisShort interval between treatment and recurrence Short interval between treatment and recurrence is a poor prognostic signis a poor prognostic signOverall survival = 75%Overall survival = 75%

Stage I Stage I = 90%= 90%Stage II Stage II = 81%= 81%Stage III Stage III = 68%= 68%Stage IV Stage IV = 20%= 20%

CONCLUSIONCONCLUSION

A rare tumourA rare tumour

Requires expertise and multidisciplinary Requires expertise and multidisciplinary approach in managementapproach in management

Surgery is associated with significant Surgery is associated with significant morbidity & therefore needs to be morbidity & therefore needs to be individualizedindividualized

THANK YOUTHANK YOU