Carcinoma of the Vulva
Incidence of malignant diseases the vulva:
3 - 4% of all gynecologic malignancies.
- The incidence increases with age.
- Recently there was a rise in the incidence, due to
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•
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Longevity
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 carcinoma of the
cervix or vagina.
4- Chronic immunosuppression.
5- Smoking
2 Types / Variants
(15%)
(85%)
(90%)
(5%)
(2-3%)
Vulvar Cancer Stages FIGO System
• Stage 0 - Carcinoma in situ, VIN 3, severe vulvar
dysplasia.
• Stage I - Tumor 2 cm or less, and confined to the vulva or
perineum
– IA - Less than 1 mm invasion below the surface layer
– IB - More than 1 mm invasion below the surface layer
• Stage II - Cancer is confined to the vulva and/or
perineum, and larger than 2 cm.
• Stage III - Cancer has spread to
– the lower urethra or vagina or anus
– and / or local lymph nodes on one side.
• Stage IV
– A - Cancer has spread to the
• Upper urethra or bladder or rectum
• or local lymph nodes on both sides.
– B - Cancer has spread to the pelvic lymph nodes and/or sites
more distant.
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
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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
Diagnosis
The diagnosis often is delayed:
1- Patients do not ask early consultation. They consider the
symptoms as a trivial skin condition.
2- Physicians may neglect small skin lesions.
Types of complaints:
• The most common complaint is a palpable vulvar lesion.
Chronic pruritus vulvae.
• Asymptomatic 20% of patients: the lesion is detected during
examination for unrelated condition.
• Later the lesion becomes necrotic cauliflower or hard
ulcerated.
– Bleeding, watery discharge, superinfection and pain may develop.
• Melanomas: frequently appear as bluish black, pigmented, or
papillary lesions.
Diagnosis
1) 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.
Prophylaxis…A high index of suspicion
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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.
Management
Modalities
Surgical Treatment
Radical Vulvectomy
Radiotherapy
Pre-operative
En Block Dissection
3-in one incision
Post-operative
Chemotherapy
Radiation Sensitizer
Metastatic
Conditions
Treatment Options by Stage
Stage Treatment Option
Partial Vulvectomy excision of the tumor, with a 1 cm safe margins. No need
Ia
for node removal.
Ib
Modified 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
II
III
Modified radical vulvectomy with bilateral groin node dissection.
IV
Individualized
- 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.
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.
Sentinel Lymph Node
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:
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•
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.
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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
Malignant Tumors of the
Vagina
• Incidence
– 1% of gynecologic malignancies.
– It is the 5th in frequency of primary genital malignant
diseases.
– Average age at diagnosis is 65 years old.
• High risk factors:
– VaIN
– Human papillomavirus infection of the cervix or the
vulva.
– Cervical or vulvar cancer.
– Exposure to diethylstilbestrol (DES) in utero is
associated with the development of vaginal adenosis
• It might progress to clear cell adenocarcinoma of the vagina
and cervix in young wome
• The mean age at diagnosis of this rare malignancy is 19 year.
Pathological Types:
• Secondary malignant tumors of the vagina are more
common than the primary tumors.
– The primary lesion may be in the cervix or elsewhere in the body.
• Primary malignant vaginal tumors:
– Squamous cell carcinomas: 85% of primary vaginal malignancies.
– Adenocarcinomas. These occur at younger age group.
– Clear cell adenocarcinomas secondary to DES exposure.
– Melanoma.
– Sarcoma: Sarcoma botryoides (embryonal rhabdomyosarcoma)
• The peak incidence is in young children at the age of 3 years.
• Symptoms of malignant lesions of the vagina:
– Abnormal vaginal bleeding: may be postcoital, intermenstrual, or
postmenopausal.
– Watery vaginal discharge.
– Dyspareunia.
– Vesicovaginal or rectovaginal fistulae are late manifestations of
vaginal cancer.
– Few patients are asymptomatic; a lesion may be discovered
during a routine pelvic examination, or a Pap smear may be
abnormal.
• Signs of malignant lesions of the vagina::
– Local examination: the need to inspect the whole vagina entails
modification from the routine speculum examination.
– A polypoid lesion is the commonest macroscopic appearance.
– A punch biopsy usually yields a diagnosis, but occasionally wide
local excision using an anesthetic is necessary. Most lesions
occur in the upper 1/3 of the vagina on the posterior wall.
– Colposcopy, cystoscopy, and proctosigmoidoscopy and bone
scan are needed to detect spread.
Spread:
• Direct spread: into the local paravaginal
tissues, bladder, or rectum.
• Lymphatic spread:
– Lesions in the lower vagina: to the inguinal
lymph nodes.
– Lesions in the upper vagina: to the pelvic lymph
nodes.
• Hematogenous spread: late event. Reach
liver, lung, bone.
Staging
Stage 0
Carcinoma in situ, intraepithelial neolasia
Stage I
Carcinoma limited to the vaginal wall
Stage II
Involved the subvaginal tissue but not extended to
lateral pelvic wall
Stage III
Extended to pelvic wall
Stage IV a Spread to adjacent organs or direct extension
beyond the true pelvis.
Stage IV b Spread to distant organs
Stages I & II
Stage III
Treatment:
• Management of stage 0:
– Topical fluorouracil for stage 0:
• It causes intense burning.
• Long standing benefits is not proven yet
• Laser therapy
• Primary localized tumors:
– RT: a combination of external beam and
brachytherapy.
– Surgery: it is an alternative treatment for early lesions.
• Radical hysterectomy with upper vaginectomy.
• Pelvic exenteration: in vesicovaginal or rectovaginal fistulas.
Prognosis:
• The 5-year survival is as follows:
– Stage I 65-70%
– Stage II 47 %
– Stage III 30%
– Stage IV 15-20%