TUMOURS OF THE VULVA AND VAGINA

DR ACHARA AMAECHI PETER

CONSULTANT OBSTETRICIAN AND GYYNAECOLOGIST

TUMOURS OF THE VULVA

• Any swelling of the vulva is called vulva tumour, and could be:

- inflammatory

- traumatic

- neoplastic

• Neoplastic tumours of the vulva could be:

- Benign

- Malignant

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Inflammatory tumours of the vulva

• Furunculosis (boils)
• Bartholin’s abscess

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Traumatic vulval tumour

• Haematoma: astride fall, rupture of vulval vericose vein in pregnancy, following episiotomy

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BENIGN TUMOURS OF THE VULVA

• Divided into tumours of epidermal and mesodermal origin.

Tumours of epidermal origin

• Cysts

- Bartholin’s cyst: caused by occlusion of the duct by fibrosis from Bartholinitis. Superimposed infection leads to abscess

- cyst-adenoma of Bartholin’s gland

- cyst-adenoma of the sweat gland of the vulva

- Endometriotic cysts

- Sebaceous cysts

- Lymphatic cysts

- Implantation cysts following circumcision and perineorrhaphy

- Sub-urethral cysts (of sub-urethral glands)

Treament: excision, except Bartholin’s cyst and abscess which are marsupialized.

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Bartholin’s gland

Anatomy

Gland cyst

Benign tumours of the vulva contd.

• Sebaceous adenoma
• Seborrhoeic keratosis
• Hidradenoma (apocrine sweat gland tumour)
• Pigmented naevi
• Vulval papillomata: condyloma acuminata is a warty cauliflower growth

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• Treatment: excision biopsy

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Tumours of mesodermal origin

• Fibroma
• Lipoma
• Neurofibroma
• Leiomyoma
• Haemangioma – e.g. cavernous haemangioma
• Lymphangioma

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• Treatment : excision biopsy and histology

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MALIGNANT TUMOURS OF THE VULVA

• Divided into Primary and secondary malignant tumours
• Primary malignant tumours of the vulva

- squamous cell carcinoma

- Basal cell carcinoma

- Melanoma

- adenocarcinoma of Bartholin’s gland

- other adenocarcinoma

- Lymphoma

- Sarcoma : Leiomyosarcoma

Fibromyosarcoma

• Secondary malignant tumours: malignant secondaries in the vulva from other primary sites e.g. cervix, vagina, uterus, ovary.

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VULVAR CANCER

• Squamous cell carcinoma 90%
• Malignant melanoma 5%
• Basal cell carcinoma 3%
• Bartholin's gland carcinoma 1%
• Sarcomas < 1%

Vulvar Cancer

• Biphasic Distribution: two main Categories of Patients
• Pre-menopausal Women: Extensive, multifocal, warty or basaloid, HPV-positive lesions (Bowenoid Papulosis)
• Post-menopausal Women: Unifocal HPV-negative keratinising lesions, most commonly located in the navicular fossa, the adjacent mucosa of the vestibule or the labia minora.
• Average Age 60 years
• 20% in patients UNDER 40 and appears to be increasing

VULVAL CARCINOMA

• Incidence is 2 per 100,000 women.
• Constitute 2 – 5% of female genital malignancy
• Aetiology remains unclear, but associated risk factors incude:
• Postmenopausal women with median age of 60
• More common amongst whites
• Increased in obesity, diabetes, hypertension and nulliparity

• HPV DNA (type 16 & 18) detected in patients with invasive vulval cancer
• Chronic pruritus vulvae
• VIN
• Lichen sclerosis
• Lymphogranuloma venerum
• Granuloma inguinale

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Vulva Cancer Etiology

• Chronic inflammatory conditions and vulva dystrophies are implicated in older patients
• Syphilis and lymphogranuloma venereum and granuloma inguinal
• HPV in younger patients
• Tobacco

Pathology of vulval cancer

• SITE: commonest site is labia majus, followed by clitoris & labia minus. Anterior 2/3 commonly affected
• NAKED EYE: Ulcerative everted raised edges, sloughing base and surrounding induration. Hypertrophic lesion with intact overlying skin that may later ulcerate
• HISTOLOGY: well differentiated squamous cell carcinoma in 90% of cases. Melanoma in 5%. Rarely adenocarcinoma of the Bartholin’s gland, basal cell carcinoma and sarcomas
• SPREAD: direct spread to urethra, vagina & rectum. Lymphatic spread is commonest and occur by embolism, involving the nodes in a sequential pattern. Blood borne spread is rare but may occur in advanced cases

• Paget’s Disease of Vulvar
• Predorminantly an intra-epithelial lesion: [adenocarcinoma insitu] of the sweat gland in the vulva, on occasion may be associated with an underlying invasive adenocarcinoma
• Eczematous weeping lesion seen on the vulva
• Associated itching and discomfort
• Biopsy suspicious lesion to confirm diagnosis
• 10% will be invasive
• 4-8% association with underlying Adenocarcinoma of the vulva

TREATMENT OF PAGET DISEASE OF THE VULVA Is Wide Local Excision,

5-florouracil Cream, Lasar ablasion,…..etc.

PAGET’S DISEASE

OF THE VULVA

Clinical features/Diagnosis of Vulva carcinoma

• Patients usually postmenopausal, aged about 60 years, often with obesity, hypertension & diabetes
• Symptoms: pruritus vulvae, swelling ± offensive discharge, vulva ulceration, bleeding, pain
• Sign: inspection reveals ulcer or fungating mass on the vulva, inguinal lymph nodes enlargement, occasionally co-existing primary tumour elsewhere in the genital tract
• Diagnosis: confirmed by biopsy
• Differential diagnosis: condyloma acuminata, syphilitic ulcer, tubercular ulcer, Lymphogranuloma venerum, soft sore

Carcinoma of the vulva

Malignant Melanoma of the vulva

Clinical staging of vulva carcinoma [FIGO 2009]

• Stage – I : tumour confined to vulva
• IA . Lesions ≤ 2 cm in size confined to vulva & perineum.Stromal invasion ≤ 1.0mm. No nodal metastasis
• IB. lesions > 2cm in size or with stromal inversion > 1.0 mm confined to vulva/perineum with negative nodes.
• Stage II: tumour of any size with extension to adjacent perineal structures [ 1/3 lower urethra, 1/3 lower vagina, anus] with negative nodes.
• Stage III: tumour of any size with or without extension to adjacent structures [1/3 lower urethra, 1/3 lower vagina, anus] with positive inguino-femoral lymph nodes.
• IIIA. With 1 lymph node metastasis ≥ 5mm. 1 – 2 lymph nodes metastasis< 5mm
• IIIB. 2 or more lymph nodes metastasis ≥ 5mm . 3or more lymph node metastasis <5 mm
• IIIC. Positive nodes with extra-capsular spread
• Stage IV: tumour invades other regional [ 2/3 upper urethra, 2/3 upper vagina] or distant structures.
• IVA. tumour invade upper urethral/vaginal mucosa, bladder mucosa, rectal mucosa, fixed to pelvic bone. Fixed/ulcerated inguino-femoral lymph nodes.
• IVB. Any distant metastasis including pelvic lymph nodes

Treatment of carcinoma of the vulva

• Early cancer lesion: wide local excision with 1 cm clear of the lesion all round to decrease local recurrence
• Advanced cancer lesion: radical vulvectomy. Triple incision technique preferred. Post-operative pelvic radiation. Pelvic node metastasis rare unless the inguino-femoral nodes are involved (sentinel nodes). Negative sentinel node biopsy for metastasis may avoid extensive lymphadenectomy
• Technically inoperable or recurrent lesions:
• Chemotherapy (cisplatin, bleomycin, 5FU) can used be as radiation sensitizers.
• Chemo-radiation
• Radiation

TUMOURS OF THE VAGINA

• Vaginal cysts – congenital or aquired
• Benign tumours
• Malignant tumours: primary or secondary

Vaginal cysts

• Congenital or Acquired
• Congenital cysts –
• Cysts of Gartner’s duct: enlarged vestigial remnants of mesonephric ducts on antero-lateral wall of the vagina
• Cysts of Skene’s gland on anterior vaginal wall
• Acquired cysts –
• Inclusion cysts: on the posterior wall, buried vaginal mucosa in childbirth/surgery undergo cystic changes
• Endometriotic cysts: implanted endometrium in scars of incisions during surgery on the vaginal wall, commonest on the posterior fornix in association with endometriosis of the recto-vaginal septum
• Treatment: excision of the cyst and marsupialisation of it base

Cyst of the vagina

Gartners cyst

Inclusion cyst

BENIGN VAGINAL TUMOURS

• Papilloma – including multiple warts. May be sessile or pedunculated
• Adenoma
• Fibroma and Fibromyoma
• Lipoma
• Angioma
• Myxoma

Treatment: if large and causing symptoms, excision of the mass is done

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CARCINOMA OF THE VAGINA

• Primary or Secondary

PRIMARY

• Incidence: very rare (about 6 per 1 million women), constitute about 1% of gynaecological malignancies
• Aetiology : exact cause is unknown. Related risk factors include:
• HPV
• VAIN
• History of cervical dysplasia/cancer – multicentric neoplasia
• DES – related to clear cell adenocarcinoma
• Previous radiotherapy
• Prolonged pessary use
• Commoner in whites than blacks

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Pathology of the carcinoma of the vagina

• Site: commonest in the upper third of the posterior wall
• Naked eye: ulcerative or fungative growth
• Histopathology: Squamous cell carcinoma accounts for about 90% of cases. Clear cell adenocarcinoma, melanomas, rhabdomyosarcomas and endodermal sinus tumours are also seen

Clinical presentation and Treatment of carcinoma of the vagina

• Patient profile: usually postmenopausal, at 60 to 80 years old
• Symptoms: may be asymptomatic. Foul smelling vaginal discharge with bleeding, sometimes post-coital bleeding.
• Sign: speculum exam reveals an ulcerative or exophytic growth. Cervix appears normal.
• Treatment: radiotherapy is the usual treatment. Stage I lesion in the upper vagina may be treated with radical hysterectomy + radical vaginectomy + pelvic lymphadenectomy. Exenteration in more advanced cases.

Staging of vaginal carcinoma [ FIGO 2009]

• Stage I: carcinoma is limited to the vaginal wall
• Stage II: the carcinoma has involved the sub-vaginal tissue but has not extended to the pelvic side wall
• Stage III: the carcinoma has extended on to the pelvic side wall.
• Stage IV: the carcinoma has extended beyond the true pelvis or has involved the mucosa of the bladder or rectum. Bullous oedema as such does not permit a case to be allocated to stage IV.
• IVA. Tumour invades bladder/rectal mucosa and/or direct extension beyond the true pelvis.
• IVB. Spread to distant organs.

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Staging

Lymphatic Drainage of Vagina

Secondary tumours of the vagina

• Secondary tumours of the vagina follows carcinoma of the vulva, cervix or urethra.

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• Metastases in the lower – third of the anterior vaginal wall or vault may follow cases of choriocarcinoma or endometrial carcinoma

Essential Aspects of Cancer Control

• Prevention
• Early Diagnosis
• Treatment
• Palliative Care

PREVENTION OF VULVA/VAGINA CANCERS

• Education, awareness creation and health promotion
• Avoid smoking, avoid multiple sexual partners, treat premalignant lesions
• HPV vaccines
• Screening

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• HPV VACCINES: Gardasil [quadri-valent, HPV 6, 11, 16 & 18], - Mark pharmacy in the USA. Cervarix [ bivalent, HPV 16 & 18] – GlaxgoWelcome U.K]
• Given to females 9 – 15 years, can be given up to 26 years
• 100% effective in preventing premalignant lesions of the cervix, vagina & vulva, as well as genital warts in women not already infected with the type of HPV found in the vaccine

QUESTIONS

• Briefly discuss the management of Bartholin’s cyst and abscess

THANK YOU