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Vulva

VIN is a pre-cancerous skin lesion of the vulva that frequently progresses to invasive vulvar cancer if left untreated. It is usually asymptomatic but can present with itch, soreness or burning. On pathology, VIN shows maturation disorder, cellular atypia and abnormal mitotic figures. Risk factors include HPV infection and immunosuppression. Treatment options include surgical excision for localized lesions or medical management with imiquimod or 5-fluorouracil cream for widespread disease. Regular follow-up is needed due to the risk of recurrence.

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Dr Sai L Daayana MBBS, MRCOG, MD, Sub-specialty training in Gynaecology Oncology (UK) Consultant in Surgical Gynaecology Oncology Apollo Cancer Institutes, Hyderabad VULVA - BENIGN, PRE-MALIGNANT & MALIGNANT CONDITIONS
WHAT IS VULVAL INTRAEPITHELIAL NEOPLASIA? Vulval (or vulvar) intraepithelial neoplasia is a pre-cancerous skin lesion of any part of the vulva. Vulval intraepithelial neoplasia (VIN) is now also called vulval squamous intraepithelial lesion (SIL). VIN is not invasive cancer but vulval squamous cell cancer (SCC) occurs in about 15% of women if VIN is left untreated.
Epidemiology of VIN Uncommon premalignant condition of the vulva Increasing in incidence Associated with HPV in young women May co-exist with CIN, immunosuppression is risk factor Clinical Features of VIN Itch, soreness, burning, asymptomatic Leukoplakic, erythematous, ulcerated or pigmented lesions on the vulva Pathology of VIN Maturation disorder, cellular atypia, abnormal mitotic figures, increased nuclear:cytoplasmic ratio, graded VIN 1 to VIN 3
VIN – high grade
Vulval Intraepithelial Neoplasia
§ Is usually asymptomatic True/False § Frequently progresses to invasive carcinoma if left untreated True/False § Is characterised by blunting of rete ridges in the epidermis True/False § Presents with parchment-like appearance of the vulva in a figure of eight distribution, with loss of vulval architecture and atrophy True/False § Is commonly seen in women with a history of abnormal cervical cytology True/False VULVAL INTRAEPITHELIAL NEOPLASIA
TREATMENT OF VULVAL INTRAEPITHELIAL NEOPLASIA §  Low-grade – asymptomatic – regular vulvoscopy every 6 months §  Low-grade – symptomatic – treatment offered §  High-grade – treatment offered to reduce the risk of developing invasive cervical cancer
TREATMENT FOR VULVAL INTRAEPITHELAIL NEOPLASIA Unilocular lesion – Surgical treatment – wide local excision with a margin of normal tissue; or skinning vulvectomy LASER ablation (under GA) – if no malignancy suspected, multifocal lesions
MEDICAL MANAGEMENT OF VIN Large areas of VIN; multiple previous excisions can be managed medically with: 1. Imiquimod cream, applied 3 times weekly for 12 to 20 weeks. This results in red, inflamed and eroded tissue often accompanied by considerable discomfort. 2. 5-fluorouracil cream, applied twice daily for several weeks. This causes quite severe inflammation (several weeks) and will not be tolerated by all women. It is less effective than imiquimod cream. 3. Photodynamic therapy (PDT) requires specialised equipment and can also be very painful. 4. Cidovir has been described to be useful in some patients.
IMIQUIMOD CREAM ¡ Immunomodulator ¡ Acts on Toll-like receptors ¡ Enhances local immune responses CIDOFOVIR MEDICAL MANAGEMENT OF VIN §  Anti-viral drug §  Anti-tumour agent
5-FLUORO URACIL CREAM ¡ Anti-metabolite ¡ Given systemically and topically in cancer treatment PHOTO DYNAMIC THERAPY MEDICAL MANAGEMENT OF VIN ¡ Uses a drug, called a photosensitizer or photosensitizing agent, and a particular type of light. They produce a free radical oxygen molecules that kills nearby cells.
RECURRENCE OF VIN Recurrence rates of VIN after treatment range from 9% to 50% Risk factor for recurrence: •  positive excision margins •  Multi-focal disease •  Smoking •  Immunosuppression
FOLLOW-UP FOR VIN Self-examination of the vulva and regular vulvoscopy follow-up is prudent Women with a complete response to therapy and no new lesions at follow-up visits scheduled 6 months and 12 months after initial treatment should be monitored by visual inspection of the vulva annually thereafter.
Lichen Sclerosus § Epithelial thinning, inflammation, histological changes in the dermis (loss of rete ridges) ¡ Clinical features § Intractable itch, soreness, dyspareunia, asymptomatic § Parchment-like appearance, figure of eight distribution, loss of vulval architecture, vulval atrophy § Adults, occasionally children § 9% incidence of progression to SCC of vulva OTHER DERMATOLOGICAL CONDITIONS OF THE VULVA
Lichen Sclerosus
Lichen Sclerosus
Lichen Planus •  Chronic inflammatory dermatitis of unknown aetiology •  Characterised by flat topped violaceous shiny papules •  Clinical features range from white reticulated papules to an erosive, desquamating process DERMATOLOGICAL CONDITIONS (2)
Lichen Planus
DERMATOLOGICAL CONDITIONS (3) Squamous Cell Hyperplasia •  Diagnosis of exclusion •  Chronic reaction to fungal vulvitis, allergies or unknown stimuli •  Thickened skin with white keratotic patches, excoriations & fissures
¡ Psoriasis ¡ Hidradenitis Suppurativa § Chronic suppurative disorder of apocrine glands § Deep painful subcutaneous nodules that ulcerate and drain leading to open sinuses and extensive scarring DERMATOLOGICAL CONDITIONS (4)
Psoriasis
Hidradenitis Suppurativa
¡ Can be solitary or multiple, painful or non painful ¡ Seen more frequently in GUM clinic ¡ Solitary, non painful ulcers include: syphilis, lymphogranuloma venerum, cancer ¡ Multiple painful ulcers include: herpes, Behcet’s disease, Crohn’s disease ¡ Investigations: serology, culture, biopsy ¡ Treatment depends on cause ULCERATIVE DERMATOSES
Crohn’s Disease
¡  Rare condition that typically affects postmenopausal Caucasian women ¡  Presents as crusty, erythematous area on vulva ¡  Associated with pruritis vulvae ¡  Often misdiagnosed as contact dermatitis or eczema ¡  Biopsy is necessary for diagnosis ¡  90% of cases are primary intraepithelial malignancies & treatment is aimed at symptom control ¡  10-15% are associated with an underlying adenocarcinoma of the vulva ¡  30% have malignancies at non-vulval sites, including breast, colon, & bladder ¡  Need to exclude invasive disease in vulva and exclude underlying internal carcinoma from another site (e.g. by colonoscopy, CXR, mammogram, hysteroscopy etc) EXTRA MAMMARY PAGETS DISEASE
Extramammary Paget’s Disease of the Vulva
§ Uncommon cancer § Most patients are elderly (60-75 yrs) § Squamous carcinoma most common § Melanoma second most common (4-9% of cases, post menopausal Caucasian women) § Basal Cell Carcinoma has excellent prognosis § Adenocarcinoma of Bartholin’s gland or in conjunction with Paget’s disease (20%) is rare VULVAL CANCER (1)
INCIDENCE OF VULVAL CANCER 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 •  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
¡ Diagnosis § Vulval itch & soreness § Painful bleeding mass § Asymptomatic lump ¡ Sites involved § Labium majus (50%) § Labium minus (20%) § Clitoris & Bartholin’s Gland (uncommon) VULVAL CANCER (3)
Squamous Cell Carcinoma of Vulva
Squamous Cell Carcinoma of Vulva
Basal Cell Carcinoma of vulva
Malignant Melanoma of vulva
VULVAL CANCER (4) §  Disease Spread Via the lymphatics to the superficial inguinal lymph nodes; metastasizes to pelvic lymph nodes late Direct spread to lower vagina, urethra & anus
FIGO STAGING OF VULVA CARCINOMA Stage 1 2 cm lesion size Or less Confined to the vulva or perineum nodes histo-Logically negative. Stage 2 > 2cm lesion size Confined to the vulva or perineum nodes histo-Logically negative. Stage 3   Tumor of any size spread to lower urethra vagina anus +/- Unilateral metastasis Stage 4 A Involvement of : Upper urethra Bladder mucosa Rectal mucosa Pelvic bone Bilateral L.N.metastasis   B Distant metastases and / or pelvic nodes
VIN Vulval cancer
VIN
Vulval carcinoma
The 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 factor ¡  Metastatic 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%. PROGNOSIS OF VULVAL CANCER
¡  Detec2on and management of VIN. ¡  Proper management of all cases with pruiritus vulvae. ¡  All vulval lesions should be diagnosed accurately especially those arising a?er menopause. ¡  All pigmented vulvar lesions should be removed for biopsy. PROPHYLAXIS…A HIGH INDEX OF SUSPICION
Treatment OptionStage Partial Vulvectomy excision of the tumor, with a 1 cm safe margins. No need for node removal. Ia 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 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 TREATMENT OF VULVAL CANCER
VULVECTOMY: 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.
Right hemi-vulvectomy
Anterior vulvectomy with bilateral groin node dissection
¡  Detec2on and management of VIN. ¡  Proper management of all cases with pruiritus vulvae. ¡  All vulval lesions should be diagnosed accurately especially those arising a?er menopause. ¡  All pigmented vulvar lesions should be removed for biopsy. PROPHYLAXIS…A HIGH INDEX OF SUSPICION
What is this lesion?
Vulval Intraepithelial Neoplasia
What is this lesion?
Lichen Sclerosus
The most effective treatment for pruritus vulva associated with lichen sclerosus is: 1. Acqueous cream 2. Topical clotrimazole 3. Anti-histamines 4. Imiquimod 5. Topical steroid creams
The most effective treatment for pruritus vulva associated with lichen sclerosus is: 1. Acqueous cream 2. Topical clotrimazole 3. Anti-histamines 4. Imiquimod 5. Topical steroid creams
What is this lesion on the vulva?
Squamous Cell Carcinoma of Vulva
What is this lesion on the vulva?
Malignant Melanoma of vulva
¡  Vulval Intraepithelial Neoplasia § Is usually asymptomatic True/False § Frequently progresses to invasive carcinoma if left untreated True/False § Is characterised by blunting of rete ridges in the epidermis True/False § Presents with parchment-like appearance of the vulva in a figure of eight distribution, with loss of vulval architecture and atrophy True/False § Is commonly seen in women with a history of abnormal cervical cytology True/False
¡  Vulval Intraepithelial Neoplasia § Is usually asymptomatic True/False § Frequently progresses to invasive carcinoma if left untreated True/False § Is characterised by blunting of rete ridges in the epidermis True/False § Presents with parchment-like appearance of the vulva in a figure of eight distribution, with loss of vulval architecture and atrophy True/False § Is commonly seen in women with a history of abnormal cervical cytology True/False