PRINCESS MARGARET CANCER CENTRE CLINICAL PRACTICE GUIDELINES GYNECOLOGIC CANCER VULVAR

Transcription

1 PRINCESS MARGARET CANCER CENTRE CLINICAL PRACTICE GUIDELINES GYNECOLOGIC CANCER VULVAR Last Revision Date July

2 Site Group: Gynecologic Cancer Vulvar Author: Dr. Stephane Laframboise 1. INTRODUCTION 3 2. PREVENTION 3 3. SCREENING AND EARLY DETECTION 3 4. DIAGNOSIS 4 5. PATHOLOGY 5 6. MANAGEMENT MANAGEMENT ALGORITHMS SURGERY CHEMOTHERAPY RADIATION THERAPY ONCOLOGY NURSING PRACTICE 8 7. SUPPORTIVE CARE PATIENT EDUCATION PSYCHOSOCIAL CARE SYMPTOM MANAGEMENT CLINICAL NUTRITION PALLIATIVE CARE 8 8. FOLLOW-UP CARE 9 Last Revision Date July

3 1. Introduction Vulvar cancer is uncommon and usually seen in older women. The average age at time of diagnosis is 70; less than 20% of women are younger than 50 years of age. It can be seen in women who have a history of dysplasia (vulva, vagina and cervix) and a history of lichen sclerosis, a chronic inflammatory condition of the vulva. 2. Prevention: Many vulvar cancers are associated with HPV (Human Papilloma Virus). Identifying and treating pre-cancerous lesions of the lower genital tract cervix, vagina and vulva Exposure to genital HPV is very common and only requires skin-to skin or skin-to -mucosa contact. In most cases, the body is able to clear the infection on its own. Chronic infection with high risk HPV types predisposes to pre-cancerous lesions (Vulvar intraepithelial neoplasia -VIN) and cancer. Use of condom is not completely protective. Vaccination against HPV: Publicly funded school-based immunization programs are available for grade 8 girls (up to grade 12 for catch up. Females between the ages of 9 to 45 and males between the ages of 9 to 26 are eligible for vaccination through consultation with the family doctor. Cessation of smoking. 3. Screening and Early Detection Women need to be aware of new changes or lesions of the vulva, perineal and perianal areas and seek medical attention for examination. If a lesion looks suspicious, a biopsy is recommended to confirm the diagnosis. The following changes may be noted: *presence of a mass *scaling of skin *raised lesion may be colored : white, red or black *pain, burning sensation, itching *bleeding or malodorous discharge *non healing lesion *no symptom Last Revision Date July

4 4. Diagnosis Stage 1 1A 1B Stage 2 Stage 3 3A 3B 3C Stage 4 4A 4B * FIGO Staging - Carcinoma of the Vulva 2009 Tumour confined to the vulva Lesions 2cm in size, confined to the vulva or perineum and with stromal invasion 1.0mm*, no nodal metastasis Lesions > 2cm in size or with stromal invasion > 1.0mm*, confined to the vulva or perineum, with negative nodes Tumour of any size with extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with negative nodes Tumour of any size with or without extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with positive inguino-femoral lymph nodes (i) with 1 lymph node metastasis ( 5mm), or (ii) 1-2 lymph node metastasis(es) (< 5mm) (i) With 2 or more lymph node metastases ( 5mm), or (ii) 3 or more lymph node metastases (< 5mm) With positive nodes with extracapsular spread Tumour invades other regional (2/3 upper urethra, 2/3 upper vagina), or distant structures Tumour invades any of the following (i) Upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic bone, or (ii) fixed or ulcerated inguino-femoral lymph nodes Any distant metastasis including pelvic lymph nodes The depth of invasion is defined as the measurement of the tumour from the epithelialstromal junction of the adjacent most superficial dermal papilla to the deepest point of invasion Initial evaluation Physical examination: A complete physical examination including evaluation of inguinal lymph nodes. WHO performance score is important as the disease often affects women at advanced age. When examining the tumour it is important to document its size, location and whether or not it involves a midline structure. i.e. clitoris, anus, urethra., and extension into the vagina. Tissue biopsy is necessary. A diagram is very useful to document the site of biopsy/excisional biopsy. Laboratory testing: CBC, Creat. LFT, as indicated. Imaging: CT Abdomen/pelvis/thorax, MRI In some situations a cystoscopy and or sigmoidoscopy may be required to determine the extent of the tumour. Pathology review Last Revision Date July

5 5. Pathology Most vulvar cancers are squamous cell cancers. The keratinizing type is seen in older women and not HPV related. The basaloid/warty types are seen in younger women and are associated with HPV. The verrucous type are slow growing. The adenocarcinomas of the vulva represent 8% of all vulvar cancers, most often arising from the Bartholin gland or sweat glands. Paget s disease is a subtype consisting of malignant cells in the superficial layer of the skin; 25% of cases will also have an invasive vulvar cancer. Melanomas s of the vulva are rare (6%) and represent a minority (5%) of all melanomas. Sarcoma are uncommon less than 2%, and seen in all age groups. Basal cell cancers are rare. Last Revision Date July

6 6. Management 6.1 Management Algorithms Vulva carcinoma diagnosed with biopsy Last Revision Date July

7 Recurrent vulvar cancer 1. In previously radiated patients, surgery will be the treatment and following resection it may be necessary to bring in a myocutaneous flap for closure of the wound and to promote healing with a new blood supply. Last Revision Date July

8 6.2 Surgery Radical Vulvectomy and Lymphadenectomy The primary tumour should be excised with lateral, medial and deep margin of at least 1 cm. Lymph node dissection is performed through separate incisions. Sentinel Lymph Node Dissection (SNL) Use of Technectium- 99m sulphur colloid and Isosulfan blue dye injection for identification of sentinel lymph nodes. If SNL cannot be identified, full lymph node dissection should be carried out. 6.3 Chemotherapy o Use of cisplatin or 5-FU in conjunction with radiation therapy 6.4 Radiation Therapy o See algorithms 6.5 Oncology Nursing Refer to general oncology nursing practices 7. Supportive Care 7.1 Patient Education Refer to general patient education practices 7.2 Psychosocial Care Refer to general psychosocial oncology care guidelines 7.3 Symptom Management Refer to general symptom management care guidelines 7.4 Clinical Nutrition Refer to general clinical nutrition care guidelines 7.5 Palliative Care Refer to general oncology palliative care guidelines Last Revision Date July

9 8. Follow-up Care Post-surgery follow up: Every 3months for 2 years, then every 6 monthsto 5 years.. Physical Exam and imaging if clinically indicated Post-radiation follow up: Every 3 months for 2 years, then every 6 months to 5 yers + Physical Exam - and imaging if clinically indicated Recurrence Consider chemotherapy, surgery and/or radiation therapy multidisciplinary approach Last Revision Date July