British Gynaecological Cancer Society (BGCS) Vulval Cancer Guidelines: Recommendations for Practice 15 May 2020 Jo Morrison, Peter Baldwin, Lynn Buckley, Lucy Cogswell, Katharine Edey, Asma Faruqi, Raji Ganesan, Marcia Hall, Kathryn Hillaby, Nick Reed, Phil Rolland, Christina Fotopoulou. The purpose of this guideline is to collate evidence and propose evidence-based guidelines for the diagnosis and management of adult patients with vulva carcinoma treated in the UK. Malignant melanoma may present via similar routes and will be discussed. The reader is referred to the Ano-uro- genital Mucosal Melanoma Full Guideline (1) for more detailed recommendations. The management of vulval sarcoma is outside of the scope of this guideline. Reviewers: Shahram Abdi, Jonathan Frost, Cathrine Holland, Sean Kehoe, Tracie Miles, Andy Nordin, Nithya Ratnavelu, Partha Sengupta, John Shepherd, Sudha Sundar. International Reviewers: Amanda Tristram; Linn Woelber. Table of Contents 1 INTRODUCTION .......................................................................................................................... 4 1.1 GRADES OF RECOMMENDATIONS ............................................................................................. 4 1.2 GUIDELINES DEVELOPMENT PROCESS ...................................................................................... 4 2 BACKGROUND AND EPIDEMIOLOGY ...................................................................................... 6 3 PREVENTION, SCREENING, PRESENTATION AND DIAGNOSIS ........................................... 6 3.1 PREVENTION AND TREATMENT OF PRE-DISPOSING CONDITIONS ................................................. 6 3.1.1 HPV-related squamous cell carcinoma ............................................................................ 7 3.1.2 Squamous cell carcinoma on a background of lichen sclerosus (LS)/lichen planus (LP) 8 3.1.3 Mucosal malignant melanoma ......................................................................................... 8 3.2 SCREENING ........................................................................................................................... 8 3.3 PRESENTATION .................................................................................................................... 10 3.4 DIAGNOSIS .......................................................................................................................... 11 3.5 PRE-OPERATIVE INVESTIGATIONS .......................................................................................... 14 3.5.1 Squamous Cell Carcinoma (SCC) ................................................................................. 14 3.5.2 Melanoma ...................................................................................................................... 14 3.5.3 Basal Cell Carcinoma .................................................................................................... 14 3.5.4 Bartholin’s Gland Carcinoma ......................................................................................... 14 3.5.5 Paget’s disease of the vulva .......................................................................................... 14 4 PATHOLOGY ............................................................................................................................. 15 4.1 PRECURSOR LESIONS ........................................................................................................... 15 4.1.1 Usual/classical VIN (HPV-associated neoplasia) ........................................................... 15 4.1.2 Differentiated VIN .......................................................................................................... 15 4.1.3 Other putative precursors .............................................................................................. 16 4.2 PATHOLOGY OF SQUAMOUS CELL CARCINOMA ........................................................................ 16 4.2.1 Types of squamous cell carcinomas .............................................................................. 16 4.2.2 Macroscopic features of importance .............................................................................. 16 4.2.3 Microscopic features of importance ............................................................................... 16 4.3 SPREAD .............................................................................................................................. 17 4.3.1 Lymph node metastasis ................................................................................................. 17 4.3.2 Sentinel lymph nodes (SLN) .......................................................................................... 17 4.3.3 Extranodal extension ..................................................................................................... 18 4.4 ANCILLARY STUDIES ............................................................................................................. 18 4.4.1 Ancillary studies in uVIN ................................................................................................ 18 4.4.2 Ancillary studies in dVIN ................................................................................................ 18 4.5 PATHOLOGY OF VULVAL PAGET’S DISEASE AND INVASIVE ADENOCARCINOMA OF THE VULVA ...... 18 4.6 PATHOLOGY OF VULVAL MELANOMA ....................................................................................... 19 5 TREATMENT OF PRIMARY DISEASE ..................................................................................... 20 5.1 SURGERY ............................................................................................................................ 20 5.1.1 Management of primary site .......................................................................................... 20 5.1.2 Surgical management of other vulval cancers ............................................................... 24 5.1.3 Management of inguinal lymph nodes ........................................................................... 28 5.1.4 Reconstructive surgery .................................................................................................. 32 5.1.5 Vacuum-assisted Closure (VAC) ................................................................................... 34 5.2 RADIOTHERAPY .................................................................................................................... 35 5.2.1 Adjuvant radiation / chemoradiation therapy.................................................................. 35 5.2.2 Primary site irradiation-primary treatment ...................................................................... 36 5.2.3 Palliative radiotherapy ................................................................................................... 37 5.3 CHEMOTHERAPY .................................................................................................................. 37 5.3.1 Squamous Cell Carcinoma ............................................................................................ 37 5.3.2 Neoadjuvant chemotherapy for invasive squamous cell carcinoma .............................. 37 5.3.3 Adjuvant chemotherapy ................................................................................................. 38 6 TREATMENT OF RECURRENT DISEASE ............................................................................... 40 6.1 RECURRENCE RATES AND SURVIVAL ...................................................................................... 40 6.2 LOCALISED RECURRENCE ..................................................................................................... 40 6.2.1 Surgery .......................................................................................................................... 40 6.2.2 Radiotherapy ................................................................................................................. 41 6.3 PALLIATIVE CHEMOTHERAPY ................................................................................................. 41 6.4 LOCAL NODAL RECURRENCE ................................................................................................. 42 7 PSYCHOLOGICAL/PSYCHOSEXUAL SUPPORT ................................................................... 43 7.1 PSYCHOSOCIAL .................................................................................................................... 43 7.2 LYMPHOEDEMA .................................................................................................................... 43 7.3 SEXUALITY/SEXUAL MORBIDITY ............................................................................................ 44 7.4 BOWEL/BLADDER FUNCTION .................................................................................................. 44 8 FOLLOW UP .............................................................................................................................. 45 8.1 FOLLOW UP OF VSCC .......................................................................................................... 45 8.2 FOLLOW UP OF BASAL CELL CARCINOMA OF THE VULVA ........................................................... 45 8.3 FOLLOW UP OF VULVAL PAGET’S DISEASE ............................................................................. 45 8.4 FOLLOW UP OF VULVAL MALIGNANT MELANOMA. .................................................................... 46 9 REFERENCES ..........................................................................................................................