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Vulval Disorders and Vulval Cancer - Coggle Diagram
Vulval Disorders and Vulval Cancer
Anatomy and Assessment
Anatomy
Examination of Vulva
Areas
Genitocrural folds
Labia majora and minora
Clitoris
Perineum and perianal area
Vaginal / cervical may be necessary (field effect of HPV / VIN / cancer)
Mons pubis
Changes
Anatomy
Introital narrowing
Fusion of clitoral hood
Induration
Changes in pigment
Lichenification
Ulceration
Fissuring
Common History Questions / Reasoning
How long
have they been experiencing symptoms
Acute → Thrush / Contact dermatitis
Chronic → Licen sclerosus / vulval intraepitelial neoplasia (VIN)
Any
other symptoms?
Discharge
Bleeding
Local pain
Coital difficulties
Skin conditions
Systemic symptoms
What
treatments
have been
tried
before?
Potential issues:
Inappropriately used
Not potent enough
Not used for long enouhg
How is the pt
cleaning vulval area?
Overwashing - skin damage
Possible contacts with
irritants
such as soaps, shampoos, scented vaginal wipes?
Soaps / gels may cause local problems
Are symptoms
Stress
related?
Lichen cimplex chronicus itches worse when stressed
Any
systemic illness?
Diabetes
Renal failure
Thyroid problems
Any othe
skin conditions
of
Fhx
of skin conditions?
Eczema
Psoriasis
Vulval Care - General Advise
Avoid contact soap, shampoo, bubble bath
Use simple emollient as soap substitute
Avoid tight fitting / synthetic garments - irriate area
Non biological washing powder, plain cotton underwear , no pads
Cotton gloves to stop nocturnal scratching
Topical / systemic antihistamines
Detailed explanation of condition with emphasis on long term implication on health / partners health - written infor
Exclude candidiases if present with itch
Lichen Sclerosis
Chronic Inflammatory dermatosis
Epidemiology
Most common in post menopausal women
Clinical presentation
Severe pruritis
Fluctuating pattern of symptoms
Worse at night
Involves vulval and perianal area in figure of 8 pattern
Skin atrophy
Erythema
Sub epithelila haemorrhage and fissuiring
Hyperkeratosis - thickened skin
Inflammatory adhesions
Midline fusion (continuing inflammation) with narrowing intritus
Dysparneunia
Dribbling incontinence
Acute urinary retension
Complications
Development of squamous cell carinoma (5% risk)
Management
Reducing Regimen of
Topical Potent Corticosteroids
0.05% clobetasol proprionate ointment / Dermovate
Use daily for 1 month → Alternate days for 1 month → Twice weekly for 1 month → PRN
Follow-Up
Review at 3 months - check response
Review at 6 months - Check Compliance / understanding of self management
Annual review in primary care
Pt education on risk of SCC, self examination, presentation to GP if new lesion
Lichen Planus
Autoimmune inflammatory skin condition with features similar to lichen sclerosus
Epidemiology
Postmenopausal women
Clinincal Presentation
Vulval itching
Vulval pain
Clinical Variants
Classical Type
Isolated skin lesions - white pearly papules on skin of vulva
Lichen planus elsewhere on body
Treatment
Topical strong corticosteroids
Skin care with emollients
Erosive Type
Eroded mucosal surfaces
Wickham's striae - mauve and pale epithelial network at edges of erosions
Pain and soreness
Treatment
Intravaginal steroid
Vulval Contact dermatitis
Pathophysiology
Damage by chemicals / physical agents occurs faster than skin can repair
Clinical Presentation
Variable changes
Well demarcated erythema
Swelling
Itching
Blistering
Minal erythema - minor degree
Causes (Irritants)
Scented products / soaps
Topical steroid creams / ointments
OTC antifungal creams
Urine
Vulval Eczema
Epidemiology
Usually have disease elswhere
Clinical Presentation
Skin erythema
Skin scaling
Skin fissuring
Usually affects labia and natal cleft
Treatment
Emollients
Topical corticosteroids
Lichen Simplex
Chronic inflammatory skin condition
Clinical Presentation
Severe intractable pruritis - esp at night
Non - specific inflammation of labia majora
Inflammation may spread to mons pubis and inner thighs
Oedematous swelling
Discrete areas of thicjening and lichenification - 2° to scratching
Can be exacerbated by chemical / contact dermatitis
Treatment
General care of vulva
Emollients
Antihistamines
Moderate / ultrapotent steroids
Psoriasis
Chronic papulosquamous proliferative inflammatory skin condition in which epidermal cell cycle is reduced leading to pruritis and soreness
Clinical Presentation
Psoriasis evident in other areas
Erythematous with a well-defined edge
Diagnosis
Biopsy
Treament
Emollients
Topical steroids (weak - moderate)
Avoid coal tar preps - not well tolerated on vulva
Sebaceous Cyst
Clinical Presentation
Generally mobile masses
Fibrous tissue tilled with inspissated sebaceous material
Common in hear bearing areas
Can be mutliple and widepread
Mangement
Excision under LA
Important to remove central punctum to prevent recurrence
Bartholin's Abcess
Pathophysiology
Duct from bartholin's gland to vaginal opening gets blocked
Infection of gland / duct leads to abcess
Epidemiology
More common in childbearing age
Clinical Presentation
Smooth and well localised
Management
Small - Abx + spontaneously drains
Big - Drainage with Word Catheter under LA
I&D and marsuplisation
Hidradenitis Supparativa
Chronic skin disorder
Clinical Presentation
Skin abcesses
Fistulas
Scarring
Affects anogenital and axillary areas
Epidemiology
1% prevalence
Unknown aetiology
Treatment
Limited options
1️⃣ Line :Topical / systemic abx
Collaborate with dermatologist
VIN - Vulval Intraepithelial Neoplasia
Definition
Pre-cancerous skin lesion that affects any part of the vulvla
Types
Unusual-Type VIN (uVIN)
HPV associated
Differentiated VIN (dVIN)
Not related to HPV
Associated with inflammatory diseases of vulva
Lichen sclerosis
Erosive lichen planus
Less common that uVIN
Vulval Cancer
Epidemiology
55 cases per yr in IRE
Number of younger women affected increasing
80% in those >65
70% present with pruritis vulvae
25% bleeding occurs
Delay in presentation - elderly women due to embarressment
Clinical Presentation
Pruritis- most common
Burning / pain / discomfort of vulva
Ulcer / swelling / lump that doesn't go away
Changed to texture / feel of skin
Changes to colour of skin
Bleeding from vulva / vagina
Risk Factors
Age
Exposure to HPV
Cigarette smoking
Immunosuppression / HIV
VIN
Lichen sclerosus
Classification
Squamous cell carinoma - 90%
Primary vulval melanoma
Basal cell carcinoma
Bartholin's gland carcinoma
Adenocarcinoma
Sarcoma - very rare
Squamous cell carcinoma of vulva
Pathogenesis
Lichen sclerosus / planus → dVIN → HPV independent SCC
HPV infection (16 mostly) → uVIN → HPV associated SCC
Clinical Features
Specific lesion
Painful
Bleeding
Fungating lesion +/- palpable groin nodes
Vulval Melonoma
Rare but aggressive
Clinical features
Irregular outline
Pigmented / non-pigmened macule, papule, patch or nodule
With / without nodule
+/- Lymph nodes
Vulval Basal Cell Carinoma
More indolent - rarely spreads to lymph nodes
Clinical features
Discrete vulval lesion without background dermatosis
Classical raised, rolled edge ulcer
Diagnostic Tests / Investigations
Punch Biopsy
Depth
At least >1mm
Ideally include edge of lesion where transition from normal to abnormal tissue
Avoid central ulcer - may not be diagnostic
Document with diagram / clinical photography
Exision biopsy
Avoid - limits options for conservative treatment with wide local exision and sentinel node biopsy
Esp if lesion is small
Document with diagram / clinical photography
Management In Primary Care
Spread
Local
Vagina
Urethra and clitoris anteriorly
Anus / rectum posteriorly
Lymphatics
Groin (Inguinofemoral) lymp nodes
Pelvic lymph nodes
Haemtogenous
Rare
Vulval Cancer Assessment
Staging
Clinical Assesssment
General exam
Pelvic exam
Vulval exam
PR exam if indicated
MRI Pelvis
Assess local involvement
Urethra
Bladder
Anal sphincter
Bowel
PET CT Scan
Ass nodal involvement and mets
FIGO 2021
https://obgyn.onlinelibrary.wiley.com/doi/full/10.1002/ijgo.13880
Stage 1
Tumour confined to vulva
Stage 2
Tumour of any size with extension to lower 1/3 of urethra / vagina / anus with negative nodes
Stage 3
Tumour of any size with extension to upper part of adjacent perineal structures, or with any number of nonfixed, nonulcerated lymph node
Stage 4
Tumour of any size fixed to bone, or fixed, ulcerated lymph node mets, or distant mets
Treatment
Stage 1a
Simple vulvectomy / Wide local excision
Stage 1b - Stage 3
Radical vulvectomy
Lymph assessment
+/- futher procedures if invading other organs
Lymph node assessment
Sentinel lymph node biopsy (tumour <4cm)
Inguinofemoral lymphadenectomy (lymph node dissection)
Adjuvant radiotherapy
Significant nodal disease
Surgical margins positive
Definitive Chemo-Radiotherapy
Consider in pts deemed inoperable due to extent of tumour and / or fitness for anaesthesia
Neoadjuvant chemotherapy +/- radioation
Patient Information
Treatment carefully planned pre-op
Ideally diagrams for pt to ensure adequate consent
Warn of effects on sexual function following surgery - esp if clitoral area involved
Show image of surgical outcomes
Complications of Lymphadenectomy
High incidence of complications
Lymphocyst formation
Suction drainage usually employed after lymphadenectomy
Wound breakdown 34%
Can delay adjuvant radiation
Lymphoedema
Sentinel Lymph Node Biopsy (SLNB)
Standard of care where indicated
Accurate
Associated with reduced morbidity
Identification
Methylene blue dye
IndoCyanine Green (ICG) ( Flourescence Imaging)
Radiolabelled isotope - Technetium 99
Formal lymphadenectomy for larger tumours
SLNB reduced sensitivity + Higher false negative rate
Follow Up
Lower Risk Recurrenc
Unifocal HPV - associated
Clinic based follow up for 5 years
Higher Risk Recurrence
HPV-independent
Multifocal HPV-associated
Recurrent VSCC
Consider long term follow up
Survival
Stage 1
80% 5 yr survival
Stage 2
50% 5 year survival
Stage 3
40% 5 year survival
Stage 4
40% 1 year survival
HPV Vaccination
Anticipated decrease as HPV16 is most common viral subtype associated with vulval cancer
Less dramati for other HPV--related malignancies - vulval dermatoses account for large proportion of vulval cancers
