1. Lichen sclerosus and Lichen planus and risk for malignancy
Maija Jakobsson
MD, Ph.D, Consultant
Helsinki University Hospital
Finland

2. Employer Helsinki University Hospital
Conflict of interest
Employer Helsinki University Hospital
No conflict of interest regarding the contents of this presentation

3. Risk factors for vulvar cancer Case reports, LS/ LP
Contents
ISSDV terminology
Risk factors for vulvar cancer
Case reports, LS/ LP
Differentiated VIN- dVIN
Finnish register study
Conclusion

4. Two pathways for vulvar cancer
High risk HPV negative
LS or LP
dVIN
SCC
High risk HPV positive
LSIL
HSIL
SCC
30%?
3- 9%

5. LSIL of the vulva HSIL of the vulva DVIN 1986 ISSVD 2003 WHO
2012
LAST
ISSVD 2015
WHO 2014
Vulvar Intraepithelial Neoplasia (VIN)
Intraepithelial Lesion
Vulvar Squamous Intraepithelial Lesion (SIL)
Squamous type
(with or whitout HPV change)
Flat condyloma or HPV effect
Low grade SIL-LSIL
(VIN 1)
LSIL of the vulva
(vulvar LSIL, flat condyloma, or HPV effect)
- VIN 1
- VIN 2
- VIN 3
(Formely Squamous cell CIS, Bowen’s disease, Erytroplasia of Queyrat, CIS simplex)
VIN usual type
-VIN warty type
-VIN basaloid type
-VIN mixed type (warty / basaloid)
High grade SIL- HSIL
(VIN 2, VIN 3)
HSIL of the vulva
Differentiated VIN
VIN, differentiated type
DVIN
Non-Squamous type
Paget’s Disease
Melanoma in situ Modified from Bornstein

6. What are the main risk factors for vulvar cancer ?

7. Risk factors for vulvar cancer
1.3 million women aged 49-65
898 vulvar cancers (ICD code C51)
Cox regression models
Risk factors:
Prior CIN3 (RR 2.68, CI )
2 % of all vulvar cancers
Obesity (RR 1.71, CI )
Menopause <50 yrs ( RR 1.52, CI )
Coffey et al 2016

8. A clinical study of 23 cases of female anogenital carcinoma
age 43-83
all had vulvar symtoms prior to diagnosis
1-30 years:
8 prior diagnosis of LS/ LP
12 clinical signs of LS
3 of LP
5 some canges both LS and LP
2 VIN3
1 no vulvar skin disease
Derrik 2000

9. How big is the risk of cancer with LS?
253 women, 6 developed cancer (2.4%) (66 months) Cooper 2004
211 women with 3 (1.4%) SCC (1 yrs 8 months) Carli 1995
107 women, 7 had co-excisting SCC
1/ 92 (1.0 %) developed SCC in the follow-up
Hart 1975
32 symtomatic LS women, 9 % developed VIN lesions, 21 % invasive SCC (mean 4 years) Carlson 1998

10. Lichen planus and vulvar cancer?
Some case reports Ramos-e Siva 2000
38 patients with LP planus accociated vulvar cancer
All were HPV-negative
Regauer 2014

11. Cumulative incidence of SCC among LS patients
Maaike 2016

12. LS OR DVIN? LS without progression (n=61)
Revision of ‘Lichen Sclerosus (et atrophicus)’ and ‘vulvar dystrophy’ by two expert pathologists
Data between
Minimum follow-up of 10 years
LS without progression (n=61)
in 58 cases (95%), diagnosis was unchanged
LS with progression to SCC (n=60)
in 25 cases (42%) were reclassified to dVIN
time to progression for dVIN 28 months
for LS 84 months (p=0.001)
van de Nieuwenhof 2011

13. Does compliance of the treatment decrease the risk of SCC?
Prospective, longitudinal cohort study in 507 women with biopsy confirmed LS
Follow-up from 2008 to 2014
Age 55.4 (range 18-86)
Duration of follow up 4.7 yrs (range )
Induvidualised regiments for corticosteroids defined by degree of hyperkeratosis
150 patients (29.6%) did not carry out adviced treatment (= partially compliant)
Lee. JAMA Dermatol.2015

14. Dergree of keratinisation

15. Outcome
Compliant
(n=357)
Partially compliant
(n=150)
P value
SCC or VIN
SCC
7 (4.7%)
3 (2.0%)
<0.001
Progression of adhesions or scaring
12 (3.4%)
60 (40.0%)
Symptom resolution
333 (93.3%)
87 (58.0%)
Improved in dyspareunia
194 (93.7%)
68 (65.4%)
Adverse effects
Corticosteroid dermatitis
8 (2.2%)
6(4.0%)
0.37
Atrophy
4 (1.1%)
0.43