Epidemiology & Pathogenesis
Clinical
Dermoscopy
Histo
Progn/Management
Vulvar
Nevi
-2% of adult female; 23% of
pigmented vulvar lesions
-may present during
childhood/premenopausal
-median age of 28-33
-Atypical Melanocytic Nevi of
the Genital Type (AMNGT) 5%
of vulvar nevi
-median age of 17-26
-symmetric macules & papules
-pink to dark brownblack/blue
-well-demarcated with regular
borders & uniform pigment
-<1 cm
-located on labia majora,
minora, & clitoral hood
-AMNGT MC on labia minora
-may have alarming features
with dark pigment & irregular
borders & large size
-Globular &
homogenous
patterns
-AMNGT: mixed
pattern with 2 or
more features
-Benign though
conflicting data
regarding risk
marker for vulvar
melanosis
-AMNGT also
with low
malignant
potential
Vulvar
Melanosis
-AKA vulvar
lentiginosis
& vulvar
melanotic
macules
-68% of pigmented vulvar lesions
in reproductive-aged women
-median age 40-44
-Children think syndromes
(Peutz-Jeghers, Carney complex,
LEOPARD, Bannayan-RileyRuvalcaba, Dowling-Degos)
-Questionable relationship to
hormonal factors
-2nd MC malignancy of vulva after
SCC
-10% of vulvar malignancies
-2% of all melanomas in females
occur on vulvar mucosa
-MC in Caucasians in 5th-8th
decade
-less marked difference in
incidence across racial-ethnic
groups
-Molecularly resemble lentiginous
rather than cutaneous melanomas
with KIT mutated gene
-Single or multiple asymmetric
macules or patches w/
variable shades of tan to black
color, irregular and poorly
demarcated borders, varying
size
-located on mucosal surfaces;
MC labia minora
-MC pattern was
ringlike
-also
homogenous,
globular, parallel,
cobblestone,
reticular like
-Common with regularly
sized, evenly distributed
nests of melanocytes, no
cytologic atypia
-AMNGT: more alarming
w/ features overlapping
with melanoma except
exhibit dermal maturation,
rare mitotic activity, lack
of cell necrosis &
ulceration
-More difficult if overlap
with LSetA
-Increased melanin in
basal layer &
normal/slightly increased
number of melanocytes
arranged in single units at
DEJ
-macules, papules, or nodules
of irregular coloration,
asymmetric borders, diameter
> 7 mm
-amelanotic possible
-symptoms like vulvar
bleeding, pruritis, discharge,
irritation & lymphadenopathy
possible
-50% on mucosal skin
-Labia majora> labia minora >
clitorial hood
-Blue, gray, white
color plus
structureless
zones
-Many of mucosal
lentiginous subtype
though may be superficial
spreading or nodular
-Atypical melanocytes
arranged as confluent
nests & sheets, pagetoid
spread, absent dermal
maturation
-often diagnosed
late w/ poor
prognosis
-mean 5-year
survival ranging
from 27%-60%
-Older & AA
patients have
decreased
survival
-Txt surgical
-If localized no
difference in
survival w/
radical
vulvectomy vs.
conservative
wide local
excision
-Imatinib, Kit
inhibitor, may be
Vulvar
Melanoma
-Benign
-Clinical f/u &
baseline
photography
recommended
promising