VULVAR MALIGNANCY

PRESENTED
BY
DR K.N.GEORGEWILL
 SYNOPSIS
• ANATOMY OF THE VULVA
• INTRODUCTION
– EPIDEMIOLOGY OF VULVAR MALIGNANCY
• CLASSIFICATION
• PATHOLOGICAL DESCRIPTION
• DIFFERENTIALS
 ANATOMY OF THE VULVA
• Vulva is the external genital organ of the female.
• Comprises:
– Mons veneris – fatty tissue, covered by skin
and over the symphysis pubis.
– Labia majora – a paired fold of skin with fat
pad, which extends posterioinferiorly from the
mons to surround the pudendal cleft, and
decreases in size posteriorly and unite
posteriorly across the midline in front of the
anus. Their outer surface has hair, inner
surface has no hair, but has sweat and
sebaceous glands
– Labia minora – a pair of skin fold with no fat,
no hair, few sweat and sebaceous glands, it is
split anteriorly to form the prepuce over the
clitoris and frenulum inferior to the clitoris,
posteriorly the pair unite to form the frenulum of
the labia.
– Vestibule – the area of the vulva enclosed by
the labia minora, it contains 6 openings
(urethra, vagina and the ducts of the skene’s
and Bartholin’s glands bilaterally)
– Clitoris – extremely sensitive erectile structure.
– Arterial supply: internal pudendal and femoral
arteries
– Lymphatic drainage: the vulva drain initially to
the superficial inguinal nodes and there after to
the deep inguinofemoral chain and there on to
the pelvic (iliac) nodes – central vulvar
structures drain bilaterally, whereas unilateral
vulvar structures drain to the ipsilateral nodes
primarily. The clitoris and other anterior central
vulvar structures may drain directly to the iliac
nodes.
– Innervation: pudendal and perineal nerves
 INTRODUCTION

• Cancer of the vulva may arise from the skin,

subcutaneous tissues, glandular elements of the
vulva, or the mucosa of the lower third of the
vagina
• It is uncommon, accounting for about 5% of
gynaecologic cancers.
• Approximately 90% of these tumors are sq cell
or epidermoid cancers
• It is primarily a disease of the elderly. Peak
incidence in the 60s. Average age @ time of
diagnosis is 65yrs
• Cause is unknown
• There appear to be at least two subsets of
patients with precursors for vulvar carcinoma:
– patients with VIN, especially younger women
– older patients who do not have VIN
• Vulvar dystrophies (lichen sclerosis)
• obesity
• Poor perineal hygiene
• Dm
• HT
• chronic granulomatous veneral dx (LGV, GI)
• The association between squamous cell carcinoma
of the cervix or vagina and squamous carcinoma of
the vulva is well established and has been reported
in 6% to 15% of cases
 CLASSIFICATION OF VULVAR
MALIGNANCIES

• Squamous cell cancer (epidermoid cancer)

• Carcinoma of Bartholin’s gland
• Basal cell cancer
• Malignant melanoma
• Sarcoma
• 2nd metastasis (8%): advanced CA cervix,
vaginal, ovarian, endometrial ca, other Ca
(kidney, urethra, bladder, breast )
 SQUAMOUS CELL CARCINOMA
• Histologic Subtypes:(varients)
– Basaloid carcinoma
– Warty (condylomatous) carcinoma
– Verrucous carcinoma
– Giant cell squamous carcinoma
– Spindle cell squamous carcinoma
– Acantholytic squamous cell carcinoma (adenoid
squamous carcinoma)
– Lymphoepithelioma-like carcinoma
– Metatypical basal cell carcinoma
(basosquamous carcinoma)
– Sebaceous cell carcinoma
• Most common vulvar malignancy (90%)
• Most frequently involve the anterior half of the
vulva.
• Mainly invovle the labia majora & minora (65%),
clitoris invovled in 25%
• Is bilatral (midline tumors) in 1/3 of cases
• Tumor appearance varies from a small ulcer
crater superimposed on a dystrophic lesion of
vulva skin to a large exophytic cauliflower- like
lesion
• There does not appear to be a +ve correlation b/w
the gross appearance of the tumor & either
histologic grade or frequency of nodal metastasis
SPREAD:
– Lymphatic vessels: primary route of spread- superficial
inguinal, deep femoral & external iliac lymph nodes.
Contralateral spread may occur as a result of rich
intercommunicating lymphatic system of the vulva.
– Direct / local spread to adjacent structures: vagina,
urethra or bladder
– Embolization to lymph nodes
– Haematogenous spread: primarily for sarcomas
STAGING:
FIGO STAGING OF VULVAR CARCINOMA (1995)
STAGE DESCRIPTION

0 (CIS) Carcinoma in-situ, VIN

I (T1 N0 M0) Lesion < 2cm confined to vulva or perineum, lymph

node negative
IA Stromal invasion < 1mm

IB Stromal invasion > 1mm

II (T2 N0 M0) Lesion > 2cm confined to vulva or perineum, lymph

node negative
III (T3 N0 M0, Tumour of any size with spread involving the lower
T1 N1 M0, T2 urethra/or vagina or anus/or +ve unilateral regional
N1 M0, T3 N1 nodes
M0)
STAGING CONT.

IV A (T1 N2 M0, Tumour involving upper urethra or bladder mucosa

T2 N2 M0, T3 N2 or rectal mucosa or pelvic bone /or bilateral
M0, T4 N1/N2 regional lymph node involvement
M0
IV B (Any T, Any Distant metastasis with +ve pelvic lymph node
N, M1 involvement
 DIFFERENTIALS

– Viral warts (condylomata acuminata)

– Paget’s disease of the vulva
– Vulvar dystrophy/dermatosis
– Benign ulcerative lesions (syphilis, herpes,
granuloma inguinale)
– Granular cell myoblastoma
– Fibroma
– Lipoma
– Neurofibroma
– Keratoacanthoma
– Other types of vulvar malignancies
 ADENOCARCINOMA OF VULVA
• Exceptionally rare unless it arises from Bartholin’s
gland or urethra
• Carcinoma of Bartholin’s gland accounts for about
1% of vulvar cancers
• Difficult to differentiate by clinical examination a
tumor of Bartholin’s gland from a benign
Bartholin’s cyst
• B/C of the location of the gland deep in the
substance of the labium, a tumor may impinge
upon the rectum & directly spread into the
ischiorectal fossa & Via lymphatic channels into
the deep pelvic nodes
 BASAL CELL CARCINOMA OF VULVA
• Accounts for 2-3% of vulvar cancers but
approximately 65% of all nonvulvar cutaneous
malignancies
• Tumor derived from the primordial basal cells in
the epidermis or hair follicle
• In the vulva involve almost exclusively the skin of
the labia majora
• Slow growing & locally invasive with well defined
inifiltrative margin
• Have a tendency to be multiple; therefore, the
finding of a single basal cell epithelioma on the
vulva should prompt a search for basal cell
lesions elsewhere on the skin
 MALIGNANT MELANOMA
• Account for about 5-9% of vulvar malignancy
• Tumor derived from melanin secreting cells
(melanocytes) in the skin. may arise either in a
preexisting mole or from apparently normal skin
• Tumor commonly involve the labia minora, majora
& clitoris and are typically solitary lesions that may
or may not be pigmented. They have a tendency
for superficial spread toward the urethra & vagina
• Presents as a pigmented, slightly raised lesion at
the mucocutaneous junction. Tumor spread
primarily lymphatic, has early tendency for
metastasis
 SARCOMA OF THE VULVA
• Account for < 2% of vulvar malignancies
• The most common variety is the
leiomyosarcoma
• Clinically, tumor may be a subcutaneous
nodule or may be exophytic & fleshy
 PAGET’S DISEASE OF THE VULVAR
• This skin disease was first described by Sir
James paget in 1874.
• It affected the nipple and areola of the breast
and was associated with an underlying breast
carcinoma.
• Vulvar Paget's disease appears as a reddish
pink area interlaced with dotted white patches
(hyperkeratotic epithelium).
• It is virtually confined to postmenopausal white
women.
• It is of apocrine origin & associated with an
adenocarcinoma.
 LICHEN SCLEROSIS
• Lichen sclerosis: chronic granulomatous lesion of
the vulva (non-neoplastic) involving the
pudendum in a figure of 8 manner.
• Xterized by thin white plaques on the affected
region (clitoris, labia minora, inner aspects of
labia majora & skin around the anus. It does not
involve the vestible, vagina or anal canal)
• Pathology: loss of skin supports
– Epidermal atrophy: loss of rete ridges
– Dermal edema & collagen hyalinization
– Subdermal chronic inflam cell infiltrate