Vulvar Malignancy

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, T1 N1 M0, T2 N1 M0, T3 N1 M0)

Tumour of any size with spread involving the lower urethra/or vagina or anus/or +ve unilateral regional nodes

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 involvement N, M1

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