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CLINICAL VIGNETTE

A Case of Vulvar Extramammary

Pagets Disease

Angel a Ruman , M.D.

Case ReportA 56-year-old postmenopausal woman presented with

complaints of vulvar itching, and she noticed a well-demarcated reddish lesion on her vulva. She had been

seen three to four months before and had initially beentreated for an eczematous lesion with topical steroids.

She denied any vaginal discharge or history of recur-rent yeast infections. She reported no change in the

lesion or her symptoms with use of the topical steroids.On the genital exam she had a macular, solitary

reddish circular lesion on her right labia. She had noother gross abnormalities.

She was sent to dermatology clinic where she hada vulvar biopsy which established the pathologicdiagnosis of extramammary Paget's disease. She was

sent to OB/Gyn for further evaluation and treatment.

DiscussionIn 1874 Sir James Paget first described mammary

Paget's disease. It was not until 1889 that Crockerdescribed the first case of extramammary Paget's

disease affecting the scrotum and penis. In 1901

Dubreiuhl reported the first case of vulvar extramam-mary Paget's disease.1

Vulvar extramammary Paget's disease is a rare

condition that comprises only 1-2% of vulvar malig-nancies.2 In 1998 only 200 cases of vulvar extramam-

mary Paget's disease were reported in the literature. 1

Due to the disease's relatively low incidence, andsince many cases are unreported, its true incidence

remains unknown.3

Extrammammary Paget's disease occurs most

commonly in the sixth and seventh decades, with anaverage age of 63 years.3 It can be found in both men

and women; however, it is most commonly seen inpostmenopausal Caucasian females.2 Typically

involved sites are the vulvar, perianal, scrotal andpenile regions; rare sites include the thighs, buttocks,

axilla, eyelids and external ear canal.4

Grossly, the lesions can appear well defined,

moist and reddish.3 The lesions can be white to red,scaling or macerated. The lesions can appear infil-

trated, eroded or look like an ulcerated plaque.5

Patients can present with complaints of vulvar

pruritus, vulvar irritation, vulvar burning or they maynotice a vulvar lesion.3 The differential diagnosis for

extramammary Paget's disease includes psoriasis,contact dermatitis, fungal infections, lichen sclerosis,

intraepithelial neoplasia, and melanoma. The nonspe-cific clinical findings often lead to misdiagnoses.Many patients do not seek medical care immediately,

and those who do are often treated for other diseaseswith extended periods of topical steroids. An average

of one year can pass before a biopsy is taken anddefinitive diagnosis is made.4

Histologically, Paget's disease resembles Bowen'sdisease and superficial spreading melanoma.5 It is

characterized microscopically by the presence ofspecific tumor cells called Paget's cells. Paget cells

are large cells with pale clear cytoplasm, large roundhyperchromatic nuclei which tend to form clusters or

solid nests. Paget cells can be found at all levels of theepidermis. They can migrate within the epidermis in a

horizontal as well as vertical manner. Because of thistype of migratory pattern, there is a high local recur-rence rate (40%) of vulvar extramammary Paget's

disease after local excision.1

The pathogenesis of extramammary Paget's

disease remains controversial. An unusual feature ofextramammary Paget's disease and one that spurred

controversy regarding its histiogenesis is its associa-tion with underlying invasive apocrine gland carci-

noma. While mammary Paget's disease is almost

always associated with an underlying invasive ductalcarcinoma and is generally accepted as being ofunderlying apocrine gland and/or ductal origin, vulvar

extramammary Paget's disease is associated with anunderlying invasive apocrine gland carcinoma in only

about 25% of cases.1 Genitourinary, gastrointestinaland breast carcinomas comprise the majority of thecoexisting cancers.2 Some authors believe that Paget's

cells of extramammary Paget's disease originate in theepidermis and should be viewed as a form of

abnormal differentiation of epidermal stem cells. Theunderlying apocrine gland invasive carcinoma found

in 25% of cases should be viewed as a secondsynchronous primary malignancy. Other authors

believe that, with regard to pathogenesis, extramam-mary Paget's disease should not be differentiated from

mammary Paget's disease. The Paget's cells in bothextramammary Paget's disease and mammary Paget's

disease are likely to be of apocrine gland and/or ductorigin and extend into the epidermis by migration ormetastasis from the underlying apocrine glands and/or

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ducts.1 This theory seems to be the more popular one.Four histologic forms of vulvar extramammary

Paget's disease have been recognized.(Table 1)1

The treatment of noninvasive extramammary

Paget's disease is wide surgical excision as the diseaseusually extends well beyond the gross lesion.6 Frozen

sections are obtained to ensure adequate excision.Paget's disease of the vulva is almost always noninva-

sive and can oftentimes be managed by simplevulvectomy or wide local excision. Mohs micro-

graphic surgery has also been used as treatment.Among treatments for noninvasive vulvar extramam-

mary Paget's disease, vulvectomy has a 15% recur-rence rate, Mohs micrographic surgery has a 27%recurrence rate and wide local excision has a 43%

recurrence rate.4 The role of laser ablation in Paget'sdisease is controversial.7 It is best used for treatment

of recurrent disease. Other modalities that are

currently being investigated for treatment of noninva-sive extramammary Paget's disease are radiotherapy,topical chemotherapy and CO2 laser vaporization and

photodynamic therapy. These treatment modalitiesare not well suited, however, for treatment of extra-

mammary Paget's disease that is invasive, poorlydefined and multicentric.4 For invasive lesions,

radical vulvectomy and bilateral groin dissection havebeen used.1

ConclusionIt is important to recognize that not all vulvar lesionsare benign nor do they all respond to topical steroids.

While the incidence of vulvar extramammary Paget'sdisease is extremely low, if a lesion is not responding

to a current treatment a biopsy is necessary to makethe diagnosis.

If vulvar extramammary Paget's disease is diag-nosed surgical treatment is the current standard.

Long-term follow up is required to exclude recurrenceof the disease and development of associated cancer.

REFERENCES1. Piura B, Rabinovich A, Dgani R. Extramammary Paget's disease of

the vulva: report of five cases and review of the literature. Eur J

Gynaecol Oncol. 1999;20(2):98-101.

2. Parker LP, Parker JR, Bodurka-Bevers D, et al. Paget's disease of

the vulva: pathology, pattern of involvement, and prognosis. Gynecol

Oncol. 2000 Apr;77(1):183-189.

3. Pliskow S. Vulvar Paget's disease. Clinicopathological review of 14

cases.J Fla Med Assoc. 1990 Jul;77(7):667-671.

4. Zollo JD, Zeitouni NC. The Roswell Park Cancer Institute experi-

ence with extramammary Paget's disease. Br J Dermatol. 2000

Jan;142(1):59-65.

5. Habif TP. Clinical Dermatology: a Color Guide to Diagnosis and

Therapy, 3rd ed. St. Louis (MO): Mosby;1996.

6. Mehta NJ, Torno R, Sorra T. Extramammary Paget's disease. South

Med J. 2000 Jul;93(7):713-715.

7. Ryan KJ, editor.Kistner's Gynecology and Women's Health, 7th ed.

St. Louis (MO): Mosby;1999.

Intra-epidermal vu lvar extramammary Paget's dis ease

The basement membrane is intact and the Paget cells are

located and confined to the epidermis only. This form accounts

for approximately 75% of vulvar extramammary Paget'sdisease.

Minimally invasive vulvar extramammary Paget's disease

The Paget cells break through the basement membrane and

invade the underlying dermis less than 1 mm. This form is rare.

Invasive vulvar extramammary Paget's disease

The Paget cells break through the basement membrane and

invade the underlying dermis more than 1 mm. This form is

very rare.

Vulvar extramammary Paget's disease with an underly ing

apocrine gland adenocarcinoma

This form accounts for 25% of vulvar extramammary Paget's

disease. One study looked at histologic appearance as one

possible means of identifying patients with a poorer prognosis.

Patients with extramammary Paget's disease confined to the

epidermis had better outcomes than patients whose extramam-

mary Paget's disease invaded the dermis and those who had

an underlying adenocarcinoma.2

Table 1: Forms of Vulvar Extramammary Pagets Disease