Pagets Disease of the Vulva Extramammary Pagets Disease

Paget's disease of the vulva presents as a velvety red lesion with overlying white plaques and may present with severe pruritis, burning, or bleeding (Fig. 31A and B).

Paget Disease The Vulva
Figure 28 (A) Neurofibromatosis: numerous lesions are seen on the mons pubis. (B) Neurofibroma: typical ''shredded carrot'' bundles of collagen in a myxoid background containing cells of neural origin.

Diagnosis

Symptoms

Clinical appearance

Differential diagnosis

Therapy

Neurofi-

None if small. Mass

Small brown nodules or

Other pig-

Excision if

broma

lesion if large. May

plaques. May be

mented

warranted

cause clitoral

pedunculated. May have

lesions

enlargement

a violaceous color

The lesion tends to recur, probably related to the fact that histologically the lesion extends beyond the grossly detectable borders. It is associated with both underlying skin adnexal and Bartholin's adenocarcinomas, as well as noncontiguous adenocar-cinomas of other organs such as the rectum or breast (58,59). The incidence of these

Figure 29 Vulvar intraepithelial neoplasia (VIN), usual type. (A) (See color insert) This VIN lesion is pigmented. (B) (See color insert) This is an example of a depigmented VIN lesion. (C) (See color insert) In the warty pattern, cytopathic features of human papilloma virus are prominent. (D) In the basaloid pattern, the entire epithelium is placed by small basaloid cells.

Figure 29 Vulvar intraepithelial neoplasia (VIN), usual type. (A) (See color insert) This VIN lesion is pigmented. (B) (See color insert) This is an example of a depigmented VIN lesion. (C) (See color insert) In the warty pattern, cytopathic features of human papilloma virus are prominent. (D) In the basaloid pattern, the entire epithelium is placed by small basaloid cells.

Table 29 Vulvar Intraepithelial Neoplasia, Usual Type

Clinical

Differential

Diagnosis

Symptoms

appearance

diagnosis

Therapy

Vulvar intraepithelial

None, pruritus

Widely

Wide, depends on

Excision,

neoplasia

variable

lesion color

imiquimod

carcinomas varies widely in different series, but these cancers have occurred in less than one-third of cases in most reports (60). Histologically, the large eosinophilic Paget cells may be seen in the basal layer of the epithelium, around skin appendages, and percolating up the epithelium (''Pagetoid spread''). Most cases of Paget's represent an in situ carcinoma; however, invasive Paget's disease may occur, with Paget cells invading the dermis. Extramammary Paget's disease may also represent a manifestation of an underlying adenocarcinoma of skin appendages such as sweat gland adenocarcinoma. Paget's disease must also be differentiated from pagetoid spread of urothelial or an anorectal carcinoma. All these variants of Paget's disease will appear similar, both clinically and histologically. Primary vulvar Paget's disease stains for CK7 and GCDFP-15, but rarely for CK20. Anorectal lesions stain for CK20 and are negative for GCDFP-15, and usually for CK7. Urothelial lesions stain for CK7 and CK20 and are negative for GCDFP-15 (61). Melanomas may also exhibit Pagetoid spread, but will stain for HMB-45, Melan-A, and S100 and be negative for cytokeratins (Table 31).

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