Mesenchymal Lesions

Dermatofibroma

Dermatofibromas are rare on the vulva (Fig. 16). They are usually slow-growing solitary reddish brown lesions that are flat or slightly raised, typically less than 1 cm in diameter. Characteristically, a "dimple sign" may be seen, a central umbilication upon squeezing the lesion. Histologically, the lesion is well circumscribed, with histiocytes and spindle cells interspersed with collagen bundles. Xanthoma cells and multinucleated giant cells may also be present. Overlying epithelium may show acanthosis and increased pigmentation (Table 16) (22).

2ZS&

Figure 16 Dermatofibroma: benign-appearing fibroblasts in a collagenous background. The lesions also contain histiocytes, and Touton giant cells may be seen in some cases.

Figure 16 Dermatofibroma: benign-appearing fibroblasts in a collagenous background. The lesions also contain histiocytes, and Touton giant cells may be seen in some cases.

Table 16 Dermatofibroma

Diagnosis

Symptoms

Clinical appearance

Differential diagnosis Therapy

Dermatofibroma Usually

Small flat or nodular lesion, reddish brown

Pigmented lesions including nevus, melanoma Dermatofibrosarcoma protuberans

Excision none

Hemangioma (De Morgan's Spots) and Senile Telangiectasiae

Small capillary hemangiomas (cherry angiomas, De Morgan's spots) of the vulva are not unusual in adults, particularly in the fair-skinned elderly (Fig. 17A). They are usually asymptomatic, but may rarely enlarge and bleed with pregnancy (30). Congenital capillary hemangiomas of the vulva may also occur in children and follow the usual history of growth, a static period, and then involution in most cases. Rarely, the potential for significant hemorrhage can occur with a larger congenital hemangioma (31). Histologically, cherry angiomas are characterized by a dermal proliferation of endothelial-lined vessels (Fig. 17B). Senile telangiectasiae occur with increasing frequency with age, and are more common in Caucasian women. They are blue-purple smooth-surfaced lesions, and occasionally rupture with trauma and bleed (Fig. 17C) (Table 17).

Caucasian Vulva

Figure 17 (A) Hemangioma: raised lesions which are red in color are seen. (B) Hemangioma: the lesion is composed of multiple dilated vascular spaces. (C) (See color insert) Senile telangiectasiae.

Clinical

Differential

Diagnosis

Symptoms

appearance

diagnosis

Therapy

Hemangioma

Usually none.

Red to purple dome-

None

None in most cases. If

May rarely bleed

shaped papules in

bleeding, can

adults

electrodessicate

Angiokeratoma

Angiokeratomas are uncommon on the vulva (Fig. 18). These benign lesions usually occur under the age of 50 and present as multiple, small purple unilateral papules. Histologically, the lesions comprise dilated vessels in the papillary dermis. The overlying epidermis shows hyperkeratosis, acanthosis, and papillomatosis. Degenerative changes may be seen in the perivascular elastic tissue. The vessels may be throm-bosed (Table 18) (32).

Pyogenic Granuloma (Granuloma Pyogenicum)

Pyogenic granulomas occur in various regions of the body, and hence may be seen on the vulva (Fig. 19). They are usually single lesions with a tendency to occur in pregnancy. Grossly they are pink-red papular or nodular lesions, which may be ped-unculated. The surface may ulcerate. Histologically, there is often central ulceration with the formation of a peripheral epithelial collarette as the epithelium grows downwards (22). The lesion is characterized by proliferation of capillaries, hence the other name, lobular capillary hemangioma, and histologically resembles granulation tissue. It is thought to occur secondary to trauma or a prior irritant condition rather than representing true neoplasm (Table 19) (33).

Lymphangioma Circumscriptum

Lymphangiomas of the vulva are rare, and may be either superficial or deep (Fig. 20). They may be either congenital or acquired. Superficial lesions (lymphangioma circumscriptum) are translucent or flesh-colored to pale papules, nodules, or vesicles. Lymphangioma circumscriptum may be congenital. For acquired lesions, the mean

Medical Vulva
Figure 18 Angiokeratoma: sis and hyperkeratosis.

cavernous vascular spaces in the papillary dermis with acantho-

Table 18 Angiokeratoma

Diagnosis

Symptoms

Clinical appearance

Differential diagnosis

Therapy

Angiokera- Usually none, Multiple purple Infectious and toma but may have papules inflammatory lesions, bleeding, < 1 cm other vascular lesions pruritus, pain (hemangioma, lymphangioma, pyogenic granuloma), various benign and malignant epithelial tumors (32)

None, or excision, electrodesicca-tion or laser therapy age is about 42, but it affects a wide age range (34). The deeper lesions form an ill-defined mass with normal-appearing overlying epithelium. Histologically lym-phangiomas are composed of dilated endothelial-lined spaces, and the lumens may contain lymphoid cells, red blood cells, or both. There may be overlying hyperkera-tosis and acanthosis of the squamous epithelium (34). Valvular structures may be seen in the dilated channels, distinguishing the lesion from hemangioma (Table 20) (22).

Angiomyofibroblastoma

Angiomyofibroblastomas of the vulva are most often seen in reproductive age or early postmenopause (Fig. 21A-C) (20). They are well circumscribed and may be clinically interpreted as a Bartholin's duct cyst or lipoma (17,35). Histologically, this benign lesion is characterized by a mass composed of hyper- and hypocellular areas containing spindle-to-round cells and delicate vessels in a collagenous or loose edematous background. Stromal cells typically stain for vimentin, desmin, ER, and PR and are negative for smooth muscle action (SMA), but there may be overlap with the staining pattern of aggressive angiomyxoma. These lesions must be distinguished histologically from aggressive angiomyxoma (see below). Vessels are more frequent and less likely to be hyalinized in angiomyofibroblastomas, and the stroma is more cellular (36). This and the circumscription of angiomyofibroblastoma, absent in aggressive angiomyxoma,

Figure 19 Pyogenic granuloma: granulation tissue, with numerous new vessels and abundant inflammatory cells.

Table 19 Pyogenic Granuloma

Diagnosis Symptoms Clinical appearance Differential diagnosis Therapy

Pyogenic None Often pedunculated Condyloma acuminatum, Excision granuloma nodule, red granulation verrucous carcinoma, tissue-like appearance hidradenoma aid in the distinction. There are sporadic case reports that suggest that rarely angio-myofibroblastoma may undergo sarcomatous transformation (Table 21) (20).

Aggressive Angiomyxoma

These lesions are locally aggressive and infiltrative, but metastasis is exceptional (Fig. 22) (20). They may be interpreted as a cyst on clinical examination. Grossly, they have a rubbery or gelatinous cut surface. Histologically, the lesion is poorly circumscribed—an important distinction from angiomyofibroblastoma. The cellular-ity is low—a distinction from the more cellular angiomyofibroblastoma. The histology of aggressive angiomyxoma is one of bland spindle cells in a myxoid matrix. Collagen and smooth muscle cells may cuff thick hyalinized vessels. Extra-vasated erythrocytes may be seen in the stroma (36). The lesions may be positive for desmin and actin (17), as well as ER and PR (20). Paravaginal origin with caudal extension may present at the vulva (Table 22).

Granular Cell Tumor

Granular cell tumors are usually benign solid lesions that can occur in a variety of locations on the body, but are seen in the vulva in about 10% of cases (Fig. 23) (37). They may be multiple, and may involve other organs as well (38). They are unencapsulated, which probably contributes to the tendency to recur locally, and rare malignant cases have been described (39,40). The name relates to the microscopic "granular" appearance of the sheets of large polygonal, spindle, or round cells with abundant eosinophilic cytoplasm. Although there have been multiple theories as to the cell of origin, granular cell tumor is now believed to most likely be of Schwannian derivation, and stains for s100 protein and CD 68. Treatment is

Figure 20 Lymphangioma: dilated lymphatic vascular spaces, some containing lymphocytes, are seen.
Table 20 Lymphangioma Circumscriptum

Diagnosis

Symptoms

Clinical appearance

Differential diagnosis

Therapy

Lymphangioma

Often none.

Small pale

Condyloma

None, or electro-

circumscrip-

May see

papules if

acuminatum,

desiccation, excision,

tum

oozing of

superficial,

blistering

laser therapy or

fluid, pain,

mass if deep

conditions

sclerosing therapy

pruritus,

for small lesions,

swelling,

excision for

cellulitis

large ones

Table 21

Angiomyofibroblastoma

Clinical

Diagnosis

Symptoms appearance

Differential diagnosis

Angiomyofi-broblastoma

Mass lesion Well circumscribed, < 5 cm

Clinical: Bartholin's cyst, lipoma. Pathological: aggressive angiomyxoma, fibroepithelial polyp, cellular angiofibroma

Excision

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