Tumoral and Hamartomatous Diseases of the Vulva

Vulvo-vaginal malignant tumors are uncommon in children but early diagnosis and prompt, effective treatment improves prognosis. Here, we will cover malignant tumors of the vagina.

Tumoral Diseases
Any tumor may involve the genital area; some are present at birth (teratoma) and others can be seen in prepubertal and pubertal girls (intraepithelial neoplasia). The diagnosis of teratoma may at times be made by ultrasound in utero. It should be emphasized that the first symptoms of sarcoma of the vagina are often vaginal discharge and bleeding and sometimes a small polypoid mass that fills, from time to time, the vaginal orifice.

These symptoms require endoscopic exploration of the vagina for diagnosis as early as possible. Carcinoma in situ, epidermoid carcinoma, and adenocarcinoma of the vulva are exceedingly rare in children before the age of 16 years. Benign tumors of the vulva include epithelial inclusion cysts, paraurethral cysts, Bartholin's duct cysts (epidermal cysts), hidradenitis, hymeneal tags, hydroceles of the vulva, lipomas, syringomas, myoblastomas, and others.

Fig. 2. Psoriasis: red well-demarcated symmetrical plaques.

Clinical examination and biopsy, and if necessary surgical resection, allow the diagnosis. In neurofibromatosis type 1, freckling in the inguinal region is a diagnostic criterion, plexiform neurofibromas may be present in the perineal area, and clitoral involvement can be confused with ambiguous genitalia. Nevus may be observed in the perineal area. Congenital nevus can be small or part of giant congenital nevus; surgical treatment is required to reduce the risk of malignant melanoma.

Hemangiomas and Vascular Dysplasia May Involve the Perineal Region
Hemangiomas are the most common benign tumors of infancy. Cutaneous ulceration is the most frequent complication with hemangiomas and the perineum is the most frequent site of this complication, in particular with perianal lesions due to maceration. Ulcerations occur most commonly during the rapid proliferation phase of the hemangiomas (before 6 months of life). Ulcerated lesions are very painful, and analgesic therapy is always quite necessary. The use of occlusive dressings and the judicious use of topical and systemic analgesics should be considered on an individual basis for patients with ulcerated hemangiomas (fig. 3). Topical therapy with barrier creams, antimicrobials, and vaseline gauze is often useful. Laser therapy (flash-pumped pulsed dye laser) can be tried as it has been effective in some cases. The use of systemic steroids should be considered when large areas are ulcerated in association with an important subcutaneous component of the hemangioma.

Fig. 3. Ulcerated hemangioma.