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.

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.

Surgical resection is indicated for lesions that fail to respond to these therapies. An angiodysplasia can involve the perineal region, and careful clinical examination of the ano-vulvo-vaginal region and limbs with auscultation will help to classify the lesion. In this localization, isolated port wine stains, Klippel-Trenaunay syndrome, cystic lymphangiomas, and lymphangiodysplasia have to be recognized and treated. In cases of pelvic angiomatous lesions, a careful clinical examination must look for signs of associated external genital and/or anal abnormalities; it is important also to check for the presence of associated midline congenital lumbosacral skin lesions, as these could be markers of spinal dysraphism.

Infantile Perianal Pyramidal Protrusion
This term is used to describe a midline protrusion anterior to the anus (

fig. 4). It has probably been reported mainly as an acrochordon or a skinfold (skin tag, skin ruga and skin tab). Clinically, it presents characteristically as a pyramidal, smooth and red or rose-colored midline protrusion just anterior to the anus. The average age of the patient is 14 months and 94% of the patients are female. No etiological factors have been determined: there is no history of child abuse. At times infantile pyramidal protrusion has been reported in association with constipation. The time of onset of the protrusion is uncertain; it may enlarge over a few months, but more often, spontaneously regresses in size.

It is important to recognize this entity in order to differentiate it from diagnoses of sexual abuse, genital warts, granulomatous lesions of inflammatory bowel disease, or rectal prolapse.

Provided by ArmMed Media
Revision date: July 3, 2011
Last revised: by Jorge P. Ribeiro, MD