Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma.

Aggressive angiomyxoma of pelvic soft parts is a rare lesion with a high risk of recurrence. We report 10 cases of angiomyofibroblastoma, a hitherto uncharacterized benign tumor of the vulva histologically mimicking aggressive angiomyxoma. All patients had a vulval mass, often clinically diagnosed as a Bartholin's cyst. There was no recurrence after excision. The tumors were well circumscribed, measuring 0.5-12 cm in maximum dimension. They were characterized by alternating hypercellular and hypocellular edematous zones in which abundant blood vessels (predominantly of the capillary type) were irregularly distributed. Spindled, plump spindled, and oval stromal cells were aggregated around the blood vessels, sometimes forming solid compact foci, or were loosely dispersed in the hypocellular areas. Their nuclei were bland, but rare ones were enlarged and hyperchromatic in four cases. Some cells had abundant eosinophilic hyaline cytoplasm and eccentrically placed nuclei. Mitotic figures were absent or very sparse. Scattered throughout were thin, wavy strands or thick bundles of collagen. Mast cells were readily seen in eight cases. Immunohistochemically, the stromal cells were reactive for vimentin and desmin, but not cytokeratin, muscle-specific actin, alpha-smooth muscle actin, or S-100 protein. Ultrastructural studies showed well-developed rough endoplasmic reticulum, Golgi apparatus, abundant intermediate filaments, and pinocytotic vesicles in the stromal cells. Angiomyofibroblastoma can be distinguished from aggressive angiomyxoma by its circumscribed borders, much higher cellularity, more numerous blood vessels (which lack prominent hyalinization), frequent presence of plump stromal cells, minimal stromal mucin, and rarity of erythrocyte extravasation.     

Angiomyofibroblastoma of the female urethra

BACKGROUND: Angiomyofibroblastoma is a relatively recently described rare tumor of the superficial soft tissues. To date, 57 cases of angiomyofibroblastoma of the external genitalia in women have been reported. METHODS/RESULTS: We describe a case of a 24-year-old woman who presented with the urinary stream flowing out in the posterior direction and whose diagnosis was a urethral tumor, angiomyofibroblastoma. CONCLUSIONS: Angiomyofibroblastoma has a potential arising from the female urethra as well as other areas of the external genitalia.     

Angiomyofibroblastoma of the Vulva: Report of a Case

A 45-year-old woman was first seen by us 2 years after becoming aware of a slightly painful subcutaneous mass in her left vulva. The mass was 7.5 × 3.0 cm in size, well circumscribed, mobile, and rubbery. It was at first clinically considered to be a benign tumor. Microscopically, the resected mass was composed of spindle or polygonal tumor cells which were cellularly or hypocellularly arranged with perivascular accentuation in a mucoid or fibrocollagenous background. Immunohistochemically, myxoid tumor cells were positive for vimentin but not for α-smooth muscle actin, CD34, CD31, desmin, or S-100 protein. The tumor was diagnosed as an angiomyofibroblastoma (AMBF), based on the typical findings of histology and immunohistochemistry. There are many histological types of vulvar tumors, and establishing a preoperative diagnosis is difficult in many patients. Rapid intraoperative pathological diagnosis should be performed if possible, considering the possibility of diseases such as AMFB and aggressive angiomyxoma (AAM). When AAM is suspected, the peripheral tissues should also be resected to prevent recurrence. Received: May 22, 2000 / Accepted: November 20, 2000     

Mesenchymal lesions of the vulva

Mesenchymal lesions of the vulva may be either regionally specific to the vulva or soft tissue neoplasms that can occur in multiple locations on the body. They may have overlapping histological and immunohistochemical features and, in routine practice, are not likely to be encountered, adding to the diagnostic difficulty. A review of mesenchymal vulvar lesions is presented.     

Benign tumors of the vulva

The varied manifestations of benign vulvar tumors are reviewed to re-acquaint physicians with the disease, since less than 200 cases have been reported in the literature. A classical case is presented in detail, clinically and pictorially.Sarcoma of the vulva may be prevented if benign vulvar simple lesions and tumors are recognized and treated early.If this treatise serves its purpose, there should be more correct diagnoses, and fewer benign lesions allowed to proceed to a stage of malignant degeneration.     

Lymphoepithelioma-like carcinoma of the vulva

Lymphoepithelioma is well known in the nasopharyngeal region. Although this neoplasm is well described in a variety of extranasal sites, where it is termed lymphoepithelioma-like carcinoma, this is only the second report of this neoplasm originating in the vulva reported in the literature. These lesions are reported to be less aggressive in extranasal sites. A 40-year-old woman presented with a lymphoepithelioma-like carcinoma of the vulva, diagnosed by excisional biopsy, which had been present for 1 month. The lesion persisted with ipsilateral lymph node involvement 4 months after initial resection, when the patient returned for definitive surgery. Lymphoepithelioma-like carcinoma can occur rarely in the vulva, and may behave aggressively.     

Benign papular lesions of the vulva

The vulva may develop conditions more familiar to dermatologists than to gynecologists. Although gynecologists may be familiar with some of these lesions, a review of these benign and often pruritic papular lesions may be helpful when receiving a diagnosis more commonly seen in dermatologic practice.