BACKGROUND/ PURPOSE: Vascular tumors and malformations are rare. This large series describes vascular anomalies of the female genitalia and their management. METHODS: An international review board-approved retrospective database and a record review from 1994 through 2004 was conducted. RESULTS: Of the 3186 female patients with a vascular anomaly, 82 (2.6%) had a lesion in the external genitalia. There were 60 malformations and 22 tumors. The most common malformations were combined capillary-lymphaticovenous, venous, and lymphatic. Tumors included 20 infantile hemangiomas, 1 kaposiform hemangioendothelioma, and 1 kaposiform lymphangioendothelioma. The referring diagnosis was incorrect in 56% of patients. Cutaneous stains, swelling, deformity, bleeding, fluid leakage, or infection were the prominent symptoms. Magnetic resonance imaging, ultrasonography, angiography, and computed tomography were used for diagnostic clarification. Malformation treatment consisted of sclerotherapy, embolization, and operative resection. Tumor management included observation, surgical excision, and antiangiogenic pharmacotherapy. CONCLUSIONS: Vascular anomalies of the female external genitalia are uncommon, and the initial diagnosis is often inaccurate. Correct diagnosis using clinical and radiographic data is feasible and leads to meaningful intervention for these frequently devastating lesions. Whereas tumors may respond to excision or antiangiogenic drugs, malformations require ablation or resection. Evaluation and management of these lesions is complex and benefits from interdisciplinary care.
BACKGROUND/ PURPOSE:Vascular tumors and malformations are rare. This large series describes vascular anomalies of the female genitalia and their management. METHODS: An international review board-approved retrospective database and a record review from 1994 through 2004 was conducted. RESULTS: Of the 3186 female patients with a vascular anomaly, 82 (2.6%) had a lesion in the external genitalia. There were 60 malformations and 22 tumors. The most common malformations were combined capillary-lymphaticovenous, venous, and lymphatic. Tumors included 20 infantile hemangiomas, 1 kaposiform hemangioendothelioma, and 1 kaposiform lymphangioendothelioma. The referring diagnosis was incorrect in 56% of patients. Cutaneous stains, swelling, deformity, bleeding, fluid leakage, or infection were the prominent symptoms. Magnetic resonance imaging, ultrasonography, angiography, and computed tomography were used for diagnostic clarification. Malformation treatment consisted of sclerotherapy, embolization, and operative resection. Tumor management included observation, surgical excision, and antiangiogenic pharmacotherapy. CONCLUSIONS:Vascular anomalies of the female external genitalia are uncommon, and the initial diagnosis is often inaccurate. Correct diagnosis using clinical and radiographic data is feasible and leads to meaningful intervention for these frequently devastating lesions. Whereas tumors may respond to excision or antiangiogenic drugs, malformations require ablation or resection. Evaluation and management of these lesions is complex and benefits from interdisciplinary care.