Congenital lymphangioma circumscriptum of the vulva

Abstract

Lymphangioma circumscriptum is a rare benign disorder of unknown etiology affecting lymph vessels in the deep dermal and subcutaneous layers. The commonest sites of presentation are the axilla, shoulders, groins, tongue and oral cavity. Lymphangioma circumscriptum of the vulva is very rare can be either congenital or secondary to lymphadenectomy or pelvic radiation in patients with genital cancer, genital tuberculosis, Crohn's disease, cellulitis, morbid obesity and immobilization. We report the case of congenital lymphangioma circumscriptum of the vulva in an adolescent with congenital lymphedema of the leg.

Unexpected Axillary Lymph Node Uptake During Lower-Limb Lymphoscintigraphy in a Case With Vulvar Lymphangioma Circumscriptum

“…Lymphangioma circumscriptum is a benign malformation of the dilated lymphatic vessels in the skin and subcutaneous tissue, which is characterized by the appearance of grouped or isolated small thin-walled vesicles filled with clear fluid. 1 The chest, proximal sites of extremities, and axilla are commonly affected sites, whereas vulvar presentation, as in our case, is uncommon and can be either acquired or congenital. 2 Surgical excision, which was lowest recurrence rate (17%), is recommended for treatment.…”

“…The liver and spleen show avid tracer uptake, indicating that radiotracer has passed freely into the systemic circulation. Lymphangioma circumscriptum is a benign malformation of the dilated lymphatic vessels in the skin and subcutaneous tissue, which is characterized by the appearance of grouped or isolated small thin-walled vesicles filled with clear fluid 1 . The chest, proximal sites of extremities, and axilla are commonly affected sites, whereas vulvar presentation, as in our case, is uncommon and can be either acquired or congenital 2 .…”

Primary Vaginal Lymphatic Malformation in A Child :Case Report and Literature Review

“…LMs can occur at any age, 65% are seen at birth, and 80% by 1 year [3] .LMs are rarely located in the female genital tract.To the best of our knowledge, there are only 14 reports of the primary lymphatic malformations related to perineum worldwide in children [4][5][6][7][8][9][10][11][12][13][14][15][16][17] (Table 1). Of these, most lesions involve the labia, only 3 patients had lesions involving the vagina [10,12,15] .…”