Acquired vulvar lymphangioma: risk factors, disease associations, and management considerations: a systematic review

Acquired vulvar lymphangioma (AVL) is not well-characterized. Diagnosis is delayed and the condition is often refractory to therapy.

The objective of this study was to provide a systematic review of AVL including risk factors, disease associations, and management options.

A primary literature search was conducted using 3 databases: PubMed, CINAHL, and OVID, from all years to 2022.

In total, 78 publications with 133 patients (48 ± 17 years) were included. Most studies were case reports/series. The most common disease association was prior malignancy (70 patients, 53% of cases) and inflammatory bowel disease (6 patients, 5% of cases). The most common malignancy was cervical cancer (57 patients, 43% of cases). Most patients had prior radiation or surgery, with 36% (n = 48) treated with radiation, 30% (n = 40) with lymph node dissection, and 27% (n = 36) with surgical resection. Common presenting symptoms included discharge/oozing, pain, and pruritus. Most patients underwent surgical treatment for AVL with 39% treated with excision, 12% with laser therapy (the majority used CO₂), and 11% with medical therapies. Most patients had failed prior therapies and there was a diagnostic delay.

Retrospective nature. Most studies were limited to case reports and case series, with interstudy variability and result heterogeneity.

AVL is an underrecognized entity and should be considered in patients with a history of malignancy or radiation to the urogenital area. Treatment should include multidisciplinary care and address underlying lymphatic changes, manage any existing inflammatory conditions, and utilize skin-directed therapies and barrier agents while addressing symptoms of pruritus and pain. Prospective studies are needed to further characterize AVL and develop treatment guidelines.