Background

Acquired lymphangioma circumscriptum of the vulva is rare and can occur subsequent to malignancies of the anogenital and pelvic region. We sought to investigate the clinicopathologic characteristics of malignancy-associated acquired vulvar lymphangioma circumscriptum (AVLC).

Methods

We identified all cases of AVLC within our institution with history of prior malignancy between 2005 - 2021. A similar search was performed in the PubMed database to identify published cases to date. The clinical and histopathologic information was recorded.

Results

A total of 71 cases were identified. The most common preceding malignancy was cervical carcinoma (71.8%, 51/71). 91.4% (64/70) of patients underwent radiation therapy and 70.2% (40/57) underwent lymph node dissection. Median interval between the diagnosis of malignancy and AVLC was 10 years (range 0-32 years). AVLC frequently presented as vesicular (31.6%, 18/57) or verrucous (28.1%, 16/57) lesions clinically. Common treatments for AVLC included excision (53.1%, 26/49) and laser therapy (16.3%, 8/49) with an overall recurrence rate of 42.9% (24/56) at a median follow-up interval of 1.8 years (range 0.04-32.3 years).

Conclusion

AVLC is a rare, late complication of anogenital and pelvic malignancies causing debilitating physical symptoms and psychological stress. Further studies are warranted to determine the most effective treatment modalities to mitigate recurrence.

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