Introduction/Background Lymphangioma is a benign lymphatic malformation defined by a dilatation of the lymphatic vessels. The vulvar location is very rare, yet this benign skin lesion causes an obvious impairment of quality of life. The cause is still not well known, it can be congenital or secondary to a trauma of the lymphatic vessels. The association with a vulvar malignancy has never been reported in the literature.We report in this article the case of an extensive vulvar lymphangioma circumscriptum associated with a squamous cell carcinoma of the greater lip.

Methodology A 60-year-old female patient with a history of diabetes and total hysterectomy for uterine myomas consulted for the appearance of an extensive vesicular lesion of the right labia associated with a suspicious 1.5 cm lesion of the right labia.

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Abstract 2022-RA-978-ESGO Figure 1

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Abstract 2022-RA-978-ESGO Figure 2

Results Biopsy of the lesions concluded to be a vulvar lymphangioma associated with squamous cell carcinoma of the right labia. The tumour was classified as FIGO stage IA and the patient had a total vulvectomy with bilateral inguinal lymphadenectomy. The evolution was without recurrence of either tumour or lymphangiomatous disease after 6 months.

Conclusion Vulvar lymphangioma is a troublesome condition that may prompt a search for an underlying obstructive cause when acquired. It poses a problem of diagnosis and management. The particularity of our case is the presence of an associated micro invasive squamous cell carcinoma of the vulva.