Lymphatic malformation of the vulva

Case contributed by Dr Ashesh Ishwarlal Ranchod

Presentation

The patient presented with a recently noticed right labial mass.

Patient Data

Age: 2 years
Gender: Female

There is a serpiginous, lobulated right labial mass lesion. The lesion appears mildly dense on pre-contrast imaging, with a subtle low density and cystic appearance post contrast administration. The lesion is completely extrapelvic. There is no calcification.There Is perilesional induration. There are no hepatic, no lymphatic, no osseus or thoracic metastases.

Reformats confirm complete extrapelvic presentation and right labial involvement.

There is no regional bony infiltration, no congenital sacral abnormality.

Pathology

Complete excision histology confirmed a lymphatic malformation (cystic lymphangioma) with fat necrosis and organizing inflammation.

Case Discussion

The child presented urgently after hours and an opportunity to perform an MRI under general anesthetic or sedation was limited. The pediatric surgeon on call requested CT imaging with a view to urgent surgical excision. The clinical discussion was to identify the mass lesion and stage the patient at the same time, especially should the lesion be an occult aggressive neoplasm (Rhabdomysosarcoma etc). Multiphasic CT imaging was thus performed.

The lymphatic malformation is mildly hyperdense due to blood and or proteinaceous fluid. With contrast administration, the poorly appreciated serpiginous cystic appearance alludes to the histologically confirmed diagnosis of a lymphatic malformation. There is no calcification and no fat content on CT imaging. The lesion is completely extrapelvic and confined to the right labia majora.

Lymphatic malformations have an incidence ranging from 1:6000-16000 live births. The majority of cases are pediatric and under 2 years of age. They can occur anywhere however lymphatic malformations of the vulva(labia) are extremely rare.

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