Vulvar High-Grade Squamous Intraepithelial Lesion in Primary Lymphedema With Severe Genital Lymphorrhea: A Case Report.
Qing Xie, Zhiying Chen, Johnson Boey et al.
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Case in brief
A 37-year-old woman with childhood-onset primary lymphedema developed recurrent right leg and genital swelling after 31 years of clinical quiescence, with vulvar erythema, follicular lesions, and persistent lymphorrhea. Indocyanine green lymphography confirmed combined genital and lower-extremity lymphedema. During surgery, abnormal perineal tissue was excised and lymphaticovenular anastomosis was performed; histopathology unexpectedly showed multifocal vulvar high-grade squamous intraepithelial lesion with lymphangiectatic change. HPV genotyping detected HPV16 and HPV42. After surgery, lymphorrhea decreased substantially and leg volume fell by 9.5%.
What made this case unusual
Multifocal vulvar HSIL was found unexpectedly in excised tissue from a patient with primary genital lymphedema and severe lymphorrhea, where chronic swelling and skin changes could obscure the premalignant lesions.
Diagnostic / clinical pearl
In chronic genital lymphedema, persistent or morphologically abnormal vulvar lesions warrant histopathological assessment; edema-directed surgery does not replace definitive oncologic treatment.