Background. Verrucous carcinoma of the vulva in a patient with Turner syndrome is extremely rare.
Case report. A 7.5 x 4 cm exophytic tumor of the vulva with a palpable ipsilateral groin lymph node developed in a 41-year-old single milligravid Japanese woman with short stature and a history of primary amenorrhea. Analysis of karyotype demonstrated mosaic Turner syndrome. She underwent total deep vulvectomy with en-block inguinal femoral lymphadenectomy. Histopathological finding of the extirpated specimen demonstrated verrucous carcinoma with clear margins and the absence of metastasis to the lymph nodes. There was no sign of HPV genome detected in the carcinoma tissue. Conclusion. This seems to be the first case of vulvar verrucous carcinoma in a patient with Turner syndrome. (C) 2003 Elsevier Inc. All rights reserved.