Rhabdoid-Tumor der Vulva - eine ungewöhnliche, zunächst fehlgedeutete hochmaligne Läsion

We report a case of malignant rhabdoid tumour of the vulva occurring in a 31 year-old woman. Clinically, the lesion was misinterpreted as a Bartholin's cyst. After histological confirmation of malignancy, a radical excision of the tumour with an extended regional lymphadenectomy was performed. Histologically, the neoplasm was originally misdiagnosed as the neoplasm was originally histologically misdiagnosed as a leiomyosarcoma. Immunohistochemical and electron microscopic studies were required for a definite histological diagnosis. Rhabdoid tumour is unusual, and most often presents as a highly aggressive renal tumour in children. Rare examples of extrarenal rhabdoid tumours of identical morphology, and with an equally adverse prognosis have been reported in young adults. There is an ongoing debate as to whether rhabdoid tumours represent a separate tumour entity, or rather a phenotype which may be acquired by various malignant lesions. To date, five rhabdoid tumours of the vulva, with a fatal outcome in three cases, have been reported in the world literature. In the present case, a local recurrence had to be removed only six months after initial radical surgery.