Recurrence and rapid metastasis formation of a granular cell tumor of the vulva.

Histologically confirmed local recurrence of the rare entity of a granular cell tumor of the vulva was diagnosed in a 55-year-old patient with no signs of distant metastasis. Intraoperatively (local excision of mons pubis and inguinal lymphnodes), widespread regional metastasis with retroperitoneal lymphnode metastases were found. Postoperative restaging detected pulmonary, hepatic and skeletal metastases and the patient died of her disseminated disease within 4 months. Early diagnosis of the malignant phenotype of granular cell tumor using expression of S-100-protein, MIB-1, vimentin and p53 is suggested. Once a malignant granular cell tumor is diagnosed histologically, thorough staging procedure should be performed to exclude disseminated disease. If this is the case, radical surgery should be tried due to the lack of efficiency of radiotherapy and of systemic treatments.