Only 27 cases of Paget's disease of the groin have been reported to the present. Our aim was to describe the clinical behavior and treatment of thais disease A retrospective analysis of patients seen at the Mayo Clinic over a period of 25 years (January of 1970 to December of 1995) was undertaken. We included patients with lesions of the groin (isolated or associated with penile/scrotal locations) and with histologic confirmation of the diagnosis. We found seven patients all male three patients had isolated lesions. Their mean (SD) age was 73 (8.3) years. Special stains and immunohistochemistry confirmed the diagnosis in all patients. A wide local excision was performed in every patient. Three patients had recurrence; one of them died with multiple pulmonary metastases 4 years later. Two patients presented with history of associated malignancy (prostatic and renal cell carcinoma), Paget's disease of the groin is extremely infrequent. Its origin seems not to be a simple extension from the genital area. Most diagnoses can be made by light microscopy. Wide local excision with free margins is the treatment of choice local recurrence occurs in half of patients with tumor-free margins by frozen section; long-term follow-up is warranted. Distant metastases occur rarely, although they can be fatal.
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