[A clinicopathological study of 11 patients with Paget's disease of the vulva]

A clinicopathological analysis of 11 patients with Paget's disease of the vulva was reported. A close association of this disease with cancer was strongly suggested in the histological examination. Nine of 11 patients had wide primary lesion; 4 patients had coexisting undifferentiated carcinoma (one patient with in situ sweat gland carcinoma) and one patient had squamous cell carcinoma in other lesion of the vulva. Five patients had downward growth of the tumor from the epidermis proper. There were, thus, only two patients who had Paget's cells confined within the epidermis. Three patients died of generalized metastasis of carcinoma and two patients had vulvar recurrence. The results of staining for PAS, PAS diastase resistant reaction, alcian blue, mucicarmin and carcinoembryonic antigen (CEA) were all positive in specimens with similar stainings to coexisting undifferentiated carcinoma of the vulva and metastasized lesions. CEA staining was positive in cytoplasm of Paget's cells and basal cells widely in the epidermis. It could be considered that the Paget's cells of the vulva originated multifocally from primitive stratum germinativum, which gave rise to the surface epithelium as well as all of the dermal appendages.