Abstract
Cytologic investigations of three cases of vulvar Paget's disease were performed. In case 1 (78 year old), Paget's disease was present on the left labium majus with metastases around the urethral orifice, in the uterine cervix and the left inguinal lymphnodes. Two nodular tumors developed later subcutaneously at both sides of the clitoris. In case 2 (73 year old), Paget's disease was located on most of the right labium majus but without any underlying carcinoma of the sweat gland. In case 3 (87 year old), Paget's disease extended widely on the vulva and the medial femoral and perianal regions. A hen egg sized tumor was observed at the right labium majus with intradermally developed cancer. Metastases at the regional lymphnodes and the bones were detected.
Smears of the intraepidermal Paget's cells from the three cases showed almost similar cytologic features. Generally speaking, the features of the exfoliated cells from the intradermally invasive cancers and the metastatic cancers (in the tumor at the urethral orifice, the uterine cervix and the lymphnodes) had more atypia although they were still basically the same as those found in the intraepidermal Paget's cells.
1. Basic features of the intraepidermal Paget's cells were predominantly arranged in a flat sheet-like formation, occasionally showing scattered distribution and rarely showing papillary arrangement. The nuclei were enlarged and somewhat round. The nuclear rims were thin and smooth. The chromatin patterns were mainly finely granular but occasionally coarsely granular. The nucleoli were single, central, round, enlarged and reddish. Perinuclear halos were frequently noted. The cytoplasms Were abundant, cyanophilic, slightly thin, polyhedral or oval and showed a finely reticular structure.
2. Cytologic features of the cancer cells which had infiltrated the dermis and the cancer cells of the metastatic lesions showed prominent anisocytosis, more coarsely granular chromatin patterns and an increased number of nucleoli in addition to the basic features of Paget's cells.‘Cell within a cell’ or ‘double crescent nuclei with cell apposition’ were often observed and not infrequently there appeared huge multinucleated tumorous cells with a marked increase and irregular distribution of chromatin.
3. From the cytologic aspects, it was impossible to differentiate whether the Paget's cells were derived from squamous or glandular cells. However, the ultrastructural aspects suggested that the intraepidermal Paget's cells might be derived from the eccrine sweat glands.
