Abstract
1. A cytologic and clinicopathologic analysis of 6 patients with vulvar extramammary Paget's disease was reported.
2. The disease was seen predominantly in the older age group: The median age at onset was 65.7 years. Clinically, the lesion appeared erythematous (4 patients) to pigmentary (one patient), and all patients complained of itching. One patient was found to have the tumor formation into the vulva.
3. A close association of the Paget's disease with cancer was evident by considering that in addition to extramammary Paget's disease of the vulva, 3 of 6 patients had primary lesion in multiple locations; two patients had underlying adenocarcinoma and one patient had squamous cell carcinoma in other lesion of the vulva. Two patient had downward growth of the tumor from the epidermis proper. There were thus only one patients who had Paget's cells confined within the epidermis.
4. The prognosis of this disease was not so good, because one patient died of coexisting underlying adenocarcinoma of the vulva and two other patients had local recurrence.
5. Cellular smears obtained by scrapping and contactpressure technique from the vulvar lesion revealed unsatisfactory cytologic detail and only two cases before surgery showed a positive results, although moderate hyperkeratosis was present in 4 of 6 cases before primary surgical therapy. But we had a positive cytological diagnosis in all of two patients with local recurrence after surgery.
6. The cytological characteristics of vulvar extramammary Paget's disease was similar to that of Paget's disease of the nipple such as described by others. Abnormal cells were characterized by enlarged nuclei, both central and peripheral in location, with an increased nuclear/cytoplasmic ratio, granular nuclear chromatin with abnormal distribution, and prominent nucleoli. A lack of squamous keratinization was common to all specimens. In two cases, “cell in a cell” and “crescent formation” were observed. A small amount of melanine granule was found in the cytoplasm in one case, which indicated the necessity to differentiate extramammary Paget's disease from malignant melanoma.
