Simon Suzana Katalenić, Bolanca Ines Krivak, Sentija Karmela, Kukura Vlastimir, Valetić Josip, Skrtić Anita
Department of Gynecology and Obstetrics, Laboratory for Cytology and Clinical Genetics, 'Merkur" University Hospital, Zagreb, Croatia.
Coll Antropol. 2010 Jun;34(2):649-52.
Vulvar Morbus Paget (MP) represents a rare intraepithelial adenocarcinoma. It accounts for less than 1% of all vulvar neoplasia and usually appears in postmenopausal women. Histologically it is analogous to Paget's disease of the breast. The most common clinical symptom is pruritus. The lesion appears as an erythematous or as an eczematous lesion with islands of hyperkeratosis. Occasionally, single anaplastic Paget's cells can be found on the vulvar smears which make cytological diagnosis of the disease possible. However, the disease can be diagnosed only by biopsy. We present a case of 49-year old woman with vulvar symptoms of pruritus, who had liver and kidney transplantation two years ago. During the standard gynecological examination the vulvar smear was taken for cytological evaluation. The smear was scanty, with inflammatory background, overloaded with squamae. There were two types of cells: dysplastic squamous cells from lower layer of the epithelium and the single, anaplastic cells with a high nuclear:cytoplasmic ratio who possessed eccentric, large nucleus. Nucleoli were rare. Cytoplasm varied from pale and delicate to densely basophilic. Accordingly, cytological diagnosis vulvar intraepithelial neoplasia (VIN III) with differential diagnosis of vulvar Paget's disease was made. The pathological verification supported the diagnosis of MP and an immunohistochemistry panel confirmed type III of Paget's disease and an evaluation of bladder was suggested.
外阴佩吉特病(MP)是一种罕见的上皮内腺癌。它占所有外阴肿瘤的比例不到1%,通常发生在绝经后女性中。在组织学上,它类似于乳腺佩吉特病。最常见的临床症状是瘙痒。病变表现为红斑或湿疹样病变,伴有角化过度岛。偶尔,在外阴涂片上可以发现单个间变的佩吉特细胞,这使得对该疾病进行细胞学诊断成为可能。然而,该疾病只能通过活检来诊断。我们报告一例49岁有外阴瘙痒症状的女性病例,她两年前接受了肝肾移植。在标准妇科检查期间,采集了外阴涂片进行细胞学评估。涂片细胞量少,有炎症背景,有大量鳞状上皮细胞。有两种类型的细胞:来自上皮下层的发育异常鳞状细胞和单个间变细胞,核质比高,核偏心且大。核仁少见。细胞质从淡而细腻到深嗜碱性不等。因此,做出了外阴上皮内瘤变(VIN III)的细胞学诊断,并对外阴佩吉特病进行鉴别诊断。病理验证支持MP的诊断,免疫组化结果证实为佩吉特病III型,并建议对膀胱进行评估。