伴粘液癌的侵袭性外阴Paget病1例

　　A case of Invasive Vulval Extramammary Paget Disease with a Mucinous Carcinoma Component

　　S. Asaka¹, H. Ota², M. Iwaya¹, A. Yoshizawa¹, K. Sano¹, T. Honda¹

　　¹Department of Laboratory Medicine, Shinshu University Hospital, ²Department of Biomedical Sciences, School of Health Sciences, Shinshu University School of Medicine, Matsumoto, Japan

　　Background: Extramammary Paget disease (EMPD) is a rare skin neoplasm that is characterized by intraepidermal proliferation of Paget cells. In advanced stage, it can invade the dermis typically with nodular, micronodular, or glandular pattern. We report an extremely rare case of EMPD that invades the dermis with a mucinous carcinoma component.

　　Case: An 80-year-old woman presented to our hospital with vulvar itching and pain that had developed over the past 7 years. Physical examination showed a vulvar and perianal erythematous plague with pigmentation and a subcutaneous nodule in the left labia majora. Preoperative skin biopsy of the lesion revealed intraepidermal proliferation of Paget cells. Surgical excision of the affected area followed by skin reconstruction was performed. Histological examination of the resected skin revealed an infiltration of Paget cells forming solid nest with mucinous stroma in the labial dermis. Immunohistochemically, tumor cells were positive for CK7, CEA, MUC2 and MUC5AC, but negative for CK20, GCDFP-15, MUC6 and CDX2. No underlying associated internal and urogenital malignancy was identified. The diagnosis of invasive EMPD with a mucinous carcinoma component was made.

　　Conclusion: Vulvar and perianal EMPD and mucinous carcinoma are frequently associated with underlying genitourinary and colorectal carcinoma. For its precise diagnosis, close clinicopathological correlation, systemic work-up and immunohistochemical evaluation should be performed. Primary cutaneous mucinous carcinoma arising on the back ground of EMPD of the vulva and perineum is extremely rare and only 2 such cases were reported. Present case may represent an early stage of mucinous carcinoma development from EMPD.

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