摘要
乳房外Paget氏病臨床上是以紅色、脫屑的斑塊伴隨有燦爛、結痂來表現。色素性乳房外Paget氏病是一種罕見的變異型，在臨床及病理上與惡性黑色素瘤很難區分。我們報告一個位於生殖部的色素性乳房外Paget氏病的案例。一位58歲的男性從5年前開始注意到陰囊有一紅色、脫屑的斑塊，其上并有一黑色的區域。經由組織病理及免疫組織化學染色證實為乳房外Paget氏病，伴隨有黑色素細胞的增加。肥大的腫瘤細胞具有大的細胞核及淡染的細胞質，單個或成群的座落在表皮上。腫瘤細胞以細胞角質-7，癌胚抗原及mucicarmine染色呈現陽性反應。細胞質內并有多量的黑色素。散佈在腫瘤之間的則是S-100及HMB-45染色呈現陽性反應的樹狀細胞。在非色素性的乳房外Paget氏病做免疫組織化學檢查，則只有少量的S-100染色呈現陽性反應的細胞分布在表皮的基底層。乳房外Paget氏病的色素性，是因為伴隨著表皮內腫瘤細胞，造成黑色素細胞的增生，或是在Paget細胞及黑色素細胞內累積大量的黑色素所造成。黑色素細胞及腫瘤細胞的交互作用至今尚未完全了解。
關鍵字
無資料並列摘要
Extramammary Pagets disease (EMPD) usually presents as an erythematous, scaly patch with crusts, or superficial erosions. Pigmented EMPD is a rare variant of EMPD that may mimic malignant melanoma clinically and histopathologically. We report a case of pigmented EMPD on the genital region. The 58-year-old man had an erythematous, scaly patch with a pigmented macule on the scrotum for 5 years. Histopathological and immunohistochemical examinations of the pigmented region confirmed EMPD with melanocytes colonization and melanin accumulation within Paget cells. Plump tumour cells with a large nucleus and pale cytoplasm proliferated singly or in nests in the epidermis. They are positive for CK-7, carcinoembryonic antigen, and mucicarmine stains. Abundant melanin within the cytoplasm was also noted, Scattering among the tumor cells were many s-i oo and HMB-45 positive dendritic cells with a large amount of melanin. Immunohistochemical studies of non-pigmented sites showed that S-100 and HMB-45 positive cells were sparse and only detected on the basal layer of the epidermis. Pigmented EMPD may result from the proliferation of melanocytes accompanying the intraepidermal neoplastic cells or/and from accumulation of melanin within the cytoplasm of Paget cells and melanocytes. The interaction between melanocytes and tumor cells are still incompletely understood.
延伸閱讀
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