Iwaya Mai, Uehara Takeshi, Yoshizawa Akihiko, Kobayashi Yukihiro, Momose Masanobu, Honda Takayuki, Ota Hiroyoshi
Department of Laboratory Medicine, Shinshu University Hospital, Matsumoto, Nagano, Japan.
Am J Dermatopathol. 2012 Dec;34(8):e139-45. doi: 10.1097/DAD.0b013e3182590ec1.
Primary signet-ring cell/histiocytoid carcinomas of the eyelid are extremely rare tumors considered to originate from sweat glands. Here, we report the case of a 72-year-old man diagnosed with primary signet-ring cell/histiocytoid carcinoma of the eyelid and present immunohistochemical analyses of the eyelid apocrine gland (Moll gland) and apocrine and eccrine sweat glands of perineum and axilla. Widespread infiltration of tumor cells with signet-ring cell or histiocytoid appearance was observed in his left eyelid, orbit, and periocular lesion. Tumor cells expressed mucins and showed immunoreactivity that was similar to that of the Moll gland: MUC6(+), GlcNAcα1→4Gal→R(-), MUC2(-), MUC5AC(-), GCDFP15(+), CD15(+), S100(-), CK7(+), CK20(-), ER(+), PgR (+), HER2(-), E-cadherin(+), p63(-), PSA(-), and TTF-1(-). The tumor cells differed from those of perineal and axillary apocrine and eccrine sweat glands, which were MUC6(-). The Moll gland was ER(-) and PgR(-), whereas perineal and axillar apocrine sweat glands were ER(+) and PgR(+), and perineal and axillary eccrine sweat glands were ER(+) and PgR(-). The tumor showed characteristics similar to that of the eyelid Moll gland, which is demonstrated to be an apocrine gland with a protein expression distinct from that of other apocrine glands. MUC6 and GCDFP15 expression are useful in identifying the Moll gland immunophenotype and GCDFP15, ER and PgR expression are useful in distinguishing primary eyelid signet-ring/histocytoid carcinoma from gastrointestinal malignancies.
原发性眼睑印戒细胞/组织细胞样癌是极其罕见的肿瘤，被认为起源于汗腺。在此，我们报告一例72岁男性被诊断为原发性眼睑印戒细胞/组织细胞样癌的病例，并展示对眼睑顶泌汗腺（Moll腺）以及会阴部和腋窝的顶泌汗腺和小汗腺的免疫组化分析。在其左侧眼睑、眼眶及眼周病变中观察到肿瘤细胞呈印戒细胞或组织细胞样外观并广泛浸润。肿瘤细胞表达黏蛋白，其免疫反应性与Moll腺相似：MUC6(+)、GlcNAcα1→4Gal→R(-)、MUC2(-)、MUC5AC(-)、GCDFP15(+)、CD15(+)、S100(-)、CK7(+)、CK20(-)、ER(+)、PgR(+)、HER2(-)、E-钙黏蛋白(+)、p63(-)、PSA(-)、TTF-1(-)。肿瘤细胞与会阴部和腋窝的顶泌汗腺及小汗腺的细胞不同，后者MUC6(-)。Moll腺ER(-)、PgR(-)，而会阴部和腋窝顶泌汗腺ER(+)、PgR(+)，会阴部和腋窝小汗腺ER(+)、PgR(-)。该肿瘤表现出与眼睑Moll腺相似的特征，Moll腺被证实是一种顶泌汗腺，其蛋白表达与其他顶泌汗腺不同。MUC6和GCDFP15表达有助于识别Moll腺免疫表型，而GCDFP15、ER和PgR表达有助于鉴别原发性眼睑印戒/组织细胞样癌与胃肠道恶性肿瘤。
