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汗腺螺旋瘤和汗腺螺旋圆柱瘤中的腺样囊性癌样模式:散发性肿瘤及与布鲁克-施皮格勒综合征相关肿瘤中的罕见特征。

Adenoid cystic carcinoma-like pattern in spiradenoma and spiradenocylindroma: a rare feature in sporadic neoplasms and those associated with Brooke-Spiegler syndrome.

作者信息

Petersson Fredrik, Kutzner Heinz, Spagnolo Dominic V, Bisceglia Michele, Kacerovska Denisa, Vazmitel Marina, Michal Michal, Kazakov Dmitry V

机构信息

Sikl's Department of Pathology, Charles University, Medical Faculty Hospital, Pilsen, Czech Republic

出版信息

Am J Dermatopathol. 2009 Oct;31(7):642-8. doi: 10.1097/DAD.0b013e3181a1573e.

Abstract

Spiradenoma is a benign, morphologically well-defined cutaneous adnexal neoplasm that is closely related to cylindroma. We present the rare occurrence of adenoid cystic carcinoma (ACC)-like areas in 7 spiradenomas and 1 spiradenocylindroma, not described in the English literature to date. The ACC-like areas were a minor but significant component in all lesions and were usually multifocal and blended with the conventionally appearing parts of the neoplasms. The ACC-like areas were typified by cribriform formations of epithelial cells concentrically arranged around gland-like spaces filled with mucin, homogeneous eosinophilic material, or granular basophilic material. In some neoplasms, only mucin occurred in these pseudoglandular structures, whereas in other cases, a combination of all 3 secretory products was encountered. Although well-developed bilayered glands with a demonstrable peripheral myoepithelial cell layer were not recognizable in the ACC-like areas, immunohistochemistry demonstrated myoepithelial differentiation in these portions of the tumors. When present in the ACC-like areas, ductal structures manifested a rather squamoid lining, without a recognizable peripheral myoepithelial cell layer. It is concluded that the ACC-like pattern, although a rare feature and of no clinical consequence, is a distinctive finding in a minority of cases and extends the morphological spectrum of spiradenoma and spiradenocylindroma occurring sporadically or in the setting of Brooke-Spiegler syndrome. It represents a potential diagnostic pitfall, particularly in a limited biopsy specimen where the changes may be misdiagnosed as ACC.

摘要

汗腺螺旋瘤是一种良性的、形态学上界限明确的皮肤附属器肿瘤,与圆柱瘤密切相关。我们报告了7例汗腺螺旋瘤和1例汗腺圆柱瘤中罕见出现腺样囊性癌(ACC)样区域的情况,这在迄今为止的英文文献中尚未有描述。ACC样区域在所有病变中是一个较小但显著的成分,通常为多灶性,并与肿瘤的常规表现部分相融合。ACC样区域以上皮细胞呈筛状排列为特征,围绕充满黏液、均匀嗜酸性物质或颗粒状嗜碱性物质的腺样间隙呈同心圆状分布。在一些肿瘤中,这些假腺样结构中仅出现黏液,而在其他情况下,则可同时见到所有3种分泌产物。虽然在ACC样区域中无法识别出具有明显外周肌上皮细胞层的发育良好的双层腺体,但免疫组化显示肿瘤的这些部分存在肌上皮分化。当ACC样区域出现导管结构时,其衬里表现为相当鳞状,无明显的外周肌上皮细胞层。结论是,ACC样模式虽然是一种罕见特征且无临床意义,但在少数病例中是一种独特的发现,扩展了散发性或布鲁克-施皮格勒综合征背景下的汗腺螺旋瘤和汗腺圆柱瘤的形态学谱。它代表了一个潜在的诊断陷阱,尤其是在有限的活检标本中,这些改变可能被误诊为ACC。

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