Pathologists, Department of Pathology, Brigham and Women's Hospital, Boston, MA.
Am J Dermatopathol. 2024 Apr 1;46(4):195-203. doi: 10.1097/DAD.0000000000002496. Epub 2023 Dec 13.
Cutaneous sebaceous neoplasia comprises a spectrum of disease ranging from benign adenomas to malignant carcinomas. The hallmark of these lesions is sebaceous differentiation. However, poorly-differentiated sebaceous carcinoma (SC), which lacks significant overt sebaceous differentiation, can show morphologic overlap with a variety of other basaloid cutaneous neoplasms. The accurate classification of SC is essential not only for diagnosis, but also because of the potential association with Muir-Torre syndrome. Androgen receptor (AR) is a sensitive, but not entirely specific immunohistochemical marker that has been used for the diagnosis of SC. PReferentially expressed Antigen in MElanoma (PRAME) demonstrates strong cytoplasmic labeling of mature sebocytes and has been reported to be expressed in a variety of sebaceous neoplasms, including in the basaloid cell component. Therefore, we sought to compare the diagnostic use of cytoplasmic PRAME expression with that of AR for the distinction of SC from a cohort of basaloid cutaneous mimics; namely basal cell carcinoma, basaloid squamous cell carcinoma, pilomatricoma, cutaneous lymphadenoma, and extra-mammary Paget disease. We report that cytoplasmic PRAME expression is uncommon in poorly differentiated SC, and although specific, it shows very low sensitivity (22%). In contrast, AR was moderately sensitive (66%) and highly specific (92%) for the distinction of SC from basaloid mimics. These attributes, in addition to the nuclear expression of AR in the sebocytic and basaloid components of SC, suggest that AR is superior to PRAME for the diagnosis of SC.
皮肤的皮脂腺肿瘤包括了一系列疾病,从良性腺瘤到恶性癌。这些病变的标志是皮脂腺分化。然而,分化不良的皮脂腺癌(SC),缺乏明显的明显的皮脂腺分化,可能与各种其他基底细胞皮肤肿瘤存在形态学重叠。SC 的准确分类不仅对诊断很重要,而且还因为与 Muir-Torre 综合征有关。雄激素受体(AR)是一种敏感但不完全特异的免疫组化标志物,已用于 SC 的诊断。PReferentially expressed Antigen in MElanoma(PRAME)显示成熟皮脂腺的细胞质标记强烈,已报道在各种皮脂腺肿瘤中表达,包括基底样细胞成分。因此,我们试图比较细胞质 PRAME 表达与 AR 在区分 SC 与一系列基底样皮肤模拟物(即基底细胞癌、基底样鳞状细胞癌、毛发基质瘤、皮肤淋巴腺瘤和乳腺外 Pagets 病)方面的诊断用途。我们报告称,细胞质 PRAME 表达在分化不良的 SC 中不常见,尽管具有特异性,但敏感性非常低(22%)。相比之下,AR 在区分 SC 与基底样模拟物方面具有中等敏感性(66%)和高度特异性(92%)。除了 AR 在 SC 的皮脂腺和基底样成分中的核表达外,这些属性表明 AR 优于 PRAME 用于诊断 SC。