Hoyle Aoisha, Davies Kerry, Rajan Neil, Melly Lucy
Department of Pathology, Southern General Hospital, Glasgow, UK.
Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
J Cutan Pathol. 2018 Oct;45(10):760-763. doi: 10.1111/cup.13311. Epub 2018 Jul 19.
Low-grade spiradenocarcinoma is a rare skin tumor, with fewer than six reported cases, arising in the context of CYLD cutaneous syndrome (CCS; syn: Brooke-Spiegler syndrome [BSS]). We report two independent cases of spiradenocarcinoma arising in a 50-year-old man with CCS. The tumors grew rapidly, prompting clinical excision. The histologic features in our cases corresponded to the salivary gland type basal cell adenocarcinoma-like pattern, low grade (BCAC-LG), that has been previously recognized as a recurrent finding in CCS. We performed genetic testing of the patient and found a novel mutation in CYLD. Recognition of low-grade spiradenocarcinoma can be difficult for the pathologist as the features can be subtle, especially in those cases where the transition from benign to malignant tumor is gradual. We examined p63 and smooth muscle actin (SMA) expression patterns in BCAC-LG and compared it with the expression pattern in the precursor spiradenoma. Our report provides information on two rare tumors in the context of CCS and suggests that the pattern of p63 and SMA staining may aid pathological detection of the BCAC-LG subtype of spiradenocarcinoma.
低级别汗腺癌是一种罕见的皮肤肿瘤，报告病例少于6例，发生于CYLD皮肤综合征（CCS；同义词：布鲁克-施皮格勒综合征[BSS]）背景下。我们报告了两例独立的、发生于一名50岁患有CCS男性的汗腺癌病例。肿瘤生长迅速，促使进行临床切除。我们病例中的组织学特征符合涎腺型基底细胞腺癌样模式，低级别（BCAC-LG），这是先前在CCS中反复发现的特征。我们对患者进行了基因检测，发现CYLD存在一种新的突变。对于病理学家来说，识别低级别汗腺癌可能很困难，因为其特征可能很细微，尤其是在那些从良性肿瘤向恶性肿瘤逐渐转变的病例中。我们检测了BCAC-LG中p63和平滑肌肌动蛋白（SMA）的表达模式，并将其与前体汗腺腺瘤中的表达模式进行了比较。我们的报告提供了CCS背景下两种罕见肿瘤的信息，并表明p63和SMA染色模式可能有助于汗腺癌BCAC-LG亚型的病理检测。
