Department of Dermatology, University Hospital Zurich, University of Zurich, Switzerland.
Department of Pathology, Clinical Research and Practical Center for Specialized Oncological Care, Saint-Petersburg, Russia.
Am J Dermatopathol. 2023 Aug 1;45(8):544-548. doi: 10.1097/DAD.0000000000002465. Epub 2023 Jun 15.
Cuticular poroma is a rare variant of poroma composed of exclusively or predominantly cuticular cells, namely of large cells with ample eosinophilic cytoplasm. We report 7 cases of this rare tumor identified among 426 neoplasms diagnosed as poroma or porocarcinoma. The patients were 4 males and 3 females, ranging in age from 18 to 88 years. All presented with a solitary asymptomatic nodule. The location included knee (2 cases), shoulder, thigh, shin, lower arm, and neck (each 1). All lesions were surgically removed. No evidence of disease was observed in 5 patients with available follow-up (range 12-124 months).Microscopically, all neoplasms were composed of variably sized, focally closed packed, or interconnecting nodules constituted mostly of cuticular cells. Small poroid cells were a focal feature in 5 tumors, whereas in the remaining 2 cases, poroid cells with conspicuous but still in minority. Five neoplasms were somewhat asymmetric, with irregular outlines. Ductal differentiation and intracytoplasmic vacuoles were seen in 6 tumors. Other features variably encountered were conspicuous intranuclear pseudoinclusions, cystic change, occasional multinucleated cells, increased mitoses, and stromal desmoplasia. Four of the 5 tumors analyzed with next-generation sequencing yielded YAP1::NUTM1 fusions. In addition, various mutations, mostly of unknown significance were identified in one neoplasm.
角化性汗孔瘤是汗孔瘤的一种罕见变异型,主要由角质细胞组成,即具有丰富嗜酸性细胞质的大细胞。我们报告了在 426 例诊断为汗孔瘤或汗孔癌的肿瘤中发现的 7 例这种罕见肿瘤。患者为 4 名男性和 3 名女性,年龄 18-88 岁。所有患者均表现为单发无症状结节。病变部位包括膝关节(2 例)、肩部、大腿、胫骨、前臂和颈部(各 1 例)。所有病变均通过手术切除。5 例患者获得随访(随访时间 12-124 个月),均未发现疾病证据。显微镜下,所有肿瘤均由大小不等、局灶性紧密排列或相互连接的结节组成,主要由角质细胞组成。小汗孔细胞是 5 个肿瘤的一个局灶性特征,而在另外 2 个病例中,汗孔细胞虽然明显但仍属少数。5 个肿瘤有些不对称,轮廓不规则。6 个肿瘤可见导管分化和胞质内空泡。其他不同程度出现的特征包括明显的核内假包涵体、囊性变、偶尔多核细胞、增加的有丝分裂和基质玻璃样变。5 个肿瘤中有 4 个通过下一代测序检测到 YAP1::NUTM1 融合。此外,在一个肿瘤中还发现了各种突变,大多数为意义不明的突变。