Fernandez-Flores Angel, Cassarino David S
Department of Cellular Pathology, Hospital El Bierzo, Ponferrada, Spain.
Biomedical Investigation Institute of A Coruña (INIBIC), Group of Translational Investigation in Cellular Comunication and Signaling (CellCOM-SB), A Coruña, Spain.
J Cutan Pathol. 2018 Jul;45(7):508-514. doi: 10.1111/cup.13151. Epub 2018 Apr 25.
Pilomatrix (pilomatrical) carcinoma is a rare cutaneous adnexal tumor with matrical differentiation and recurrent and metastatic potential. Sarcomatoid pilomatrix carcinoma is a rare variant which shows a sarcomatoid component intermingling with the epithelial one. There are only 4 cases previously published. We present an additional case on the hand of a 78 year-old man which appeared as a 6 mm hyperkeratotic, focally ulcerated plaque. A shave biopsy demonstrated a dermal infiltrative neoplasm, composed of markedly atypical-appearing basaloid cells with focal necrotic/ghost cells, as well as an intimately associated population of atypical oval to spindle-shaped cells. Both the epithelial and the sarcomatoid components expressed cytokeratins (CKs) AE1/AE3, CK 5/6 and CAM 5.2, as well as beta-catenin and LEF-1. The tumor failed to express CK7, CK20, S100, thyroid transcription factor 1 (TTF1), CDX2, prostate-specific antigen (PSA) and CD34. The tumor was completely excised with Mohs surgery, and there has been no recurrence in the 8 months of follow-up to date.
毛母质（毛母质瘤性）癌是一种罕见的皮肤附属器肿瘤，具有毛母质分化以及复发和转移潜能。肉瘤样毛母质癌是一种罕见的变异型，其肉瘤样成分与上皮成分相互混杂。此前仅发表过4例病例。我们报告1例发生于一名78岁男性手部的病例，表现为一个6毫米的角化过度、局部溃疡的斑块。削切活检显示为真皮浸润性肿瘤，由明显非典型的基底样细胞组成，伴有局灶性坏死/鬼影细胞，以及与之密切相关的一群非典型椭圆形至梭形细胞。上皮成分和肉瘤样成分均表达细胞角蛋白（CKs）AE1/AE3、CK 5/6和CAM 5.2，以及β-连环蛋白和淋巴细胞增强因子-1（LEF-1）。肿瘤不表达CK7、CK20、S100、甲状腺转录因子1（TTF1）、尾型同源盒转录因子2（CDX2）、前列腺特异性抗原（PSA）和CD34。该肿瘤通过莫氏手术完全切除，在迄今8个月的随访中未出现复发。
