毛细胞癌的病例报告及诊断要点

Lewkowicz Milagros, Greco Alejandro, Lezcano Gabriel, Cachau Victoria, Díaz Florencia
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摘要

毛瘤样癌(PC)是一种罕见的伴有基质分化的附件恶性肿瘤。它的良性对应物(毛瘤)在日常病理实践中被诊断得更频繁。这两种实体都有共同的基因改变,但恶性对应物获得突变,使其发展为攻击性行为[1]。我们描述了一名33岁的男子,他在左耳出现7 x 6厘米的结节性溃疡病变,上个月生长明显加快。我们怀疑是鳞状细胞癌还是化脓性肉芽肿。组织学切片显示溃疡碎片被基底样细胞增殖浸润,其间穿插着一组“幽灵细胞”。肿瘤细胞呈不规则片状排列,边界呈浸润性。还发现了具有毛膜角化和坏死灶的鳞状细胞群。活检被诊断为具有毛基质分化的附件肿瘤,这表明由于存在侵袭性特征,其完全切除具有安全边际。随后对切除活检的研究显示出与先前描述的相似的特征。臭名昭著的是,发现了耳廓软骨的局灶性浸润,这使我们对毛基质癌的诊断毫无疑问。
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Case Report and Highlight Clues on the Diagnosis of Pilomatrical Carcinoma
Pilomatrical Carcinoma (PC) is a rare malignant adnexal tumor with matrical differentiation. Its benign counterpart (Pilomatrixoma) is diagnosed much more frequently in daily pathological practice. Both entities share genetic alterations but the malignant counterpart acquires mutations that make it develop an aggressive behavior [1]. We describe a 33-year-old man who presented with a 7 x 6 cm nodular ulcerated lesion in the left ear with markedly accelerated growth in the last month. Incisional biopsy was referred to us with suspicion of squamous cell carcinoma versus pyogenic granuloma. Histologic sections showed ulcerated fragments infiltrated by a basaloid cell proliferation interspersed with groups of “ghost cells”. The neoplastic cells were arranged in irregular sheets with infiltrative borders. Groups of Squamous cells with trichilemmal keratinization and foci of necrosis were also identified. The biopsy was diagnosed as an adnexal neoplasm with pilomatrical differentiation, suggesting its complete resection with safety margins due to the presence of aggressive characteristics. The subsequent study of the excisional biopsy showed similar characteristics to those previously described. Notoriously, focal infiltration of the auricular cartilage was identified, leading us to the undoubted diagnosis of pilomatrical carcinoma.
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