关键词: BCC MCC cCS heterologous pilomatrix carcinoma porocarcinoma rare skin tumors

来  源:   DOI:10.3390/dermatopathology11030022   PDF(Pubmed)

Abstract:
A cutaneous carcinosarcoma (cCS) is a rare and aggressive skin cancer characterized by both carcinomatous (epithelial) and sarcomatous (mesenchymal) components, making it a biphasic tumor. Despite its occurrence in various organs, a cCS is exceptionally rare in the skin, predominantly affecting older males. The etiology of a cCS is unclear, but it may originate from a single progenitor cell capable of dual differentiation or from a collision of carcinoma and sarcoma cells. Clinically, a cCS presents as a rapidly growing, painful, ulcerated nodule or plaque on sun-exposed skin, with a high risk of local invasion and metastasis. Histopathologically, a cCS includes various epithelial components, such as squamous cell carcinoma and basal cell carcinoma, along with undifferentiated sarcomatous components resembling atypical fibroxanthoma. The tumor may also exhibit heterologous differentiation like angiosarcomatous or rhabdomyosarcomatous features. We present three cases of a cCS, highlighting their clinical and histological characteristics and comparing them with previously reported cases. Understanding a cCS is complicated by its rarity and diverse presentation, emphasizing the need for further research to elucidate its pathogenesis and optimal management.
摘要:
皮肤癌肉瘤(cCS)是一种罕见的侵袭性皮肤癌,其特征是癌(上皮)和肉瘤(间充质)成分。使其成为双相肿瘤.尽管它发生在各种器官中,cCS在皮肤中非常罕见,主要影响老年男性。cCS的病因尚不清楚,但它可能起源于能够双重分化的单个祖细胞或来自癌和肉瘤细胞的碰撞。临床上,cCS呈现为快速增长的,痛苦,暴露在阳光下的皮肤上的溃疡结节或斑块,具有较高的局部侵袭和转移风险。组织病理学,cCS包括各种上皮成分,例如鳞状细胞癌和基底细胞癌,连同类似非典型纤维黄瘤的未分化肉瘤成分。肿瘤还可以表现出异源分化,如血管肉瘤或横纹肌肉瘤特征。我们介绍了三例cCS,强调其临床和组织学特征,并将其与以前报道的病例进行比较。理解cCS因其稀有性和多样化的表现而变得复杂,强调需要进一步研究以阐明其发病机制和最佳管理。
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