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一种罕见的皮肤恶性肿瘤:左颞部原发性皮肤癌肉瘤伴附属器上皮成分(毛母质癌肉瘤)

A Rare Cutaneous Malignancy: A Primary Cutaneous Carcinosarcoma with Adnexal Epithelial Component (Trichoblastic Carcinosarcoma) of Left Temporal Region.

作者信息

Nagose Vaishali B, Ingle Sachin S, Chandak Nilesh O, Gondane Shirish R

机构信息

From the Department of Pathology, GMC, Sindhudurg, Maharashtra, India.

Department of Pathology, DUPMC, Jalgaon, Maharashtra, India.

出版信息

Indian J Dermatol. 2024 Jan-Feb;69(1):77-80. doi: 10.4103/ijd.ijd_243_23. Epub 2024 Feb 27.

Abstract

Primary cutaneous carcinosarcoma (PCCS) is a rare cutaneous biphasic tumour with just 120 cases being reported till date. The epithelial component trichoblastic carcinoma/ malignant trichoblastoma (trichoblastic carcinosarcoma) is even rarer, so is the present case. An eighty years old male patient presented with a rapidly progressing ulceroproliferative nodular growth on the left temporal region. The incisional biopsy showed primary cutaneous carcinomasarcoma (PCCS). IHC showed epithelial and stromal component marked exclusively for pancytokeratin (AE1/AE3) CK5&6/ p40 and vimentin respectively. Both components were immunopositive for D2-40 and immunonegative for BerEP4/ S100/ Melan A/ MITF, thus final diagnosis given was Trichoblastic carcinosarcoma. Wide excision of the lesion with local rotation flap and skin grafting (thigh skin) was done. Patient remains disease free after four years also. Present case thus highlights the importance of HPE, IHC and early surgery for excellent prognosis of PCCS.

摘要

原发性皮肤癌肉瘤(PCCS)是一种罕见的皮肤双相肿瘤,迄今为止仅报告了120例。其上皮成分毛母质癌/恶性毛母质瘤(毛母质癌肉瘤)更为罕见,本病例亦是如此。一名80岁男性患者左侧颞部出现迅速进展的溃疡增生性结节状肿物。切开活检显示为原发性皮肤癌肉瘤(PCCS)。免疫组化显示上皮和间质成分分别仅对全细胞角蛋白(AE1/AE3)、CK5&6/p40和波形蛋白呈阳性。两种成分对D2-40均呈免疫阳性,对BerEP4/S100/黑素A/MITF均呈免疫阴性,因此最终诊断为毛母质癌肉瘤。对病变进行了广泛切除,并采用局部旋转皮瓣和皮肤移植(大腿皮肤)。四年后患者仍无疾病。本病例突出了组织病理学检查、免疫组化和早期手术对PCCS良好预后的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05fa/10986867/3ceee44add8c/IJD-69-77-g001.jpg

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