类似血管肉瘤的转移性鳞状细胞癌并发营养不良大疱性表皮松解症。

Dermatologica Pub Date : 1991-01-01 DOI:10.1159/000247803
J A McGrath, O M Schofield, B J Mayou, P H McKee, R A Eady
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引用次数: 28

摘要

我们报告一个患有广泛性隐性营养不良大疱性表皮松解症(RDEB)的患者,他发展为3个鳞状细胞癌。肿瘤同时出现在两个上肢肢端,分化较差。尽管手术和放疗,患者在6个月内死于转移性疾病。该病例突出了RDEB并发症的许多典型特征,包括总体预后不良。特别令人感兴趣的是其中一个肿瘤的组织学,它造成了诊断困难:血红素和伊红染色提示血管肉瘤病理,但免疫细胞化学的使用证明肿瘤是鳞状细胞癌的起源。
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Metastatic squamous cell carcinoma resembling angiosarcoma complicating dystrophic epidermolysis bullosa.

We report a patient with generalized recessive dystrophic epidermolysis bullosa (RDEB) who developed 3 squamous cell carcinomas. The tumours appeared simultaneously at acral sites on both upper limbs and were poorly differentiated. Despite surgery and radiotherapy the patient died from metastatic disease within 6 months of presentation. This case highlights many of the typical features of this complication of RDEB, including the overall poor prognosis. Of particular interest was the histology of one of the tumours which caused diagnostic difficulties: haematoxylin and eosin staining suggested an angiosarcomatous pathology, but the use of immunocytochemistry proved that the tumour was a squamous cell carcinoma in origin.

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