Greither A, Rehrmann A
Dermatologica. 1980;160(6):361-70.
The report concerns a southern Italian female patient of--at her death--67 years, who developed in 1975 within several months Spiegler basaliomas of the scalp. The tumors increased in size and number, and developed immediately to dedifferentiated, relapsing, metastasizing and finally lethal tumors which resisted to each therapy. Histologically only the structural rests of Spiegler basaliomas could be seen, but the cellular picture demonstrated completely dedifferentiated carcinomas. In this observation there was no dominant inheritance, as it is known in Spiegler basaliomas. The late manifestation of the tumors with immediate malignancy, the lacking affection of other members of the family, the high familiar incidence of internal carcinomas, and, finally, the combination with other associated symptoms (brachydactyly, racket nails, the latter ones also in the mother) revealed this disorder as a new syndrome of Spiegler carcinomas with associated symptoms.
该报告涉及一名意大利南部女性患者，她去世时67岁，于1975年在几个月内头皮上出现了斯皮格勒基底细胞瘤。肿瘤的大小和数量不断增加，并迅速发展为去分化、复发性、转移性且最终致命的肿瘤，对各种治疗均有抵抗。组织学上仅可见斯皮格勒基底细胞瘤的结构残余，但细胞图像显示为完全去分化的癌。在该观察病例中，不存在斯皮格勒基底细胞瘤中已知的显性遗传。肿瘤的晚期表现且伴有直接的恶性特征、家族其他成员未受影响、内部癌症的家族发病率高，以及最终与其他相关症状（短指畸形、球拍状指甲，后者其母亲也有）的合并出现，表明这种疾病是一种伴有相关症状的斯皮格勒癌新综合征。
