Multiple malignant Spiegler tumors were observed on the scalp of a 67-year-old female patient with brachydactyly and racket-nails affecting all fingers of both hands. The occurrence of similar clinical features in the family of the patient is suggestive of a hereditary disorder. The light and electron microscopical findings are described.

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.

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