Malignant cylindroma in Brooke-Spiegler syndrome

A 68-year-old female patient presented with a history of gradual appearance of multiple nodules situated predominantly on the scalp and neck, with a f ew nodules on the trunk. The nodules began to appear at the age of 30. Fami ly history revealed that the patient's brother, son, father and grandmother had similar cutaneous lesions. The dominant histopathological pattern was that of a cylindroma. Features of both cylindroma and spiradenoma (spiraden ocylindroma) were present within the same lesion in some biopsies. Most les ions on the scalp were skin-colored with a smooth surface. The largest tumo rs located on the neck were tender and ulcerated. Histopathologically, thes e tumors had the morphology of a high-grade malignant solid neoplasm with e pithelioid features. Copyright (C) 2000 S. Karger AG. Basel.