Abstract
A 40 yr old man had dermatofibrosarcoma protuberans of the neck with rapid local recurrence, satellite nodules and a cervical lymph node metastasis, occurring simultaneously with an atypical fibroxanthoma of the scrotum. These 2 tumors, with distinct clinical and pathologic features, are classified as histogenetically related lesions within the spectrum of fibrous histiocytomas. The place for dermatofibrosarcoma protuberans at the malignant end of this spectrum is clinically and ultrastructurally documented further in this patient.