Malignant neoplasms associated with nevus sebaceus of Jadassohn

Nine patients with nevus sebaceus of Jadassohn (NSJ) developed associated morphologically aggressive neoplasms. Four of the tumors were apocrine carcinomas, two of which metastasized to regional lymph nodes, and in one of the two generalized metastases developed. Three tumors were adnexal carcinomas with probable pilar differentiation, but none showed progression of the disease after wide surgical excision. One patient has squamous cell carcinoma and died with generalized metastasis, and one patient had a complex adnexal and squamous cell carcinoma with a large area of anaplastic carcinoma that recurred and invaded the skull.