Brooke-Spiegler syndrome: report of a case with combined lesions containing cylindromatous, spiradenomatous, trichoblastomatous, and sebaceous differentiation

Am J Dermatopathol. 2005 Feb;27(1):27-33. doi: 10.1097/01.dad.0000138049.86662.3e.

Abstract

Brooke-Spiegler syndrome is an autosomal dominantly inherited disease with predisposition to cutaneous adnexal neoplasms, most commonly cylindromas and trichoepitheliomas. We report a patient in whom 11 lesions were removed from the scalp and face for various reasons over a period of 3 years. The histopathological survey revealed a plethora of benign adnexal neoplasms showing apocrine, follicular, and sebaceous differentiation occurring independently and conjointly. The histopathological spectrum in our patient included cylindromas, spiradenomas, trichoepitheliomas, small nodular trichoblastomas, and lymphadenomas. Many lesions had hybrid features of two or more neoplasms. By far the most common composite tumor was spiradenocylindroma. Some spiradenocylindromas demonstrated prominent sebaceous or trichoblastomatous differentiation or both. We suggest the terms "sebaceous spiradenocylindroma" and "trichospiradenocylindroma" for these lesions. The occurrence of sebaceous and trichoblastic differentiation in spiradenocylindromas is a further proof that spiradenoma and cylindroma are not eccrine tumors but neoplasms of the folliculosebaceousapocrine unit.

Publication types

  • Case Reports

MeSH terms

  • Adenoma, Sweat Gland / metabolism
  • Adenoma, Sweat Gland / pathology
  • Adenoma, Sweat Gland / surgery
  • Aged
  • Biomarkers, Tumor / metabolism
  • Carcinoma, Adenoid Cystic / metabolism
  • Carcinoma, Adenoid Cystic / pathology
  • Carcinoma, Adenoid Cystic / surgery
  • Carcinoma, Skin Appendage / metabolism
  • Carcinoma, Skin Appendage / pathology*
  • Carcinoma, Skin Appendage / surgery
  • Hair Diseases / metabolism
  • Hair Diseases / pathology
  • Hair Diseases / surgery
  • Hair Follicle / metabolism
  • Hair Follicle / pathology
  • Humans
  • Immunoenzyme Techniques
  • Male
  • Neoplasms, Multiple Primary / pathology*
  • Neoplastic Syndromes, Hereditary / pathology*
  • Sebaceous Glands / metabolism
  • Sebaceous Glands / pathology
  • Skin Neoplasms / metabolism
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / surgery
  • Syndrome

Substances

  • Biomarkers, Tumor