Unspecific, idiopathic isolated osteolysis (Gorham-Stout syndrome) of the mandibular condylar process with its radiological, histological and clinical features: a case report and review of literature

Oral Maxillofac Surg. 2014 Mar;18(1):75-9. doi: 10.1007/s10006-013-0394-5. Epub 2013 Feb 3.

Abstract

Background: The unspecific, idiopathic isolated osteolysis is a rare condition in the various known osteopathies and better known as Gorham-Stout syndrome (Gorham's disease, GD). It belongs to the primary idiopathic osteolysis, and its aetiology is poorly understood. Many different localisations have been described, but there are only 48 cases affecting the maxillofacial region until now.

Case report: A 76-year-old patient with the singular localisation of GD in the left condylar process is presented. After radical resection of the condylar process, the clinical, radiological and pathological examinations made the diagnosis of GD. Until now, there is no recurrence for two and a half years.

Discussion: The unspecific and inconsistent clinical and radiological symptoms complicate the diagnosis of GD. Clinical, pathological and radiological correlation is essential to make the diagnosis. The different therapeutical options and the specific outcome are discussed.

Publication types

  • Case Reports
  • Review

MeSH terms

  • ADP-ribosyl Cyclase 1 / analysis
  • Aged
  • Capillaries / pathology
  • Diagnosis, Differential
  • Humans
  • Ki-67 Antigen / analysis
  • Lymphatic Vessels / pathology
  • Male
  • Mandibular Condyle / diagnostic imaging*
  • Mandibular Condyle / pathology*
  • Membrane Glycoproteins / analysis
  • Osteoclasts / pathology
  • Osteolysis, Essential / diagnostic imaging*
  • Osteolysis, Essential / pathology*
  • Platelet Endothelial Cell Adhesion Molecule-1 / analysis*
  • Radiography, Panoramic*
  • Tomography, X-Ray Computed*

Substances

  • Ki-67 Antigen
  • Membrane Glycoproteins
  • Platelet Endothelial Cell Adhesion Molecule-1
  • CD38 protein, human
  • ADP-ribosyl Cyclase 1