Massive osteolysis with histologic features of eosinophilic angiocentric fibrosis

Background Massive osteolysis (MO) is a rare destructive process of unknown etiology, first described by Gorham and Stout in 1954. This condition is exceedingly rare with approximately 150 cases described in the literature and 20% involving the jaws and skull. The disease is characterized by a proliferation of thin-walled vasculature channels and inflammation with regional osteolysis. Eosinophilic angiocentric fibrosis (EAF) is a newly described entity also of unknown etiology. Previously reported cases of EAF have been restricted to the nose and upper airway tract. The histology of EAF is characterized by a perivascular fibrosis and associated tissue eosinophilia. A 16-year-old girl with MO affecting the right mandible and bilateral maxilla and histologic features of EAF is described. Objective We attempt to determine whether similarities exist between MO and EAF and if these 2 entities can be distinguished based on the clinical, radiographic, or histopathologic features. Study design A comprehensive search of the Medline database from 1966 through 2003 and a review of the reference lists of relevant articles of MO and EAF formed the basis of this study. These data were compared to the present case of MO with histologic features of EAF. Results The case report has clinical and radiographic features of MO. The process was progressive, with uncontrolled osteolysis despite aggressive surgery and radiation therapy. The histologic features identified are comparable to those described for EAF. However our literature review could not identify clinical and radiographic similarities between this case and those of EAF reported in the literature. Conclusion The clinical and radiographic features of this case are consistent with a diagnosis of MO, yet the histopathology is more typical of EAF. The relationship between these 2 poorly understood conditions, if any, remains obscure.