PURPOSE: Cherubism is an uncommon fibro-osseous disorder of the jaws that presents with varying degrees of involvement and a tendency toward spontaneous remission. Lesions are characterized by replacement of bone with fibrovascular tissue containing abundant multinucleated giant cells. We attempted to study the relationships among the degree of cherubism, the radiographic extent of the jaw lesions, the histopathologic findings, and the clinical course of 7 patients. PATIENTS AND METHODS: In 7 patients diagnosed with cherubism, we evaluated the degree of fibrosis and perivascular cuffing, the presence of focal hemosiderin deposits, and giant multinucleated cell density (absent, few, moderate, or severe). Clinical course and progression were also assessed using a 4-point scale (improvement, no changes, modest progression, and marked progression). RESULTS: The patients were followed up for an average of 8.5 years. Two patients exhibited clinical and radiographic improvement, while 3 showed no changes, and 2 progressed despite surgical treatment in 1 of them. CONCLUSION: The course of cherubism in 1 of our patients may represent evidence of an association between the presence of abundant multinucleated giant cells, an increased extent of the lesions, and a more aggressive behavior of the disease.
PURPOSE: Cherubism is an uncommon fibro-osseous disorder of the jaws that presents with varying degrees of involvement and a tendency toward spontaneous remission. Lesions are characterized by replacement of bone with fibrovascular tissue containing abundant multinucleated giant cells. We attempted to study the relationships among the degree of cherubism, the radiographic extent of the jaw lesions, the histopathologic findings, and the clinical course of 7 patients. PATIENTS AND METHODS: In 7 patients diagnosed with cherubism, we evaluated the degree of fibrosis and perivascular cuffing, the presence of focal hemosiderin deposits, and giant multinucleated cell density (absent, few, moderate, or severe). Clinical course and progression were also assessed using a 4-point scale (improvement, no changes, modest progression, and marked progression). RESULTS: The patients were followed up for an average of 8.5 years. Two patients exhibited clinical and radiographic improvement, while 3 showed no changes, and 2 progressed despite surgical treatment in 1 of them. CONCLUSION: The course of cherubism in 1 of our patients may represent evidence of an association between the presence of abundant multinucleated giant cells, an increased extent of the lesions, and a more aggressive behavior of the disease.
Authors: Maria E Papadaki; Steven A Lietman; Michael A Levine; Bjorn R Olsen; Leonard B Kaban; Ernst J Reichenberger Journal: Orphanet J Rare Dis Date: 2012-05-24 Impact factor: 4.123
Authors: Michel Ntetani Aloni; Renault Sitwaminya Kambere; Antoine Molua; Joseph Nzinga Dilu; Pierre Manianga Tshibassu; Aimé Kazadi-Lukusa; René Makuala Ngiyulu; Raphael Mbona Kalengayi; Jean Lambert Gini Ehungu Journal: Rare Tumors Date: 2015-02-18