Clinicoradiologic follow up of cherubism with aggressive characteristics: a series of 3 cases
Autor: | Shyam Kishor Sah, Shyama Jain, Ernst J Reichenberger, Khushboo Singh, Anju Garg, Seema Kapoor, Sunita Gupta, Nirupama Trehanpati, P.S. Bhandari |
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Rok vydání: | 2019 |
Předmět: |
medicine.medical_specialty
Mandible Fibrous tissue Condyle World health Pathology and Forensic Medicine Lesion 03 medical and health sciences 0302 clinical medicine medicine Humans Radiology Nuclear Medicine and imaging Dentistry (miscellaneous) Child business.industry Cherubism 030206 dentistry medicine.disease Maxillary enlargement medicine.anatomical_structure Jaw Giant cell 030220 oncology & carcinogenesis Surgery Radiology Oral Surgery medicine.symptom business Follow-Up Studies Orbit (anatomy) |
Zdroj: | Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology. 128:e191-e201 |
ISSN: | 2212-4403 |
Popis: | Cherubism is a rare autosomal dominant disorder characterized by replacement of bone with fibrous tissue containing multinucleated giant cells. It manifests as bilateral mandibular and/or maxillary enlargement. The 2017 World Health Organization classification lists cherubism as a giant cell lesion of the jaws, distinct from fibro-osseous disorders. We discuss 3 cases of familial cherubism having aggressive characteristics and present clinicoradiologic evaluations of the lesions over 12, 18, and 1.5 years, respectively. Follow-up was observational, without active intervention. Analysis of the lesions for change in size and functional impairments was correlated with periodic imaging. All patients are currently being monitored. The outcome in 2 cases has been excellent without intervention, but 1 case had extensive involvement of the jaws and involvement of the condyle and orbit. A secondary giant cell lesion involved the palate in one patient's mother, who had had cherubic lesions in childhood. |
Databáze: | OpenAIRE |
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