1. Central xanthoma of the jaw in association with Noonan syndrome
- Author
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Francine B. de Abreu, Vincent A. Memoli, Nicholas J. Olson, and Rocco R. Addante
- Subjects
Male ,0301 basic medicine ,Pathology ,medicine.medical_specialty ,Adolescent ,Biopsy ,030105 genetics & heredity ,Xanthoma ,Pathology and Forensic Medicine ,Diagnosis, Differential ,Lesion ,03 medical and health sciences ,0302 clinical medicine ,Predictive Value of Tests ,Xanthomatosis ,medicine ,Humans ,Mandibular Diseases ,Histiocyte ,business.industry ,Noonan Syndrome ,Mandible ,Soft tissue ,medicine.disease ,Immunohistochemistry ,Giant cell ,030220 oncology & carcinogenesis ,Noonan syndrome ,medicine.symptom ,Tomography, X-Ray Computed ,business - Abstract
Xanthomas are histiocytic lesions of the skin, soft tissue, and bone and are generally considered to be reactive in nature. When they arise in the bones of the jaw, they are referred to as central xanthomas. New evidence supports the hypothesis that central xanthomas are a separate and distinct entity from their extragnathic counterparts. Noonan syndrome (NS) is an autosomal dominant disorder that has been associated with giant cell lesions, which also commonly occur in the jaw. We present a case of a 15-year-old boy with NS who presented with a radiolucent lesion of the mandible that on excision was found to be a central xanthoma. Although giant cell lesions have been well described in NS, xanthomas of the jaw have not been reported. We will also discuss the entities that must be excluded before making a diagnosis of central xanthoma, as this can affect both treatment and follow-up.
- Published
- 2018
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