Crouzon with Acanthosis Nigricans and Odontogenic Tumors

Author:

Xu Wen12,McDonald-McGinn Donna M.34,Melchiorre Alexandra J.3,Zackai Elaine H.34,Bartlett Scott P.12,Taylor Jesse A.12

Affiliation:

1. Division of Plastic Surgery, the Children’s Hospital of Philadelphia, Philadelphia, PA, USA

2. Division of Plastic Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

3. Division of Human Genetics, Clinical Genetics Center, and 22q and You Center, The Children’s, Hospital of Philadelphia, Philadelphia, PA, USA

4. Department of Pediatrics, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA, USA

Abstract

Crouzon syndrome with acanthosis nigricans (CAN) is caused by a mutation in the fibroblast growth factor receptor ( FGFR) 3 gene that presents clinically as Crouzonoid craniofacial features in association with other anomalies such as acanthosis nigricans and benign odontogenic tumors. Diagnosis through the use of genetic mutational analysis is critical, as it alerts the surgeon to the need for careful screening for jaw tumors so that timely treatment in the form of curettage or segmental resection can be provided.

Publisher

SAGE Publications

Subject

Otorhinolaryngology,Oral Surgery

Cited by 6 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. A rare case of cemento-ossifying fibroma in a 13-year-old boy with Crouzon syndrome with acanthosis nigricans;Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology;2023-10

2. An unusual presentation of bilateral optic pathway glioma in Crouzon Syndrome;Pediatric Hematology and Oncology;2023-06-19

3. Acanthosis Nigricans: An Updated Review;Current Pediatric Reviews;2023-02

4. Dental Consequences of FFMBA;Frontofacial Monobloc Advancement with Internal Distraction;2023

5. Crouzon Syndrome and Acanthosis Nigricans With Fibrous Dysplasia of the Maxilla: An Unreported Suggested Triad;Journal of Craniofacial Surgery;2020-11-04

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