Craniofacial Fibrous Dysplasia Associated With McCune-Albright Syndrome

An 18-year-old boy presented to our hospital in August 2006 for management of an extremely large lesion that was causing considerable distortion of his face (Fig 1). The patient was an orphan raised by his grandmother who had suffered from dementia for years. Therefore, part of the patient's cl...

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Veröffentlicht in:Journal of oral and maxillofacial surgery 2009-03, Vol.67 (3), p.637-644
Hauptverfasser: Chen, Yu, MD, Ding, Xueqiang, MD, Yang, Yiqiang, MD, Yan, Wangxiang, MD, Chen, Dan, MD, Li, Zhibin, MD
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container_issue 3
container_start_page 637
container_title Journal of oral and maxillofacial surgery
container_volume 67
creator Chen, Yu, MD
Ding, Xueqiang, MD
Yang, Yiqiang, MD
Yan, Wangxiang, MD
Chen, Dan, MD
Li, Zhibin, MD
description An 18-year-old boy presented to our hospital in August 2006 for management of an extremely large lesion that was causing considerable distortion of his face (Fig 1). The patient was an orphan raised by his grandmother who had suffered from dementia for years. Therefore, part of the patient's clinical history was described by his neighbors, and the information was not as accurate as desired. The patient was said to have had a rigid tumor-like lesion protruding from the palatal vault into his oral cavity about 10 years previously. The lesion was estimated to be as large as 3 cm in diameter in the beginning. According to the neighbors' accounts, the lesion may have originated from the left maxilla. The lesion was painless, and increased very slowly in the early years, but it had been swelling rapidly since 2002 (Fig 2), especially in the previous 3 months, and the maximum diameter of the lesion had reached 20 cm when he visited our hospital. The giant lesion had caused the patient great difficulties in speaking, eating, and even breathing. The patient also stated that the lesion had recently become painful, and the surface of the lesion often ulcerated. For financial reasons, he had not received any therapy before he visited our hospital.
doi_str_mv 10.1016/j.joms.2008.01.068
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The patient was an orphan raised by his grandmother who had suffered from dementia for years. Therefore, part of the patient's clinical history was described by his neighbors, and the information was not as accurate as desired. The patient was said to have had a rigid tumor-like lesion protruding from the palatal vault into his oral cavity about 10 years previously. The lesion was estimated to be as large as 3 cm in diameter in the beginning. According to the neighbors' accounts, the lesion may have originated from the left maxilla. The lesion was painless, and increased very slowly in the early years, but it had been swelling rapidly since 2002 (Fig 2), especially in the previous 3 months, and the maximum diameter of the lesion had reached 20 cm when he visited our hospital. The giant lesion had caused the patient great difficulties in speaking, eating, and even breathing. The patient also stated that the lesion had recently become painful, and the surface of the lesion often ulcerated. For financial reasons, he had not received any therapy before he visited our hospital.</description><identifier>ISSN: 0278-2391</identifier><identifier>EISSN: 1531-5053</identifier><identifier>DOI: 10.1016/j.joms.2008.01.068</identifier><identifier>PMID: 19231793</identifier><identifier>CODEN: JOMSDA</identifier><language>eng</language><publisher>New York, NY: Elsevier Inc</publisher><subject>Adolescent ; Biological and medical sciences ; Dentistry ; Diagnosis, Differential ; Diseases of the osteoarticular system ; Endocrinopathies ; Facial Bones - pathology ; Fibrous Dysplasia, Polyostotic - pathology ; Fibrous Dysplasia, Polyostotic - surgery ; Humans ; Male ; Malformations and congenital and or hereditary diseases involving bones. Joint deformations ; Medical sciences ; Non tumoral diseases. Target tissue resistance. Benign neoplasms ; Otorhinolaryngology. 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The patient was an orphan raised by his grandmother who had suffered from dementia for years. Therefore, part of the patient's clinical history was described by his neighbors, and the information was not as accurate as desired. The patient was said to have had a rigid tumor-like lesion protruding from the palatal vault into his oral cavity about 10 years previously. The lesion was estimated to be as large as 3 cm in diameter in the beginning. According to the neighbors' accounts, the lesion may have originated from the left maxilla. The lesion was painless, and increased very slowly in the early years, but it had been swelling rapidly since 2002 (Fig 2), especially in the previous 3 months, and the maximum diameter of the lesion had reached 20 cm when he visited our hospital. The giant lesion had caused the patient great difficulties in speaking, eating, and even breathing. The patient also stated that the lesion had recently become painful, and the surface of the lesion often ulcerated. For financial reasons, he had not received any therapy before he visited our hospital.</description><subject>Adolescent</subject><subject>Biological and medical sciences</subject><subject>Dentistry</subject><subject>Diagnosis, Differential</subject><subject>Diseases of the osteoarticular system</subject><subject>Endocrinopathies</subject><subject>Facial Bones - pathology</subject><subject>Fibrous Dysplasia, Polyostotic - pathology</subject><subject>Fibrous Dysplasia, Polyostotic - surgery</subject><subject>Humans</subject><subject>Male</subject><subject>Malformations and congenital and or hereditary diseases involving bones. Joint deformations</subject><subject>Medical sciences</subject><subject>Non tumoral diseases. Target tissue resistance. Benign neoplasms</subject><subject>Otorhinolaryngology. Stomatology</subject><subject>Parathyroids. Parafollicular cells. 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Joint deformations</topic><topic>Medical sciences</topic><topic>Non tumoral diseases. Target tissue resistance. Benign neoplasms</topic><topic>Otorhinolaryngology. Stomatology</topic><topic>Parathyroids. Parafollicular cells. Cholecalciferol. 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subjects Adolescent
Biological and medical sciences
Dentistry
Diagnosis, Differential
Diseases of the osteoarticular system
Endocrinopathies
Facial Bones - pathology
Fibrous Dysplasia, Polyostotic - pathology
Fibrous Dysplasia, Polyostotic - surgery
Humans
Male
Malformations and congenital and or hereditary diseases involving bones. Joint deformations
Medical sciences
Non tumoral diseases. Target tissue resistance. Benign neoplasms
Otorhinolaryngology. Stomatology
Parathyroids. Parafollicular cells. Cholecalciferol. Phosphocalcic homeostasis (diseases)
Skull - pathology
Surgery
title Craniofacial Fibrous Dysplasia Associated With McCune-Albright Syndrome
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