頭蓋顔面多骨性線維性異形成症のCT所見:特に嚢胞様骨吸収像について

「緒言」線維性異形成症(FD)は遺伝子GNAS1(guanine nucle-otide-binding protein, α-stimulating activity polypeptide 1)の変異により, 骨の形成異常がひきおこされ線維組織や未成熟骨に置換される疾患である1,2. FDの発生頻度は骨腫瘍の2.5%, 良性骨腫瘍の7%であり3,4, 小児や若年者, 女性に多くみられる1,5. FDは単骨性および多骨性にわかれ1, 多骨性FDが内分泌異常をともなう場合はMcCune-Albright症候群に分類される1,6,7. 好発部位は長管骨, 顎骨, 頭蓋骨, 肋骨であり, 顎骨では...

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Published inShika Hoshasen Vol. 50; no. 1; pp. 1 - 7
Main Authors 木瀬, 祥貴, 内藤, 宗孝, 有地, 淑子, 泉, 雅浩, 有地, 榮一郎, 後藤, 真一
Format Journal Article
LanguageJapanese
Published 特定非営利活動法人 日本歯科放射線学会 2010
日本歯科放射線学会
Japanese Society for Oral and Maxillofacial Radiology
Subjects
Online AccessGet full text
ISSN0389-9705
2185-6311
DOI10.11242/dentalradiology.50.1

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Abstract 「緒言」線維性異形成症(FD)は遺伝子GNAS1(guanine nucle-otide-binding protein, α-stimulating activity polypeptide 1)の変異により, 骨の形成異常がひきおこされ線維組織や未成熟骨に置換される疾患である1,2. FDの発生頻度は骨腫瘍の2.5%, 良性骨腫瘍の7%であり3,4, 小児や若年者, 女性に多くみられる1,5. FDは単骨性および多骨性にわかれ1, 多骨性FDが内分泌異常をともなう場合はMcCune-Albright症候群に分類される1,6,7. 好発部位は長管骨, 顎骨, 頭蓋骨, 肋骨であり, 顎骨では下顎骨よりも上顎骨に多く, 隣接する頬骨や蝶形骨にも発生する1. 頭蓋顔面骨のFDは多骨性に発現することが多いとされる8. FDの診断は病理組織のみでは難しく, 高品質の画像診断の役割は大きい9,10, また骨の生検は病的骨折の危険性を増すので一般的にはさけるべきとされている11.
AbstractList 「緒言」線維性異形成症(FD)は遺伝子GNAS1(guanine nucle-otide-binding protein, α-stimulating activity polypeptide 1)の変異により, 骨の形成異常がひきおこされ線維組織や未成熟骨に置換される疾患である1,2. FDの発生頻度は骨腫瘍の2.5%, 良性骨腫瘍の7%であり3,4, 小児や若年者, 女性に多くみられる1,5. FDは単骨性および多骨性にわかれ1, 多骨性FDが内分泌異常をともなう場合はMcCune-Albright症候群に分類される1,6,7. 好発部位は長管骨, 顎骨, 頭蓋骨, 肋骨であり, 顎骨では下顎骨よりも上顎骨に多く, 隣接する頬骨や蝶形骨にも発生する1. 頭蓋顔面骨のFDは多骨性に発現することが多いとされる8. FDの診断は病理組織のみでは難しく, 高品質の画像診断の役割は大きい9,10, また骨の生検は病的骨折の危険性を増すので一般的にはさけるべきとされている11.
Objective: To clarify CT image features of the craniofacial bones in patients with polyostotic fibrous dysplasia (FD).Methods: Five patients in our hospital and 32 patients in 27 previously reported studies were selected for this study. CT image features were evaluated by 3 maxillofacial radiologists and classified into 3 patterns (sclerotic, mixed, and cyst-like lytic patterns).Results: The bones affected by FD were the sphenoid bone, maxilla, zygoma, temporal bone, mandible, and so on. There was a significant difference in the distribution of CT image patterns by the type of bone (p = 0.0012). A lytic pattern was frequently found in the mandible, sphenoid bone, frontal bone, and maxilla. The clinical data significantly affected by the distribution of CT image patterns were the periods of symptoms and the anamnesis about the previous operation: when the symptoms were persistent, the ratio of the lytic pattern was high. There was a significant difference in pattern distribution in the frontal bone and maxilla in the surgical anamnesis. No significant difference was seen by age and sex. The cyst-like lytic area was shown to be a mucocele or aneurysmal bone cyst in the frontal and sphenoid bones, whereas an inflammatory change in the jaw bones,Conclusion: There was a significant difference in the distribution of CT image patterns by the types of craniofacial bones affected by polyostotic FD.
Author 内藤, 宗孝
後藤, 真一
泉, 雅浩
有地, 淑子
木瀬, 祥貴
有地, 榮一郎
Author_FL Naitoh Munetaka
Ariji Eiichiro
Izumi Masahiro
Gotoh Masakazu
Kise Yoshitaka
Ariji Yoshiko
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  fullname: Kise Yoshitaka
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  fullname: Ariji Yoshiko
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  fullname: Gotoh Masakazu
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Shika Hoshasen
Dent. Radiol
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日本歯科放射線学会
Japanese Society for Oral and Maxillofacial Radiology
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References_xml – reference: 14. Diah E, Morris DE, Lo LJ, Chen YR. Cyst degeneration in craniofacial fibrous dysplasia: clinical presentation and managemen J Neurosurg. 2007; 107: 504-508.
– reference: 28. Szwajkun P, Chen YR, Yeow VK, Breidahl AF. The “Taiwanese giant”: hormonal and genetic influences in fibrous dysplasia. Ann Plast Surg. 1998; 41: 75-80.
– reference: 34. Mahapatra AK, Gupta PK, Ravi RR. Craniofacial surgery and optic canal decompression in adult fibrous dysplasia. Neurol India. 2003; 51: 123-124.
– reference: 31. Clauser L, Marchetti C, Piccione M, Bertoni F. Craniofacial fibrous dysplasia and Ollier's disease: combined transfrontal and transfacial resection using the nasal-cheek flap. J Craniofac Surg. 1996; 7: 140-144.
– reference: 36. Breidahl AF, Klug GL, Holmes AD. Allogeneic membranous bone grafting for a large calvarial defect. Plast Reconstr Surg. 2005; 115: 43e-49e.
– reference: 32. Djeric D, Stefanovic P. Fibrous dysplasia of the temporal bone and maxillofacial region associated with cholesteatoma of the middle ear. Auris Nasus Larynx. 1999; 26: 79-81.
– reference: 19. Akintoye SO, Otis LL, Atkinson JC, Brahim J, Kushner H, Robey PG, Collins MT. Analyses of variable panoramic radiographic characteristics of maxillo-mandibular fibrous dysplasia in McCune-Albright syndrome. Oral Dis. 2004; 10: 36-43.
– reference: 4. Mortensen A, Bojsen-Møller M, Rasmussen P. Fibrous dysplasia of the skull with acromegaly and sarcomatous transformation. Two cases with a review of the literature. J Neurooncol. 1989; 7: 25-29.
– reference: 22. Hirabayashi S, Kagawa K, Ohkubo E, Sugawara Y, Sakurai A. Craniofacial fibrous dysplasia with rapidly increasing proptosis due to a mucocele behind the globe. Ann Plast Surg. 1998; 40: 182-185.
– reference: 42. Papay FA, Morales L Jr, Flaharty P, Smith SJ, Anderson R, WAlker JM, Hood RS, Hardy S. Optic nerve decompression in cranial base fibrous dysplasia. J Craniofac Surg. 1995; 6: 5-14.
– reference: 3. Ricalde P, Horswell BB. Craniofacial fibrous dysplasia of the fronto-orbital region: a case series and literature review. J Oral Maxillofac Surg. 2001; 59: 157-168.
– reference: 11. Chapurlat RD, Meunier PJ. Fibrous dysplasia of bone. Baillieres Best Pract Res Clin Rheumatol. 2000; 14: 385-398.
– reference: 18. Riminucci M, Liu B, Corsi A, Shenker A, Spiegel AM, Robey PG, Bianco P. The histopathology of fibrous dysplasia of bone in patients with activating mutations of the Gs alpha gene: site-specific patterns and recurrent histological hallmarks. J Pathol. 1999; 187: 249-258.
– reference: 29. Kim HJ, Kim KD, Lee MH. Becker's melanosis associated with fibrous dysplasia. Int J Dermatol. 2002; 41: 384-386.
– reference: 39. Toba M, Hayashida K, Imakita S, Fukuchi K, Kume N, Shimotsu Y, Cho I, Ishida Y, Takamiya M, Kumita S. Increased bone mineral turnover without increased glucose utilization in sclerotic and hyperplastic change in fibrous dysplasia. Ann Nucl Med. 1998; 12: 153-155.
– reference: 45. Valentini V, Cassoni A, Marianetti TM, Terenzi V, Fadda MT, Iannetti G. Craniomaxillofacial fibrous dysplasia: conservative treatment or radical surgery? A retrospective study on 68 patients. Plast Reconstr Surg. 2009; 123: 653-660.
– reference: 17. Yüceer N, Kutluhan A, Bekerecioglu M, Arslan H, Akman E. Polyostotic fibrous dysplasia with craniofacial localization presenting with frontal lobe compression in a 14-year-old girl. Acta Neurochir (Wien). 1999; 141: 203-207.
– reference: 1. Jundt G. Fibrous dysplasia. In: Barnes L, Eveson JW, Reichart P, Sidransky D. Pathology and genetics of head and neck tumors. Lyon: IARC Press; 2005: p321-322.
– reference: 5. Schonder A. Fibrous dysplasia of bone with proptosis. Am J Dis Child. 1977; 131: 678-679.
– reference: 20. Simpson AH, Creasy TS, Williamson DM, Wilson DJ, Spivey JS. Cystic degeneration of fibrous dysplasia masquerading as sarcoma. J Bone Joint Surg Br. 1989; 71: 434-436.
– reference: 23. Nguyen BD, Lugo-Olivieri CH, McCarthy EF, Frassica FJ, Ma LD, Zerhouni EA. Fibrous dysplasia with secondary aneurysmal bone cyst. Skeletal Radiol. 1996; 25: 88-91.
– reference: 9. Waldron CA. Fibro-osseous lesions of the jaws. J Oral Maxillofac Surg. 1985; 43: 249-262.
– reference: 48. Ferretti C, Coleman H, Dent M, Altini M. Cystic degeneration in fibrous dysplasia of the jaws: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1999; 88: 337-342.
– reference: 44. Chen YR, Fairholm D. Fronto-orbito-sphenoidal fibrous dysplasia. Ann Plast Surg. 1985; 15: 190-203.
– reference: 43. Posnick JC, Wells MD, Drake JM, Buncic JR, Armstrong D. Childhood fibrous dysplasia presenting as blindness: a skull base approach for resection and immediate reconstruction. Pediatr Neurosurg. 1993; 19: 260-266.
– reference: 6. Viljoen DL, Versfeld GA, Losken W, Beighton P. Polyostotic fibrous dysplasia with cranial hyperostosis: new entity or most severe form of polyostotic fibrous dysplasia? Am J Med Genet. 1988; 29: 661-667.
– reference: 33. Mizuno A, Kuroyanagi-Nakajima M, Akiyama Y, Katayama T, Suzuki H, Nakano Y. Facial fibrous dysplasia: report of a case. Oral Surg Oral Med Oral Pathol. 1991; 72: 284-288.
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Snippet 「緒言」線維性異形成症(FD)は遺伝子GNAS1(guanine nucle-otide-binding protein, α-stimulating activity polypeptide 1)の変異により, 骨の形成異常がひきおこされ線...
Objective: To clarify CT image features of the craniofacial bones in patients with polyostotic fibrous dysplasia (FD).Methods: Five patients in our hospital...
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SubjectTerms Craniofacial bone
Fibrous dysplasia
Jaw
Polyostotic
多骨性
線維性異形成症
頭蓋顔面骨
顎骨
Title 頭蓋顔面多骨性線維性異形成症のCT所見:特に嚢胞様骨吸収像について
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Volume 50
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ispartofPNX 歯科放射線, 2010, Vol.50(1), pp.1-7
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