WordNet
- abnormal development (of organs or cells) or an abnormal structure resulting from such growth
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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/01/22 10:13:03」(JST)
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Osteofibrous dysplasia (also known as ossifying fibroma) is a rare, benign non-neoplastic condition with no known cause. It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula,[1] and coined the term. In 1942, however, Jaffe and Lichtenstein described the same condition under the name "nonosteogenic fibroma",[2] so the condition is often known as Jaffe-Campanacci Syndrome. The syndrome's exact nature is under debate, with some authors suggesting that it may be a manifestation of neurofibromatosis.[3]
Contents
- 1 Presentation
- 2 Treatment
- 3 References
- 4 External links
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Presentation
The tibia is the most commonly involved bone, accounting for 85% of cases.[4] It is usually painless, although there may be localized pain or fracture, and presents as a localized firm swelling of the tibia in children less than two decades old (median age for males 10, females 13[5]). Several authors have related this non-neoplastic lesion to adamantinoma - a tumor involving subcutaneous long bones - stating the common cause to be fibrovascular defect.[6] However, the latter is distinguished from an osteofibrous dysplasia by the presence of soft tissue extension, intramedullary extension, periosteal reaction and presence of hyperchromic epithelial cells under the microscope.
Osteofibrous dysplasia may also be mistaken for fibrous dysplasia of bone, although osteofibrous dysplasia is more likely to show an immunohistochemical reaction to osteonectin, neurofibromin[disambiguation needed], and S-100 protein.[5]
Treatment
Osteofibrous dysplasia is treated with marginal resection with or without bone grafting, depending on the size of the lesion and the extent of bony involvement. However, due to the high rate of recurrence in skeletally immature individuals, this procedure is usually postponed till skeletal maturity.
References
- ^ Campanacci M (August 1976). "Osteofibrous dysplasia of long bones a new clinical entity". Ital J Orthop Traumatol 2 (2): 221–37. PMID 1024109.
- ^ Jaffe HL, Lichtenstein L (March 1942). "Non-osteogenic fibroma of bone". Am. J. Pathol. 18 (2): 205–221. PMC 2032933. PMID 19970624. //www.ncbi.nlm.nih.gov/pmc/articles/PMC2032933/.
- ^ Colby RS, Saul RA (November 2003). "Is Jaffe-Campanacci syndrome just a manifestation of neurofibromatosis type 1?". Am. J. Med. Genet. A 123A (1): 60–3. doi:10.1002/ajmg.a.20490. PMID 14556247.
- ^ Robert Mervyn Letts, Osteofibrous Dysplasia, http://emedicine.medscape.com/article/1256595-overview
- ^ a b Steven P. Meyers (2008). MRI of bone and soft tissue tumors and tumorlike lesions. Thieme. http://books.google.com/books?id=V-7y1nNatmoC&pg=PA654.
- ^ Hatori M, Watanabe M, Hosaka M, Sasano H, Narita M, Kokubun S. (2006), "A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature", Tohoku J Exp Med. (1): 53-59, PMID 16636523, http://www.ncbi.nlm.nih.gov/pubmed/16636523
External links
- E-medicine overview
- humpath
UpToDate Contents
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English Journal
- Neonatal osteofibrous dysplasia associated with pathological tibia fracture: a case report and review of the literature.
- Çetinkaya M, Özkan H, Köksal N, Sarısözen B, Yazıcı Z.SourceDepartment of Neonatology, Uludag University, Faculty of Medicine, Bursa, Turkey. drmerih@yahoo.com
- Journal of pediatric orthopaedics. Part B.J Pediatr Orthop B.2012 Mar;21(2):183-6.
- Osteofibrous dysplasia is a rare and benign disease that originates from the tibia or fibula. The symptoms of osteofibrous dysplasia include painless enlargement and bowing of the tibia and pain occurring in the presence of pathological fracture. Herein a male infant who was admitted with redness an
- PMID 21383639
- Analysis of stromal cells in osteofibrous dysplasia and adamantinoma of long bones.
- Taylor RM, Kashima TG, Ferguson DJ, Szuhai K, Hogendoorn PC, Athanasou NA.SourceNuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Nuffield Orthopaedic Centre, Oxford, UK.
- Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc.Mod Pathol.2012 Jan;25(1):56-64. doi: 10.1038/modpathol.2011.141. Epub 2011 Oct 7.
- Adamantinoma of long bones and osteofibrous dysplasia are rare, osteolytic primary bone tumours of uncertain origin containing areas of fibrous and fibro-osseous proliferation. We investigated the nature of the stromal cells in adamantinoma of long bones and osteofibrous dysplasia, and determined ce
- PMID 21983933
Japanese Journal
- 臨床室 8年の自然経過にて病巣の消失を認めた骨線維性異形成の1例
- 骨線維性異形成(OFD)に対し巨大骨欠損をβ-TCPのみにて補填した3例
- 比嘉 聖,帖佐 悦男,坂本 武郎,渡邊 信二,関本 朝久,濱田 浩朗,野崎 正太郎,前田 和徳,中村 嘉宏,舩元 太郎
- 整形外科と災害外科 56(3), 458-461, 2007-09-25
- NAID 10019942311
Related Links
- Osteofibrous dysplasia is a rare, nonneoplastic condition of unknown etiology that affects the long bones. It frequently is asymptomatic. [1] Most lesions of osteofibrous dysplasia affect the cortex of the tibia, predominantly the middle third of the diaphysis, as seen in the image below. The cortex often is ...
- Osteofibrous dysplasia. 16 likes · 0 talking about this. Osteofibrous dysplasia (also known as ossifying fibroma) is a rare, benign non-neoplastic condition with no known cause. It is considered a fibrovascular defect. Campanacci ...
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