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nonmalignant tumor of connective tissue

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/03/25 02:50:04」(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]

Presentation[edit]

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[edit]

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[edit]

^ 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.
^ 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
^ ^a ^b Steven P. Meyers (2008). MRI of bone and soft tissue tumors and tumorlike lesions. Thieme.
^ 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. 209 (1): 53–59. PMID 16636523.

External links[edit]

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1. 小児および思春期における良性骨腫瘍：概要 benign bone tumors in children and adolescents an overview
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English Journal

Chromosome 12 long arm rearrangement covering MDM2 and RASAL1 is associated with aggressive craniofacial juvenile ossifying fibroma and extracranial psammomatoid fibro-osseous lesions.

Tabareau-Delalande F1, Collin C2, Gomez-Brouchet A3, Bouvier C4, Decouvelaere AV5, de Muret A6, Pagès JC2, de Pinieux G7.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc.Mod Pathol.2014 Jun 13. doi: 10.1038/modpathol.2014.80. [Epub ahead of print]
To evaluate the diagnostic value of MDM2 status in craniofacial fibro-osseous lesions, we investigated MDM2 expression by immunohistochemistry and analyzed MDM2 amplification by qPCR in 30 cases of ossifying fibroma (including 13 cases of the juvenile variant) and 17 cases of fibrous dysplasia. Two
PMID 24925056

An unusual presentation of peripheral ossifying fibroma in an elderly man.

Parmar YS1, Tarsariya VM1, Jayam C2, Bandlapalli A3.
BMJ case reports.BMJ Case Rep.2014 Jun 12;2014. pii: bcr2014204606. doi: 10.1136/bcr-2014-204606.
Peripheral ossifying fibroma is a common solitary gingival overgrowth thought to arise from the gingival corium, periosteum or periodontal ligament. Ossifying fibroma has the propensity to occur in cranial bones, but the peripheral variety shows a close relationship with the periodontal ligament and
PMID 24925549

A rare benign odontogenic neoplasm: peripheral odontogenic fibroma.

Reddy SV1, Medikonda SK1, Konda A1, Natta S2.
BMJ case reports.BMJ Case Rep.2014 Jun 11;2014. pii: bcr2013201065. doi: 10.1136/bcr-2013-201065.
The peripheral odontogenic fibroma is a relatively rare, benign, unencapsulated and gingival mass of fibrous connective tissue, considered to be the extraosseous counterpart of the central odontogenic fibroma. Peripheral odontogenic fibroma was earlier quite commonly confused with peripheral ossifyi
PMID 24920510

Japanese Journal

上顎に発生した巨大な骨形成性エプーリスの1例

傳田祐也,北島晴比古,清水武 [他]
日本口腔外科学会雑誌 57(10), 553-557, 2011-10
NAID 40019039239

手術シミュレーションに基づく腫瘍分割摘出により顎骨を保存した得た下顎骨骨形成線維腫の1例

金塚文子,豊島貴彦,鈴木麻衣子,河本清司,新谷悟
日本口腔腫瘍学会誌 22(2), 75-80, 2010-06-15
骨形成線維腫はセメント質あるいは骨様の硬組織形成を伴う線維性結合組織の腫瘍性増殖物であり，画像所見においては境界明瞭な透過像の中に大小様々な不透過物が散在する。今回，われわれは40歳代女性の左側下顎骨骨体部に発症した骨形成線維腫に対し，顎骨の温存を目的に腫瘍分割による摘出術を施行し，良好な結果を得たので報告する。症例は左側下顎骨骨形成線維腫に対して，他病院にて下顎骨区域切除と腸骨移植による即時再建 …
NAID 10029089459