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(of meat) full of sinews; especially impossible to chew (同)sinewy, stringy, unchewable
abnormal development (of organs or cells) or an abnormal structure resulting from such growth

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繊維の,繊維状(質)の

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/08/11 18:05:18」(JST)

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Fibrous dysplasia is an abnormal bone growth where normal bone is replaced with fibrous bone tissue. Fibrous dysplasia causes abnormal growth or swelling of bone. Fibrous dysplasia can occur in any part of the skeleton but the bones of the skull, thigh, shin, ribs, upper arm and pelvis are most commonly affected. Fibrous dysplasia is very rare, and there is no known cure. Fibrous dysplasia is not a form of cancer.

Most lesions are monostotic, asymptomatic and identified incidentally and can be treated with clinical observation and patient education.

This disorder is usually diagnosed in childhood or early adulthood and can affect one or several bones. Males and females of any race are equally affected.

Pathophysiology[edit source | edit]

In fibrous dysplasia, the medullary cavity of bones is filled with fibrous tissue, causing the expansion of the areas of bone involved. The bony trabeculae are abnormally thin and irregular, and often likened to Chinese characters.

The cause of this transformation, in turn, is not completely known, however. Levels of the transcription factor C-fos are raised in fibrous dysplasia, leading to gene over-expression and tumour formation.^[1]^[2] It is not hereditary.^[3] There are two types of fibrous dysplasia:

Monostotic (Involving a single bone, or adjacent bones, like both the upper and lower jaw)
Polyostotic (Involving many bones).

The most severe form of polyostotic fibrous dysplasia is known as McCune-Albright syndrome. Fibrous dysplasia of bone may also be an associated abnormality in Neurofibromatosis type II.

Presentation[edit source | edit]

Fibrous dysplasia of the right zygomatic bone (left in the image).Corresponding T2-weighted MRI (left) and CT (right) of the same patient.

Especially when involving the skull or facial bones, the lesions can cause externally visible deformities. The skull is often, but not necessarily, affected, and any other bone(s) can be involved.

Many patients have lesions localized in only one bone (monostotic fibrous dysplasia), affecting 70–80%.
Others have them in many bones (polyostotic fibrous dysplasia).

In 3% of cases, people suffering from fibrous dysplasia also have endocrine diseases and skin pigmentation; the three together constitute McCune-Albright syndrome. These endocrine diseases include precocious (early) puberty, often occurring as early as 6 years old.

In most cases, fibrous dysplasia has no symptoms and is only diagnosed by accident during investigations for an unrelated medical problem. When symptoms occur, they can include:

Pain - caused by the expansion of bone. Treatment for this is pain management therapy, analgesics and anti-inflammatory medication.
Irregular bone growth
Bone deformity
Increased susceptibility to bone fractures (rare)

Micrograph of fibrous dysplasia (right of image) jutxaposed with unaffected bone (left of image). H&E stain.

Treatment[edit source | edit]

Treatment options may include:

Medication to strengthen bones (amino-bisphosphonates)
Medication to treat onset of pain
Pain management therapy
Surgery

References[edit source | edit]

^ Candeliere GA, Glorieux FH, Prud'homme J, St-Arnaud R (June 1995). "Increased expression of the c-fos proto-oncogene in bone from patients with fibrous dysplasia". N. Engl. J. Med. 332 (23): 1546–51. doi:10.1056/NEJM199506083322304. PMID 7739708.
^ Kashima TG, Nishiyama T, Shimazu K, et al. (February 2009). "Periostin, a novel marker of intramembranous ossification, is expressed in fibrous dysplasia and in c-Fos-overexpressing bone lesions". Hum. Pathol. 40 (2): 226–37. doi:10.1016/j.humpath.2008.07.008. PMID 18799196.
^ Fibrous Dysplasia at eMedicine

External links[edit source | edit]

MRI showing fibrous dysplasia of the femoral neck
Duke Orthopedics fibrous_dysplasia
Fibrous Dysplasia Pictures MedPix Imaging Database
Fibrous Dysplasia of the Skull Images radRounds Radiology Image Database
Fibrous dysplasia from Radiopaedia.
Fibrous Dysplasia Foundation

UpToDate Contents

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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
2. 線維筋異形成症の臨床症状および診断 clinical manifestations and diagnosis of fibromuscular dysplasia
3. 胸壁疾患 diseases of the chest wall
4. デスモイド腫瘍：疫学、危険因子、分子学的病因、臨床症状、診断、および局所療法 desmoid tumors epidemiology risk factors molecular pathogenesis clinical presentation diagnosis and local therapy
5. 骨形成不全症：臨床的特徴および診断 osteogenesis imperfecta clinical features and diagnosis

English Journal

The role of dual-phase Tc-99m MIBI in the evaluation of potentially operable lesions detected by bone scanning in fibrous dysplasia: a long-term prospective follow-up study.

Ones T, Erol B, Aras M, Dede F, Erdil TY, Inanir S, Turoglu HT.SourceaDepartments of Nuclear Medicine bOrthopaedics and Traumatology, Marmara University School of Medicine, Istanbul, Turkey.
Nuclear medicine communications.Nucl Med Commun.2012 Mar;33(3):288-96.
PURPOSE: The aim of this study was to determine the role of whole-body Tc-99m MDP bone scintigraphy (BSc) with dual-phase Tc-99m MIBI scintigraphy (DPMSc) in the assessment of fibrous dysplasia (FD) and the value of DPMSc in the detection of potentially operable lesions for guiding surgical treatmen
PMID 22198723

Aspects of liver pathology in adult patients with MDR3/ABCB4 gene mutations.

Wendum D, Barbu V, Rosmorduc O, Arrivé L, Fléjou JF, Poupon R.SourceService d'Anatomie Pathologique, APHP, Hôpital St Antoine, 75012, Paris, France, dominique.wendum@sat.aphp.fr.
Virchows Archiv : an international journal of pathology.Virchows Arch.2012 Feb 14. [Epub ahead of print]
The aims of this study were to describe the histological liver lesions in adult patients with MDR3/ ABCB4 mutation and to study the usefulness of MDR3 immunostaining as a diagnostic tool. All adult patients from our institution with an MDR3/ABCB4 mutation and a liver histology were included (n =�
PMID 22331132

Bisphosphonates: effects on osteoblast.

Maruotti N, Corrado A, Neve A, Cantatore FP.SourceDepartment of Rheumatology, University of Foggia Medical School, Foggia, Italy.
European journal of clinical pharmacology.Eur J Clin Pharmacol.2012 Feb 9. [Epub ahead of print]
PURPOSE: Bisphosphonates are synthetic analogues of pyrophosphate usually used in treating bone disorders such as osteoporosis, Paget's disease, fibrous dysplasia, hypercalcemia of malignancy, and inflammation-related bone loss. Though therapeutic effects of bisphosphonates depend primarily on their
PMID 22318756

Japanese Journal

私の手術と合併症回避のコツ(第56回)頭蓋顔面の線維性骨異形成症(fibrous dysplasia)の外科治療

久徳茂雄
形成外科 54(8), 891-901, 2011-08
NAID 40018940391

Polyostotic fibrous dysplasia with gigantism and huge pelvic tumor : a rare case of McCune-Albright syndrome

SAKAYAMA Kenshi,SUGAWARA Yoshifumi,KIDANI Teruki,FUJIBUCHI Taketsugu,KITO Katsumi,TANJI Nozomu,NAKAMURA Atsushi
International journal of clinical oncology 16(3), 270-274, 2011-06-01
NAID 10029527154

Related Pictures

★リンクテーブル★

リンク元	「線維性骨異形成」「繊維性骨異形成症」
拡張検索	「fibrous dysplasia of bone」
関連記事	「fibrous」

「線維性骨異形成」

Fibrous dysplasia
	Classification and external resources
	; Micrograph showing fibrous dysplasia with the characteristic thin, irregular (Chinese character-like) bony trabeculae and fibrotic marrow space. H&E stain.
ICD-10	K10.8, M85.0, Q78.1
ICD-9	526.89, 733.29, 756.54
MedlinePlus	001234
eMedicine	radio/284
MeSH	D005357

　　[★]

英: fibrous dysplasia、fibrous dysplasia of bone
同: 線維性骨形成異常 fibrous bone dysplasia、繊維性骨異形成症、線維性骨異形成症、骨の線維性異形成、播種性線維性骨炎

「繊維性骨異形成症」

　　[★]

英: fibrous dysplasia
関: 線維性骨異形成症、線維性骨異形成

「fibrous dysplasia of bone」

　　[★]

線維性骨異形成症、線維性骨異形成、骨の線維性異形成

関: fibrous dysplasia、osteitis fibrosa disseminata

「fibrous」

　　[★]

adj.

線維性の、線維状の

関: fibrotic、filamentous

[1] Candeliere GA, Glorieux FH, Prud'homme J, St-Arnaud R (June 1995). "Increased expression of the c-fos proto-oncogene in bone from patients with fibrous dysplasia". N. Engl. J. Med. 332 (23): 1546–51. doi:10.1056/NEJM199506083322304. PMID 7739708.

[2] Kashima TG, Nishiyama T, Shimazu K, et al. (February 2009). "Periostin, a novel marker of intramembranous ossification, is expressed in fibrous dysplasia and in c-Fos-overexpressing bone lesions". Hum. Pathol. 40 (2): 226–37. doi:10.1016/j.humpath.2008.07.008. PMID 18799196.

[3] Fibrous Dysplasia at eMedicine

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fibrous dysplasia