WordNet

autosomal dominant disease characterized by numerous neurofibromas and by spots on the skin and often by developmental abnormalities (同)von Recklinghausen''s disease

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/08 04:33:27」(JST)

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The term fibromatosis refers to a group of benign soft tissue tumors^[1] which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressive clinical behavior with frequent local recurrence.

Terminology

Other names include "musculoaponeurotic fibromatosis," referring to the tendency of these tumors to be adjacent to and infiltrating deep skeletal muscle, aggressive fibromatosis and "desmoid tumor." A clear difference should be made between intra-abdominal and extra-abdominal localizations. Fibromatosis is a different entity from neurofibromatosis.

Treatment

Treatment is mainly surgical; radiotherapy or chemotherapy is usually an indication of relapse. Head and neck desmoid fibromatosis is a serious condition due to local aggression, specific anatomical patterns and the high rate of relapse. For children surgery is particularly difficult, given the potential for growth disorders.^[2]

Treatment includes prompt radical excision with a wide margin and/or radiation. Despite their local infiltrative and aggressive behavior, mortality is minimal to nonexistent for peripheral tumours. In intra-abdominal fibromatosis associated with Familial adenomatous polyposis (FAP), surgery is avoided if possible due to high rates of recurrence within the abdomen carrying significant morbidity and mortality. Conversely, for intra-abdominal fibromatosis without evidence of FAP extensive surgery may still be required for local symptoms, but the risk of recurrence is low.^[3]

Subtypes

Subtypes of fibromatosis include -

juvenile fibromatosis
fibromatosis colli: Non-neoplastic sternocleidomastoid muscle enlargement in early infancy. Does not generally require resection and responds well to physiotherapy.
infantile digital fibromatosis
infantile myofibromatosis
ipofibromatosis
fibromatosis hyalinica multiplex
plantar fibromatosis
penile fibromatosis (Peyronie's disease)
palmar fibromatosis (Dupuytren's contracture)

Associated conditions

Multiple fibromatoses are seen in Gardner's syndrome, a syndrome which also includes multiple colon polyps and osteomas.

References

^ "fibromatosis" at Dorland's Medical Dictionary
^ Cecilia Petrovan, Diana Nekula (Nov 2011). "Submandibular juvenile desmoid fibromatosis: case report of a 2 years old child". Rev. chir. oro-maxilo-fac. implantol. (in Romanian) 2 (3): 15–19. ISSN 2069-3850. 41. Retrieved 2012-06-06. (webpage has a translation button)
^ Wilkinson MJ, Fitzgerald JEF, Thomas JM, Hayes AJ, Strauss DC. Surgical resection for non familial adenomatous polyposis related intra-abdominal fibromatosis. British Journal of Surgery 2012; 99: 706–713. DOI: 10.1002/bjs.8703. PMID 22359346

External links

derm/778 at eMedicine
maxillofacialcenter

UpToDate Contents

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1. 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
2. デスモイド腫瘍：全身療法 desmoid tumors systemic therapy
3. ガングリオンと結節 ganglia and nodules
4. デュピュイトラン拘縮 dupuytrens contracture
5. 後腹膜線維症の臨床症状および診断 clinical manifestations and diagnosis of retroperitoneal fibrosis

English Journal

Juvenile psammomatoid ossifying fibroma of the orbit and paranasal sinuses. A case report.

Linhares P, Pires E, Carvalho B, Vaz R.SourceDepartment of Neurosurgery, Hospital de Sao Joao, Alameda Prof. Hernani Monteiro, 4200-319, Porto, Portugal.
Acta neurochirurgica.Acta Neurochir (Wien).2011 Oct;153(10):1983-8. Epub 2011 Aug 10.
Juvenile psammomatoid ossifying fibroma (JPOF) is an uncommon benign fibro-osseous lesion predominantly arising in the paranasal sinuses and orbits of children and young adults. We report a case of JPOF involving the paranasal sinuses and orbit in a 15-year-old boy that presented due to progressive
PMID 21826543

Japanese Journal

E-16)Deep fibromatosisの一例(E 骨軟部腫瘍,2010年度小児腫瘍症例検討会)

大喜多肇,松岡健太郎,中野夏子,中澤温子
小児がん : 小児悪性腫瘍研究会記録 48(1), 55, 2011-02-25
NAID 110008608603

Related Pictures

★リンクテーブル★

リンク元	「線維腫症」
拡張検索	「type 2 neurofibromatosis」「neurofibromatosis 1 gene」

「線維腫症」

Fibromatosis
	The cut surface of desmoid-type fibromatosis is firm, white, and whorled. The white tumor infiltrates the adjacent skeletal muscle (red tissue - lower left) and fat (yellow tissue - upper left). This tendency for invasion of adjacent normal tissues and structures is the reason that desmoid-type fibromatosis has a relatively high rate of local recurrence, even after surgical removal.
Classification and external resources
ICD-10	M72.9
ICD-9	728.7
MeSH	D005350