線維腫症
WordNet
- autosomal dominant disease characterized by numerous neurofibromas and by spots on the skin and often by developmental abnormalities (同)von Recklinghausen''s disease
Wikipedia preview
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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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.
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Classification and external resources |
ICD-10 |
M72.9 |
ICD-9 |
728.7 |
MeSH |
D005350 |
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.
Contents
- 1 Terminology
- 2 Treatment
- 3 Subtypes
- 4 Associated conditions
- 5 References
- 6 External links
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
Connective/soft tissue tumors and sarcomas (ICD-O 8800–9059) (C45–C49/D17–D21, 171/214–215)
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Not otherwise specified |
- Soft-tissue sarcoma
- Desmoplastic small-round-cell tumor
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Connective tissue neoplasm |
Fibromatous |
Fibroma/fibrosarcoma: |
- Dermatofibrosarcoma protuberans
- Desmoplastic fibroma
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Fibroma/fibromatosis: |
- Aggressive infantile fibromatosis
- Aponeurotic fibroma
- Collagenous fibroma
- Diffuse infantile fibromatosis
- Familial myxovascular fibromas
- Fibroma of tendon sheath
- Fibromatosis colli
- Infantile digital fibromatosis
- Juvenile hyaline fibromatosis
- Plantar fibromatosis
- Pleomorphic fibroma
- Oral submucous fibrosis
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Histiocytoma/histiocytic sarcoma: |
- Benign fibrous histiocytoma
- Malignant fibrous histiocytoma
- Atypical fibroxanthoma
- Solitary fibrous tumor
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Myxomatous |
- Myxoma/myxosarcoma
- Cutaneous myxoma
- Superficial acral fibromyxoma
- Angiomyxoma
- Ossifying fibromyxoid tumour
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Fibroepithelial |
- Brenner tumour
- Fibroadenoma
- Phyllodes tumor
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Synovial-like |
- Synovial sarcoma
- Clear-cell sarcoma
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Lipomatous |
- Lipoma/liposarcoma
- Myelolipoma
- Myxoid liposarcoma
- PEComa
- Chondroid lipoma
- Intradermal spindle cell lipoma
- Pleomorphic lipoma
- Lipoblastomatosis
- Spindle cell lipoma
- Hibernoma
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Myomatous |
general: |
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smooth muscle: |
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skeletal muscle: |
- Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma
- Alveolar rhabdomyosarcoma
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- Leiomyoma
- Angioleiomyoma
- Angiolipoleiomyoma
- Genital leiomyoma
- Leiomyosarcoma
- Multiple cutaneous and uterine leiomyomatosis syndrome
- Multiple cutaneous leiomyoma
- Neural fibrolipoma
- Solitary cutaneous leiomyoma
- STUMP
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Complex mixed and stromal |
- Adenomyoma
- Pleomorphic adenoma
- Mixed Müllerian tumor
- Mesoblastic nephroma
- Wilms' tumor
- Malignant rhabdoid tumour
- Clear-cell sarcoma of the kidney
- Hepatoblastoma
- Pancreatoblastoma
- Carcinosarcoma
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Mesothelial |
- Mesothelioma
- Adenomatoid tumor
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Index of muscle
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Description |
- Anatomy
- head
- neck
- arms
- chest and back
- diaphragm
- abdomen
- genital area
- legs
- Muscle tissue
- Physiology
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Disease |
- Myopathy
- Soft tissue
- Connective tissue
- Congenital
- abdomen
- muscular dystrophy
- Neoplasms and cancer
- Injury
- Symptoms and signs
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Treatment |
- Procedures
- Drugs
- anti-inflammatory
- muscle relaxants
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Soft tissue disorders / Rheumatism / Connective tissue arthropathy (M65–M79, 725–728)
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Capsular joint |
Synoviopathy |
- Synovitis/Tenosynovitis
- Calcific tendinitis
- Stenosing tenosynovitis
- Trigger finger
- DeQuervain's syndrome
- Transient synovitis
- Ganglion cyst
- osteochondromatosis
- Synovial osteochondromatosis
- Plica syndrome
- villonodular synovitis
- Giant cell tumor of the tendon sheath
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Bursopathy |
- Bursitis
- Olecranon
- Prepatellar
- Trochanteric
- Subacromial
- Achilles
- Retrocalcaneal
- Ischial
- Iliopsoas
- Synovial cyst
- Calcific bursitis
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Noncapsular joint |
Ligamentopathy |
- Ligamentous laxity
- Hypermobility
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Enthesopathy/Enthesitis
(and general tendinopathy) |
upper limb |
- Adhesive capsulitis of shoulder
- Impingement syndrome Rotator cuff tear
- Golfer's elbow
- Tennis elbow
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lower limb |
- Iliotibial band syndrome
- Patellar tendinitis
- Achilles tendinitis
- Calcaneal spur
- Metatarsalgia
- Bone spur
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other/general: |
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Nonjoint |
Fasciopathy |
- Fasciitis: Plantar
- Nodular
- Necrotizing
- Eosinophilic
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Fibromatosis/contracture |
- Dupuytren's contracture
- Plantar fibromatosis
- Aggressive fibromatosis
- Knuckle pads
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Index of joint
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Description |
- Anatomy
- head and neck
- cranial
- arms
- torso and pelvis
- legs
- bursae and sheathes
- Physiology
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Disease |
- Arthritis
- acquired
- back
- childhood
- soft tissue
- Congenital
- Injury
- Symptoms and signs
- Examination
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Treatment |
- Procedures
- Drugs
- rheumatoid arthritis
- gout
- topical analgesics
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Index of muscle
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Description |
- Anatomy
- head
- neck
- arms
- chest and back
- diaphragm
- abdomen
- genital area
- legs
- Muscle tissue
- Physiology
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Disease |
- Myopathy
- Soft tissue
- Connective tissue
- Congenital
- abdomen
- muscular dystrophy
- Neoplasms and cancer
- Injury
- Symptoms and signs
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Treatment |
- Procedures
- Drugs
- anti-inflammatory
- muscle relaxants
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UpToDate Contents
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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)
Related Links
- The term fibromatosis refers to a group of benign soft tissue tumors (fibromas) which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of ...
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- 英
- fibromatosis
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- 線維腫
[★]
神経線維腫症2型
- 関
- acoustic neuroma、acoustic schwannoma、bilateral acoustic neurofibromatosis、neurofibromatosis 2、neurofibromatosis II、neurofibromatosis type 2、neurofibromatosis type II、type II neurofibromatosis
[★]
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