Fibromatosisfibromatosis, fibromatosis colli
The term fibromatosis refers to a group of soft tissue tumors1 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 It is classed by the world health organisation as an intermediate soft tissue tumor related to the sarcoma family
- 1 Terminology
- 2 Treatment
- 3 Subtypes
- 4 Associated conditions
- 5 References
- 6 External links
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 is mainly surgical; radiotherapy or chemotherapy is usually an indication of relapseclarification needed 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 disorders2
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 low3
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
- Fibromatosis hyalinica multiplex
- Plantar fibromatosis
- Penile fibromatosis Peyronie's disease
- Palmar fibromatosis Dupuytren's contracture
Multiple fibromatoses are seen in Gardner's syndrome, a syndrome which also includes multiple colon polyps and osteomas
- ^ "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 JE, Thomas JM, Hayes AJ, Strauss DC 2012 "Surgical resection for non familial adenomatous polyposis related intra-abdominal fibromatosis" British Journal of Surgery 99 5: 706–713 PMID 22359346 doi:101002/bjs8703
- derm/778 at eMedicine
|Not otherwise specified||
|Connective tissue neoplasm||
|Complex mixed and stromal||
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