Fibromatosis
| Fibromatosis | |
|---|---|
| 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 (fibromas)[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.
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[edit] Terminology
Other names include "musculoaponeurotic fibromatosis," referring to the tendency of these tumors to be adjacent to and infiltrating deep skeletal muscle, and "desmoid tumor," an obsolete term which more specifically refers to the occurrence of these tumors in the abdominal wall of a pregnant woman.
Fibromatosis is a different entity from neurofibromatosis.
[edit] 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 the local infiltrative and aggressive behavior of these tumors, mortality secondary to these tumors is minimal to nonexistent.
[edit] 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)
[edit] Associated conditions
Multiple fibromatoses are seen in Gardner's syndrome, a syndrome which also includes multiple colon polyps and osteomas.
[edit] 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". Revista de chirurgie oro-maxilo-facială și implantologie 2 (3): 15–19. http://www.revistaomf.ro/(1)Colectia-pe-ani/(15)Anul-2011/(37)Numarul-3-2011/(41)Prezentarea-unui-caz-clinic-defibromatoza-desmoida-infantila-submandibulara-la-un-copil-de-2-ani.(in Romanian, webpage has a translation button)
[edit] External links
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