Fibromatoses (overview) - Altmeyers Encyclopedia

Synonym(s)

Fibromatosis; Myofibroblastosis infantile

Definition
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Heterogeneous group of neoplasms of the connective tissue, which nosologically stand between reactive neoplasms and true neoplasias. Nevertheless, they show locally infiltrating and destructive growth but without metastatic tendency. Especially congenital forms and familial occurrence and the detection of mutations (see below fibromatosis, hyaline juvenile) speak for a genetic basis at least for some members of this disease group.

Classification
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The skin, oral mucosa, fascia and musculature (more rarely in internal organs) are differentiated:

Superficial (superficial) fibromatosis (or Dupuytren's group):
- Dupuytren's contracture (palmar fibromatosis)
- plastic penis induratio
- Plantar fibromatosis
- Knuckle pads real.
Deep fibromatosis (or desmoid group):
- Desmoid (intraabdominal and extraabdominal [in scars]).
Infantile fibromatosis:
- Hamartoma, fibrous of childhood
- Myofibromatosis, infantile (fibromatosis, congenital, generalized)
- Fibromatosis gingivae
  - Fibromatosis gingivae with hypertrichosis lanuginosa
  - Fibromatosis gingivae with fibroadenomatosis mammae
- Fibromatosis, digital infantile
- Fibromatosis, hyaline juvenile (Fibromatosis, infantile aggressive)
- Fibromatosis, infantile, desmoid
- Fibromatosis colli.

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Progression/forecast
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See below the individual clinical pictures.

Note(s)
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Proliferation from fibroblasts and myofibroblasts, which initially have a cell-rich stage, later a cell-poor, fibrous stage.

Literature
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Burgdorf WHC et al (2003) Benign and malignant tumors. in: Schachner et al. (eds). Pediatric dermatology. Edinburgh, Mosby, S. 863-899
de Bree E et al (2004) Incidence and treatment of recurrent plantar fibromatosis by surgery and postoperative radiotherapy. On J Surgery 187: 33-38
Hanks S et al (2003) Mutations in the gene encoding capillary morphogenesis protein 2 cause juvenile hyaline fibromatosis and infantile systemic hyalinosis. On J Hum Genet73: 791-800
Kadwa S (2003) Juvenile aggressive fibromatosis. SADJ 58: 299-302
Graells Estrada J et al (2003) Familial plantar fibromatosis. Clin Exp Dermatol 28: 669-670
Ulrich D et al (2003) Matrix metalloproteinases and tissue inhibitors of metalloproteinases in sera and tissue of patients with Dupuytren's disease. Plast Reconstr Surgery 112: 1279-1286