Fibromatosis
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Through Skeletal muscle
Diagnosis
Juvenile subtypes
Subtypes of juvenile fibromatosis include:
- Infantile myofibromatosis: solitary tumors commonly occurring in the head and neck regions; multiple tumors occurring in the skin, subcutaneous tissue, muscles, and/or less commonly bones; or, rarely, tumors occurring in an internal organ(s).
- Aponeurotic fibroma
- Infantile digital fibromatosis
- Aggressive infantile fibromatosis
- Fibromatosis colli: benign sternocleidomastoid muscle tumor developing in infants within 8 weeks (average: 24 days) of delivery. It generally does not require resection and responds well to physiotherapy.
- Dermatofibrosis lenticularis (Buschke–Ollendorff syndrome)
- Fibromatosis hyalinica multiplex (juvenile hyaline fibromatosis)
- Lipofibromatosis
Adult subtypes
Superficial
- Palmar fibromatosis (Dupuytren's contracture)
- Plantar fibromatosis (Ledderhose disease)
- Penile fibromatosis (Peyronie's disease)
- Pachydermodactyly
- Knuckle pads
- Dermatofibroma
- Nodular fasciitis
- Elastofibroma
- Fibrous papule of the face
Deep
- Aggressive fibromatosis (desmoid tumors)
- Abdominal, intra-abdominal, extra-abdominal
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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.
Treatment includes prompt radical excision with a wide margin and/or radiation. For aggressive fibromatosis, the consensus on treatment is observation for new tumors rather than immediate surgery. Despite their local infiltrative and aggressive behavior, mortality is minimal to nonexistent for peripheral tumors. 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.
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.