Fibromatosis

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

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.(webpage has a translation button)

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.

References

References

1. {{DorlandsDict. three/000040062. fibromatosis
2. (1999-01-01). "Pediatric Myofibromatosis of the Head and Neck". [[Archives of Otolaryngology–Head & Neck Surgery]].
3. "Fibromatosis".
4. (January 2021). "Primary Resection and Immediate Autologous Reconstruction of Fronto-orbital Infantile Myofibromatoses". Plastic and Reconstructive Surgery. Global Open.
5. (April 2021). "Bilateral sternocleidomastoid pseudotumors-a case report and literature review". Radiology Case Reports.
6. (October 2017). "An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)". [[Annals of Oncology]].
7. (2012). "Surgical resection for non familial adenomatous polyposis related intra-abdominal fibromatosis". [[Semantic Scholar]].