Fibromatosis
poorly defined neoplasm arising from the soft tissues
The term fibromatosis refers to a group of soft tissue tumors 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 Organization as an intermediate soft tissue tumor related to the sarcoma family. Arthur Purdy Stout coined the term fibromatosis (in the name congenital generalized fibromatosis, describing myofibromatosis), in 1954.
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.
Treatment includes prompt radical excision with a wide margin and/or radiation.
Begin with the source’s own compact description: “Fibromatosis” is poorly defined neoplasm arising from the soft tissues. The dossier treats that line as a proposition to test through Fibromatosis, poorly and defined, not as a finished interpretation.
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This entry incorporates text from “Fibromatosis” on English Wikipedia. Contributors are listed in the page history. Text is available under the Creative Commons Attribution-ShareAlike 4.0 License. Selected authority identifiers and statements are retrieved from Wikidata under CC0; their references and qualifiers remain part of the verification path.