The term fibromatosis refers to a group of

soft tissue Soft tissue connective tissue, connects and surrounds or supports internal organs and bones, and includes muscle, tendons, ligaments, Adipose tissue, fat, fibrous tissue, Lymphatic vessel, lymph and blood vessels, fasciae, and synovial membranes.� ...

tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

s which have certain characteristics in common, including absence of cytologic and clinical

malignant Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...

features, a

histology Histology, also known as microscopic anatomy or microanatomy, is the branch of biology that studies the microscopic anatomy of biological tissue (biology), tissues. Histology is the microscopic counterpart to gross anatomy, which looks at large ...

consistent with proliferation of well-differentiated

fibroblasts A fibroblast is a type of biological cell typically with a spindle shape that synthesizes the extracellular matrix and collagen, produces the structural framework ( stroma) for animal tissues, and plays a critical role in wound healing. Fibrobla ...

, an infiltrative growth pattern, and aggressive clinical behavior with frequent local recurrence. It is classed by the

World Health Organization The World Health Organization (WHO) is a list of specialized agencies of the United Nations, specialized agency of the United Nations which coordinates responses to international public health issues and emergencies. It is headquartered in Gen ...

as an intermediate soft tissue tumor related to the

sarcoma A sarcoma is a rare type of cancer that arises from cells of mesenchymal origin. Originating from mesenchymal cells means that sarcomas are cancers of connective tissues such as bone, cartilage, muscle, fat, or vascular tissues. Sarcom ...

family.

Arthur Purdy Stout Arthur Purdy Stout (1885–1967) was an American surgeon and pathologist. Early years and education Arthur Purdy Stout was the fourth son of Joseph and Julia Frances (née Purdy) Stout. He attended the Pomfret School and Yale University, where he ...

coined the term ''fibromatosis'' (in the name ''

congenital generalized fibromatosis A birth defect is an abnormal condition that is present at birth, regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can range from mild to severe. Birth def ...

'', describing myofibromatosis), in 1954.

Diagnosis

Juvenile subtypes

Subtypes of juvenile fibromatosis include: *

Infantile myofibromatosis Infantile myofibromatosis (IMF) is a rare tumor found in 1 in 150,000 to 1 in 400,000 live births. It is nonetheless the most common tumor derived from fibrous connective tissue that occurs primarily in infants and young children. IMF tumors are ...

: 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 Peyronie's disease (PD) is a benign, acquired penile connective tissue disease characterized by the occurrence of fibrotic plaques within the Tunica albuginea (penis), tunica albuginea — the dense elastic covering of the Corpora cavernosa of pe ...

) * Pachydermodactyly * Knuckle pads * Dermatofibroma * Nodular fasciitis * Elastofibroma * Fibrous papule of the face

Deep

Aggressive fibromatosis Aggressive fibromatosis or desmoid tumor is a rare condition. Desmoid tumors are a type of fibromatosis and related to sarcoma, though without the ability to spread throughout the body (metastasize). The tumors arise from cells called fibrobla ...

(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 In physics, radiation is the emission or transmission of energy in the form of waves or particles through space or a material medium. This includes: * ''electromagnetic radiation'' consisting of photons, such as radio waves, microwaves, infr ...

. 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 Familial adenomatous polyposis (FAP) is an autosomal dominant inherited condition in which numerous adenomatous polyps form mainly in the epithelium of the large intestine. While these polyps start out benign, malignant transformation into colon ...

(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 Skeletal muscle (commonly referred to as muscle) is one of the three types of vertebrate muscle tissue, the others being cardiac muscle and smooth muscle. They are part of the somatic nervous system, voluntary muscular system and typically are a ...

, 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 Neurofibromatosis (NF) refers to a group of three distinct genetic conditions in which tumors grow in the nervous system. The tumors are non-cancerous (benign) and often involve the skin or surrounding bone. Although symptoms are often mild, e ...

References

External links

* {{Authority control Soft tissue disorders