The term fibromatosis refers to a group of

soft tissue Soft tissue is all the tissue in the body that is not hardened by the processes of ossification or calcification such as bones and teeth. Soft tissue connects, surrounds or supports internal organs and bones, and includes muscle, tendons, ...

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. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

features, a

histology Histology, also known as microscopic anatomy or microanatomy, is the branch of biology which studies the microscopic anatomy of biological tissues. Histology is the microscopic counterpart to gross anatomy, which looks at larger structures vi ...

consistent with proliferation of well-differentiated

fibroblasts A fibroblast is a type of biological cell that synthesizes the extracellular matrix and collagen, produces the structural framework ( stroma) for animal tissues, and plays a critical role in wound healing. Fibroblasts are the most common cells ...

, 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 specialized agency of the United Nations responsible for international public health. The WHO Constitution states its main objective as "the attainment by all peoples of the highest possible level of ...

as an intermediate soft tissue tumor related to the sarcoma family.

Diagnosis

Subtypes

Subtypes of fibromatosis include – * Juvenile fibromatosis *

Fibromatosis colli Fibromatosis colli (FMC), also termed sternocleidomastoid tumor of infancy, pseudotumor of infancy, and infancy sternocleidomastoid pseudotumor, is an uncommon (incidence: 0.4%–1.3% of live births), congenital tumor in one of the two sternocleid ...

: 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. * Infantile digital fibromatosis * 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). * Ipofibromatosis * Fibromatosis hyalinica multiplex * Penile fibromatosis (

Peyronie's disease Peyronie's disease is a connective tissue disorder involving the growth of fibrous plaques in the soft tissue of the penis. Specifically, scar tissue forms in the tunica albuginea, the thick sheath of tissue surrounding the corpora cavernosa, c ...

) * Palmar fibromatosis (

Dupuytren's contracture Dupuytren's contracture (also called Dupuytren's disease, Morbus Dupuytren, Viking disease, palmar fibromatosis and Celtic hand) is a condition in which one or more fingers become progressively bent in a flexed position. It is named after Guill ...

) * Plantar fibromatosis (

Ledderhose disease Plantar fascial fibromatosis, also known as Ledderhose's disease, Morbus Ledderhose, and plantar fibromatosis, is a relatively uncommon non-malignant thickening of the feet's deep connective tissue, or fascia. In the beginning, where nodules start ...

)

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. Despite their local infiltrative and aggressive behavior, mortality is minimal to nonexistent for peripheral tumours. 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, 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) is a group of three conditions in which tumors grow in the nervous system. The three types are neurofibromatosis type I (NF1), neurofibromatosis type II (NF2), and schwannomatosis. In NF1 symptoms include light brown sp ...

References

External links

* {{Authority control Soft tissue disorders