Fibromatosis
Encyclopedia
The term fibromatosis refers to a group of benign
soft tissue
tumor
s (fibroma
s) 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.
, and "desmoid tumor," an obsolete term which more specifically refers to the occurrence of these tumors in the abdominal wall of a pregnant woman.
Fibromatosis is a different entity from neurofibromatosis
.
. Despite the local infiltrative and aggressive behavior of these tumors, mortality secondary to these tumors is minimal to nonexistent.
, a syndrome which also includes multiple colon polyps and osteomas.
Benign
A benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...
soft tissue
Soft tissue
In anatomy, the term soft tissue refers to tissues that connect, support, or surround other structures and organs of the body, not being bone. Soft tissue includes tendons, ligaments, fascia, skin, fibrous tissues, fat, and synovial membranes , and muscles, nerves and blood vessels .It is sometimes...
tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
s (fibroma
Fibroma
Fibromas are benign tumors that are composed of fibrous or connective tissue. They can grow in all organs, arising from mesenchyme tissue. The term "fibroblastic" or "fibromatous" is used to describe tumors of the fibrous connective tissue...
s) which have certain characteristics in common, including absence of cytologic and clinical malignant
Malignant
Malignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis...
features, a histology
Histology
Histology is the study of the microscopic anatomy of cells and tissues of plants and animals. It is performed by examining cells and tissues commonly by sectioning and staining; followed by examination under a light microscope or electron microscope...
consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressive clinical behavior with frequent local recurrence.
Terminology
Other names include "musculoaponeurotic fibromatosis," referring to the tendency of these tumors to be adjacent to and infiltrating deep skeletal muscleSkeletal muscle
Skeletal muscle is a form of striated muscle tissue existing under control of the somatic nervous system- i.e. it is voluntarily controlled. It is one of three major muscle types, the others being cardiac and smooth muscle...
, and "desmoid tumor," an obsolete term which more specifically refers to the occurrence of these tumors in the abdominal wall of a pregnant woman.
Fibromatosis is a different entity from neurofibromatosis
Neurofibromatosis
Neurofibromatosis is a genetically-inherited disorder in which the nerve tissue grows tumors that may be benign or may cause serious damage by compressing nerves and other tissues...
.
Treatment
Treatment includes prompt radical excision with a wide margin and/or radiationRadiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
. Despite the local infiltrative and aggressive behavior of these tumors, mortality secondary to these tumors is minimal to nonexistent.
Subtypes
Subtypes of fibromatosis include -- juvenile fibromatosis
- fibromatosis colliFibromatosis colliFibromatosis colli is a benign proliferation of fibrous tissue infiltrating the lower third of the sternocleidomastoid or should region, and is the most common cause of neonatal torticollis....
: Non-neoplastic sternocleidomastoid muscle enlargement in early infancy. Does not generally require resection and responds well to physiotherapy. - infantile digital fibromatosis
- infantile myofibromatosis
- ipofibromatosis
- fibromatosis hyalinica multiplex
- plantar fibromatosis
- penile fibromatosis (Peyronie's diseasePeyronie's diseasePeyronie's Disease Peyronie's Disease Peyronie's Disease (also known as "Induratio penis plastica", or more recently Chronic Inflammation of the Tunica Albuginea (CITA), is a connective tissue disorder involving the growth of fibrous plaques in the soft tissue of the penis affecting up to 10% of men...
) - palmar fibromatosis (Dupuytren's contractureDupuytren's contractureDupuytren's contracture , is a fixed flexion contracture of the hand where the fingers bend towards the palm and cannot be fully extended...
)
Associated conditions
Multiple fibromatoses are seen in Gardner's syndromeGardner's syndrome
Gardner syndrome, also known as familial colorectal polyposis, is an autosomal dominant form of polyposis characterized by the presence of multiple polyps in the colon together with tumors outside the colon...
, a syndrome which also includes multiple colon polyps and osteomas.