Fibromatosis

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Fibromatosis
Classification and external resources
Desmoid-type fibromatosis.gross pathology.jpg
The cut surface of desmoid-type fibromatosis is firm, white, and whorled. The white tumor infiltrates the adjacent skeletal muscle (red tissue - lower left) and fat (yellow tissue - upper left). This tendency for invasion of adjacent normal tissues and structures is the reason that desmoid-type fibromatosis has a relatively high rate of local recurrence, even after surgical removal.
ICD-10M72.9
ICD-9728.7
MeSHD005350
 
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Fibromatosis
Classification and external resources
Desmoid-type fibromatosis.gross pathology.jpg
The cut surface of desmoid-type fibromatosis is firm, white, and whorled. The white tumor infiltrates the adjacent skeletal muscle (red tissue - lower left) and fat (yellow tissue - upper left). This tendency for invasion of adjacent normal tissues and structures is the reason that desmoid-type fibromatosis has a relatively high rate of local recurrence, even after surgical removal.
ICD-10M72.9
ICD-9728.7
MeSHD005350

The term fibromatosis refers to a group of benign soft tissue tumors[1] 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.

Terminology[edit]

Other names include "musculoaponeurotic fibromatosis," referring to the tendency of these tumors to be adjacent to and infiltrating deep skeletal 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.

Treatment[edit]

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.[2]

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 (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.[3]

Subtypes[edit]

Subtypes of fibromatosis include -

Associated conditions[edit]

Multiple fibromatoses are seen in Gardner's syndrome, a syndrome which also includes multiple colon polyps and osteomas.

References[edit]

External links[edit]