Fibromatosis

The term fibromatosis refers to a group of benign 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.

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Terminology

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

Treatment includes prompt radical excision with a wide margin and/or radiation. 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 colli
• infantile digital fibromatosis
• infantile myofibromatosis
• ipofibromatosis
• fibromatosis hyalinica multiplex
• plantar fibromatosis
• penile fibromatosis (Peyronie's disease)
• palmar fibromatosis (Dupuytren's contracture).

Associated conditions

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

References

• derm/778 at eMedicine
• maxillofacialcenter
• Rosai, J. Rosai and Ackerman's Surgical Pathology. Mosby Inc., 2004. pg 2249-2252.