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Fibromatosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Fibromyxoma; myxofibroma; musculoaponeurotic fibromatosis

Overview

Overview

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. 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. 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.

Classification

Classification

Subtypes of fibromatosis include juvenile fibromatosis, fibromatosis colli, infantile digital fibromatosis, infantile myofibromatosis, lipofibromatosis, fibromatosis hyalinica multiplex, penile fibromatosis (Peyronie’s disease), and palmar fibromatosis (Dupuytren’s contracture). Multiple fibromatoses are seen in Gardner’s syndrome, a syndrome which also includes multiple colon polyps and osteomas. Fibromatosis is a different entity from neurofibromatosis.

References

References

Template:Diseases of the musculoskeletal system and connective tissue


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