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Desmoid

Etiology: • Unknown.

Pathogenesis: • The lesion represents an intermediate between a reparative lesion and a true malignancy.

Epidemiology: • The intra-abdominal variety is associated with pregnancy. • The extra-abdominal variety is seen equally in men and women. • The tumor is common in patients with Gardner's syndrome, a disease associated with a diverse array of neoplastic lesions, including colonic polyps, osteomas and epidermal inclusion cysts.

General Gross Description: • Also known as aggesssive fibromatosis. • Lesions characterized by tan-white poorly demarcated masses. • They are rubbery, firm and blend into the surrounding tissue.

General Microscopic Description: • Histologically characterized by active fibroblasts within a dense collagenous matrix. • Mitotic activity is not frequent. • Fragments of skeletal muscle cells can be seen embedded within the tumor.

Clinical Correlations: • Desmoids occur in two locations: intra-abdominal and extra-abdominal. • The abdominal variety is frequently seen in the anterior abdominal wall of women during or after pregnancy.

References: •Robbins "Pathologic Basis of Disease". (Cotran, Kumar and Robbins, Eds.) 5th Edition. pp 90, 1265.