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Arch Otolaryngol Head Neck Surg. 2008;134(5):560-561.

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Diagnosis: Fibromatosis (desmoid tumor) of the neck

Fibromatosis was first described in 1832.¹ It is a rare, cytologically bland, monoclonal fibroblastic proliferation that arises within the musculoaponeurotic structures throughout the body.^1-4 The lesion is frequently referred to as a desmoid tumor because of its tendonlike appearance.^4-5 Several other terms have been used to describe this disorder, including aggressive fibromatosis, desmoplastic fibroma, desmoma, well-differentiated nonmetastasizing fibrosarcoma, and grade I fibrosarcoma.⁵ There may be a genetic component in the pathogenesis of fibromatosis, as demonstrated by a cumulative lifetime risk of 20.6% in patients with familial adenomatous polyposis.¹ Other factors, such as trauma and endocrine, also seem to be associated with the development of fibromatosis, but the exact cause is still unknown.^{1, 4-5}

Fibromatosis is an extremely uncommon entity, with an incidence of 2 to 4 cases per million individuals per year; approximately one-third of the cases are localized in the extra-abdominal region.⁶ Only 11% to . . . [Full Text of this Article]

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Arch Otolaryngol Head Neck Surg. 2008;134(5):559.
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