Published online by Cambridge University Press: 01 March 2011
The designation fibromatosis refers to a specific group of locally recurring and potentially aggressive but nonmetastasizing fibroblastic and myofibroblastic tumors. These include palmar and plantar fibromatosis, which are often together referred to as superficial fibromatoses. Penile fibromatosis (Peyronie's disease) is often attached to this group, although it is not certain whether it is truly comparable with other fibromatoses.
Deep, fascia-associated or intramuscular fibromatoses are called desmoid tumors, synonymously known as aggressive fibromatoses and musculoaponeurotic fibromatoses. All fibromatoses are composed of fibroblasts with a substantial myofibroblastic component. General features and clonal chromosomal changes suggest that both superficial and deep fibromatoses are clonal, neoplastic processes. Although there have been misgivings about the continuing use of the term desmoid fibromatosis (or briefly, desmoid), the existence of different types of fibromatoses (especially in childhood) justifies this as a specifying designation.
Some fibromatoses, such juvenile fibromatosis and lipofibromatosis, occur exclusively in children. Several childhood conditions called fibromatosis are not true fibromatoses, but reactive processes (fibromatosis colli), peculiar hyperplastic processes (gingival fibromatosis), or aberrations in morphogenesis (juvenile hyaline fibromatosis). These childhood lesions termed fibromatoses are discussed in Chapter 9.
PALMAR AND PLANTAR FIBROMATOSIS
Clinical Features
Palmar fibromatosis is also known as Dupuytren's contracture, named after an early nineteenth-century French surgeon who described this condition. Palmar fibromatosis is common especially in men of northern and eastern European, Scottish, and Irish descent.
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