Fibromatosis, Clinical Image This fibromatosis is unusually superficial and arose in the chest wall of a young adult. These tumors are typically deep (deep fascia or within muscle).
Fibromatosis, Low Magnification This image of a deep-type fibromatosis shows sweeping fascicles of spindle cells separated by regularly spaced collagen. Even at this magnification, small vessels are readily apparent .
Fibromatosis, Gross Specimen This gross photograph shows a large, deep fibromatosis of the shoulder that has eroded into the scapula. Superficial portions of such lesions can be encountered on skin biopsies.
Fibromatosis, Neck This radiologic image shows a large fibromatosis of the head and neck in an elderly alcoholic man. Although more arise in the shoulder girdles (or in the abdominal wall in young women), the head and neck is a common location for fibromatoses.
TERMINOLOGY
Synonyms
• Deep fibromatosis: Aggressive fibromatosis, desmoid tumor
• Palmar fibromatosis: Nodular myofibroblastic proliferation of volar surface of hand that is prone to local persistence but does not metastasize
• Plantar fibromatosis: Nodular myofibroblastic proliferation of plantar surface of foot that is prone to local persistence but does not metastasize
• Peyronie disease: Penile fibrous lesion causing various deformities; initially pain with erection, erectile dysfunction
• Knuckle pads: Well-circumscribed thickening of skin over metacarpophalangeal and, more commonly, proximal interphalangeal joints
• Deep fibromatosis: Myofibroblastic proliferation of deep soft tissues with infiltrative growth pattern; prone to local recurrences but does not metastasize
CLINICAL ISSUES
Epidemiology
• Incidence
Palmar fibromatosis
– 4-6% of Caucasian adults > 50 years of age; reports of up to 75% of Celtic male patients
– Uncommon in nonwhites; marked male predominance
Plantar fibromatosis
– 1-2 per 100,000 persons per year (Northern Europe)
– Most patients 30-50 years of age; slight male predominance
Penile fibromatosis
– ~ 3.5% of white men > 50 years of age
Deep fibromatosis
– 2.40-4.43 new cases per 100,000 persons per year (Scandinavian data)
Knuckle pads (rare)
Presentation
• Superficial fibromatoses present as nodular lesions on palms, soles, knuckles, or penis
Variable tenderness
• Deep fibromatoses present as firm large masses, typically with intramuscular component
Relationship to age and gender
– In children and older adults, no gender predominance: Lesions of shoulders, chest wall, back, thigh, head, and neck
– In women in childbearing years: Abdominal wall
May also arise in shoulder girdles, chest wall, back, thigh, head, and neck
Usually clinically concerning for sarcomas based on large size, deep location
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Relationship to age and gender
