Fibromatosis
Elizabeth A. Montgomery, MD
Key Facts
Terminology
Deep fibromatosis: Desmoid tumor
Palmar fibromatosis: Dupuytren contracture
Plantar fibromatosis: Ledderhose disease
Penile fibromatosis: Peyronie disease
Myofibroblastic proliferations with infiltrative growth pattern that show local recurrences but not metastasis
Clinical Issues
Palmar fibromatosis
4-6% of Caucasian adults > 50 years of age
Deep fibromatosis
2.4-4.43 new cases per 100,000 persons per year
Recurrences common for both superficial and deep fibromatoses, but deaths only from deep fibromatoses
Increased incidence in familial adenomatous polyposis (FAP)
Microscopic Pathology
Sweeping fascicles of myofibroblasts
Smooth nuclear membranes and small nucleoli in most cells
Occasional cells with stellate cytoplasmic contours
Occasional foci with storiform pattern similar to nodular fasciitis
Some cases show keloid-like collagen
Small but conspicuous vessels
Gaping, thin-walled vessels with perivascular sclerosis often feature of mesenteric fibromatosis
Ancillary Tests
β-catenin positive (nuclear), especially in deep lesions, SMA(+); desmin(−), keratins(−), CD117(+/−)
 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. |
 Hematoxylin & eosin shows sweeping fascicles of spindle cells separated by regularly spaced collagen. Even at this magnification, small vessels are readily apparent  . |
TERMINOLOGY
Synonyms
Deep fibromatosis: Aggressive fibromatosis, desmoid tumor
Superficial fibromatoses: Palmar fibromatosis (Dupuytren contracture, Dupuytren disease), plantar fibromatosis (Ledderhose disease), penile fibromatosis (Peyronie disease), knuckle pads
Definitions
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 males
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
About 3.5% of white men > 50 years of age
Deep fibromatosis
2.4-4.43 new cases per 100,000 persons per year (Scandinavian data)
Knuckle pads (rare)
