Fibromatosis



Fibromatosis


Elizabeth A. Montgomery, MD










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 image.


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)

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Fibromatosis
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