Fibromatosis



Fibromatosis


Elizabeth A. Montgomery, MD









Gross photograph shows a large deep fibromatosis involving the shoulder. This lesion has eroded into the scapula, although typically fibromatoses do not erode bone.






Hematoxylin & eosin shows sweeping fascicles of spindle cells separated by regularly spaced collagen. Even at this magnification, small vessels image are readily apparent.


TERMINOLOGY


Synonyms



  • Superficial fibromatoses



    • Palmar fibromatosis: Dupuytren contracture, Dupuytren disease


    • Plantar fibromatosis: Ledderhose disease


    • Penile fibromatosis: Peyronie disease


    • Knuckle pads


  • Deep fibromatosis: Aggressive fibromatosis, desmoid tumor


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


    • Some associated with Dupuytren contractures, most idiopathic


  • Deep fibromatosis



    • Myofibroblastic proliferations of deep soft tissues with infiltrative growth pattern


    • Prone to local recurrences but do not metastasize


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Palmar fibromatosis



      • 4-6% of Caucasian adults over 50 years of age


      • Reports of up to 75% of Celtic men


      • 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 over 50 years of age


    • Deep fibromatosis



      • 2.4-4.43 new cases per 100,000 persons per year (Scandinavian data)


Presentation



  • Superficial fibromatoses present as nodular lesions on palms, soles, knuckles, or penis



    • Most common in older white men


  • Deep fibromatoses present as firm large masses



    • Relationship to age and gender



      • In children, no gender predominance: Lesions of shoulders, chest wall, back, thigh, head, and neck


      • In women in childbearing years: Abdominal wall


      • In older adults, no gender predominance: Lesions of shoulders, chest wall, back, thigh, head, and neck


    • Mesenteric fibromatoses



      • Most have asymptomatic abdominal mass


      • Gastrointestinal hemorrhage or perforation


  • Lesions associated with familial adenomatous polyposis (FAP)



    • Risk of fibromatoses is 2.56/1,000 person-years


    • Comparative risk is 852x that of general population


  • Occasionally associated with scar (“cicatricial fibromatosis”)


Treatment



  • Superficial fibromatoses treated by excision




  • Nonsurgical treatments for penile lesions



    • Verapamil treatment administered by intraplaque injection


    • Colchicine, aminobenzoate potassium (Potaba), L-carnitine, and liposomal superoxide dismutase


  • Deep fibromatoses treated by wide excision



    • For unresectable lesions, radiation, chemotherapy, hormone therapy


Prognosis



  • Recurrences common for both superficial and deep fibromatoses


  • Occasional deaths from deep fibromatoses



    • FAP-associated mesenteric fibromatosis


MACROSCOPIC FEATURES


General Features



  • Superficial fibromatoses



    • Small (1-3 cm), nodular, firm, white lesions; some with gritty cut surface


    • Large, infiltrative, firm, white lesions; some with gritty cut surface


MICROSCOPIC PATHOLOGY


Histologic Features

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Fibromatosis
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