Soft tissue
Fibroblastic / myofibroblastic tumors
Fibromatosis - superficial

Author: Komal Arora, M.D. (see Authors page)

Revised: 17 August 2016, last major update July 2012

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Fibromatosis [title] superficial

Definition / General
Palmar fibromatosis (Dupuytren's contracture)
  • Nodular proliferative process of palmar aponeurosis, surrounding adipose and occasionally dermis, due to fibroblasts, myofibroblasts and fibrocytes
  • Most common type of fibromatosis (1 - 2% of population), prevalence increases with age (24% at age 65+)

Plantar fibromatosis (Ledderhose's disease)
  • Nodular proliferative process of plantar aponeurosis and surrounding adipose due to fibroblasts, myofibroblasts and fibrocytes
  • Heterogeneous group of conditions with plantar location, mature collagen and fibroblasts, but no malignant features (eMedicine)

Penile fibromatosis (Peyronie's)
  • Fibrous thickening of dermis and Buck's fascia between corpora cavernosa and tunica albuginea, causing curvature towards side of lesion and restricting movement of these structures during erection
  • Etiology may be related to Parc protein (BJU Int 2010;106:1706) or Wnt2 (J Sex Med 2012;9:1430)
Epidemiology
Palmar fibromatosis (Dupuytren's contracture)
  • 75% are men
  • 50% are bilateral, 10% also have plantar disease, 1 - 4% have penile fibromatosis

Plantar fibromatosis (Ledderhose's disease)
  • Common in boys < 10 years old and teenagers
  • Associated with palmar and penile fibromatosis, also continuous phenobarbital treatment for epilepsy (Epilepsia 2008;49:1965)
  • Usually NOT associated with contractures

Penile fibromatosis (Peyronie's)
Clinical Features
Palmar fibromatosis (Dupuytren's contracture)
  • Puckers overlying skin as it ages
  • Causes flexion contracture of digits 4 and 5 due to cord-like expansion of digital aponeurotic slips
  • Does not involve deep structures such as tendons or skeletal muscle
  • May be caused by fibrogenic cytokines (J Hand Surg Br 2005;30:557)

Plantar fibromatosis (Ledderhose's disease)
  • Only 10 - 25% bilateral
  • May be nodular Often presents with firm subcutaneous nodule or thickening associated with pain after standing or walking typically on the medial aspect of the sole
  • Clinically resembles melanoma, synovial sarcoma, Kaposi's sarcoma

Case Reports
Penile fibromatosis (Peyronie's)
Treatment
Palmar fibromatosis (Dupuytren's contracture)
  • Observation, excision or incision of contracture band
  • Often recurs

Plantar fibromatosis (Ledderhose's disease)
Penile fibromatosis (Peyronie's)
Clinical Images

Images hosted on PathOut servers:

Plantar fibromatosis (Ledderhose's disease)

CT, heel, courtesy of Mark R. Wick, M.D.



Images hosted on other servers:

Palmar fibromatosis (Dupuytren's contracture)

Various images



Plantar fibromatosis (Ledderhose's disease)

Various images

Gross Description
Palmar fibromatosis (Dupuytren's contracture)
  • Small nodules or nodular masses associated with aponeurosis and subcutaneous fat, with gray-yellow-white cut surface (color depends on collagen content)

Plantar fibromatosis (Ledderhose's disease)
  • 2 - 3 cm nodules associated with aponeurosis and subcutis, with gray-yellow-white cut surface (color depends on collagen content)

Micro Description
Palmar fibromatosis (Dupuytren's contracture)
  • Proliferative phase:
    • Uniform, plump, immature spindle cells (myofibroblasts and fibroblasts) with bland nuclei and indistinct nucleoli
    • Moderate collagen and elongated vessels
  • Older lesions:
    • More dense collagen, less cellularity
    • Variable mitotic figures
    • Occasional attachment to dermis or cartilaginous metaplasia
    • Usually no infiltration of surrounding tissue beyond subcutis

Plantar fibromatosis (Ledderhose's disease)
  • Proliferative phase:
    • Hypercellular collection of uniform, plump, immature spindle cells with bland nuclei and indistinct nucleoli
    • Moderate collagen and elongated vessels
    • Variable multinucleated giant cells (Am J Surg Pathol 2002;26:244)
  • Older lesions:
    • Denser collagen, less cellularity
    • Often prominent chronic inflammation, variable mitotic figures and hemosiderin

Penile fibromatosis (Peyronie's)
  • Disorganization of collagen of tunica albuginea with formation of nodules, often hyalinizing fibrosis, perivascular lymphocytic infiltrate in 1/3, linear band of calcification in 1/4 (J Urol 1997;157:282)
Micro Images

Images hosted on PathOut servers:

Palmar fibromatosis (Dupuytren's contracture)

Nodule of variably cellular fibroblastic tissue infiltrates an aponeurosis

Mitotic figures may be present in cellular regions



Plantar fibromatosis (Ledderhose's disease)

Courtesy of Mark R. Wick, M.D.



Images hosted on other servers:

Palmar fibromatosis (Dupuytren's contracture)

Immuno-
histochemical
features of
palmar fibromatosis

Fascia with infiltrates of spindle cells

Fascial tissue with infiltrates of fibroblasts and myofibroblasts

Positive Stains
Palmar fibromatosis (Dupuytren's contracture)
Plantar fibromatosis (Ledderhose's disease)
Negative Stains
Palmar fibromatosis (Dupuytren's contracture)
Electron Microscopy Description
Palmar fibromatosis (Dupuytren's contracture)
  • Fibroblasts and myofibroblasts

Plantar fibromatosis (Ledderhose's disease)
  • Fibroblasts and myofibroblasts

Penile fibromatosis (Peyronie's)
  • Penile plaques are composed of collagen fibrils, amorphous particulate material and fibroblasts (Int J Urol 1997;4:274)
Molecular / Cytogenetics Description
Palmar fibromatosis (Dupuytren's contracture)
Plantar fibromatosis (Ledderhose's disease)
Differential Diagnosis
Palmar fibromatosis (Dupuytren's contracture)
  • Fibrosarcoma: single large mass of deep soft tissue with intersecting bundles of cells whose nuclei have abnormal chromatin
  • Epithelioid sarcoma: common in hands, but some cells have distinctive epithelioid appearance with abundant bright eosinophilic cytoplasm, also necrosis, keratin+, CD34+
  • Desmoid tumors: rare in hand, dominant mass infiltrates skeletal muscle

Plantar fibromatosis (Ledderhose's disease)
  • Calcifying aponeurotic fibroma: plump or epithelioid fibroblasts palisading around cartilage and spotty calcification
  • Desmoid fibromatosis: rare in feet, infiltrates skeletal muscle, > 3 cm, often beta-catenin+
  • Fibrosarcoma: single large mass of deep soft tissue with intersecting bundles of cells with abnormal chromatin, herringbone pattern
  • Monophasic synovial sarcoma: uniformly hypercellular, often staghorn vascular pattern or ropy collagen

Penile fibromatosis (Peyronie's)
Additional References
Palmar fibromatosis (Dupuytren's contracture)
Penile fibromatosis (Peyronie's)