Soft tissue
Fibroblastic / myofibroblastic tumors
Palmar / plantar fibromatosis


Topic Completed: 16 March 2020

Minor changes: 24 March 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Fibromatosis [title] superficial

Related Topic: Peyronie disease

Brian D. Stewart, M.D.
Alessandra F. Nascimento, M.D.
Page views in 2019: 16,413
Page views in 2020 to date: 5,568
Cite this page: Stewart BD, Nascimento AF. Palmar / plantar fibromatosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuefibromatosissuperficial.html. Accessed July 5th, 2020.
Definition / general
  • Palmar fibromatosis
    • Benign disordered nodular proliferative process of palmar aponeurosis, surrounding adipose and occasionally dermis, due to fibroblasts, myofibroblasts and fibrocytes with subsequent formation of nodules
  • Plantar fibromatosis
    • Benign disordered nodular proliferative process of plantar aponeurosis and surrounding adipose tissue due to fibroblasts, myofibroblasts and fibrocytes with subsequent formation of nodules
Essential features
  • Palmar fibromatosis
    • Most common type of fibromatosis (proliferation of fibroblasts and myofibroblasts)
    • Mostly in white, middle aged and older men occurring at the distal palmar crease leading to nodules and contracture (Dupuytren)
    • Many recur
  • Plantar fibromatosis
    • Proliferation of fibroblasts and myofibroblasts on the plantar aponeurosis of mostly middle aged patients that may lead to painful nodules but usually does not lead to contracture
Terminology
  • Palmar fibromatosis: Dupuytren disease or contracture
  • Plantar fibromatosis: Ledderhose disease, morbus Ledderhose
ICD coding
  • ICD-10: M72.0 - Palmar fascial fibromatosis (Dupuytren)
  • ICD-10: M72.2 - Plantar fascial fibromatosis
  • ICD-11: FB51.0 - Palmar fascial fibromatosis
  • ICD-11: FB51.1 - Knuckle pads
  • ICD-11: FB51.Y - Other specified fibroblastic disorders (for plantar)
  • ICD-11: XH75J5 - Palmar/plantar type fibromatosis
Epidemiology
  • Palmar fibromatosis
    • Most common type of superficial fibromatosis (1 - 2% of population)
      • Prevalence increases with age (~20% of population at age 65)
    • M:F = 3 - 4:1
    • 50% bilateral
    • Most common in northern Europeans
      • Rare in black population
  • Plantar fibromatosis
Sites
  • Palmar fibromatosis
  • Plantar fibromatosis
    • Most often the medial and central bands of plantar aponeurosis
Pathophysiology
Etiology
Clinical features
  • Palmar fibromatosis
    • Often presents with subcutaneous nodules on distal palmar crease
    • Puckers overlying skin as it ages
    • Causes flexion contracture, most commonly of digits 4 and 5, due to cord-like expansion of digital aponeurotic slips
    • Does not involve deep structures (i.e. tendons or skeletal muscle)
    • 10% also have plantar disease, 1 - 4% have penile fibromatosis
  • Plantar fibromatosis
    • 0.5 - 3.0 cm in diameter subcutaneous nodule(s), slow growing and located in the medial or central plantar aponeurosis
    • Initially painless but later associated with pain after standing or walking, typically on the medial aspect of the sole (arch)
    • Associated with concomitant palmar and penile fibromatoses and keloids
    • Usually not associated with contractures (Foot Ankle Int 2018;39:751)
Diagnosis
  • Usually clinical, however, occasionally may be a histologic confirmation
Radiology description
Radiology images

Contributed by Mark R. Wick, M.D.

Plantar fibromatosis

CT, heel



Images hosted on other servers:

Palmar fibromatosis

Ultrasound

MRI

T1 weighted MR

Plantar fibromatosis

Coronal proton density weighted MR

T2 MRI

T1 weighted (500/20)

Prognostic factors
  • Palmar fibromatosis
    • Worse in white men with a strong family history, bilateral involvement, severe disease and ectopic manifestations (BMJ 2006;332:397)
  • Plantar fibromatosis
Case reports
Treatment
Clinical images

Contributed by Brian D. Stewart, M.D.

Palmar fibromatosis

Nodules and contractures

Excision of contraction band

Post-operative improvement



Images hosted on other servers:

Palmar fibromatosis

Subcutaneous cords and nodules

 Plantar fibromatosis

Nodular soft tissue mass along medial plantar surface

Gross description
  • Palmar fibromatosis
    • Small nodules or nodular masses associated with aponeurosis and subcutaneous fat with gray-yellow-white cut surface (color depends on collagen content)
  • Plantar fibromatosis
    • 2 - 3 cm nodules associated with aponeurosis and subcutis with gray-yellow-white cut surface (color depends on collagen content)
  • Reference: Fletcher: WHO Classification of Tumours of Soft Tissue and Bone, 4th Edition, 2013
Gross images

Contributed by Brian D. Stewart, M.D.

Palmar fibromatosis

Excised contraction band

Frozen section description
  • Palmar / plantar fibromatosis
    • Same as microscopic description (below) but immaturity (bluish nature) of the fibroblasts are less evident
Frozen section images

Contributed by Brian D. Stewart, M.D.

Plantar fibromatosis

Spindle cell proliferation

Microscopic (histologic) description
  • Palmar fibromatosis
    • Proliferative phase:
      • Uniform, plump, spindle cells (myofibroblasts and fibroblasts) with bland nuclei and indistinct nucleoli usually with a “bluer” appearance than the surrounding aponeurotic tissue
      • Moderate collagen and elongated vessels
    • Older lesions:
      • Denser collagen, less cellularity
      • Variable mitotic figures
      • Occasional attachment to dermis or cartilaginous metaplasia
      • Usually no infiltration of surrounding tissue beyond subcutis
  • Plantar fibromatosis
    • Proliferative phase:
      • Hypercellular collection of uniform, plump, spindle cells with bland nuclei and indistinct nucleoli usually with a “bluer” appearance than surrounding aponeurotic tissue
      • Moderate collagen and elongated vessels
      • Variable multinucleated giant cells (Am J Surg Pathol 2002;26:244)
    • Active phase:
      • Nodule formation
    • Residual phase (older lesions):
      • Denser collagen, less cellularity
      • Often prominent chronic inflammation, variable mitotic figures and hemosiderin
Microscopic (histologic) images

Contributed by Brian D. Stewart, M.D. and AFIP

Palmar fibromatosis

Fibroblastic proliferation

Fibroblastic nodules

Fibroblastic nodule

Tendinous infiltration

Infiltrates an aponeurosis

Mitotic figures



Plantar fibromatosis

Nodular proliferation

Plantar aponeurotic infiltration

Demarcation from plantar fascia

Virtual slides

Images hosted on other servers:
Missing Image

Male with six month history of palm nodule

Cytology description
  • Usually limited to touch preps during rare frozen sections (Acta Cytol 1993;37:323)
  • Low cellularity, clusters of bland spindle cells with oval to elongated nuclei, no atypical features, no to rare mitotic activity
Cytology images

Contributed by Brian D. Stewart, M.D.

Plantar fibromatosis

Touch prep

Positive stains
Negative stains
Electron microscopy description
  • Palmar fibromatosis
    • Fibroblasts and myofibroblasts
  • Plantar fibromatosis
    • Fibroblasts and myofibroblasts
Electron microscopy images

Images hosted on other servers:
Missing Image

Fibroblast

Molecular / cytogenetics description
Videos

Plantar fibromatosis

Sample pathology report
  • Soft tissue, hand nodule, excision:
    • Palmar fibromatosis (Dupuytren contracture)
  • Soft tissue, foot nodule, excision:
    • Plantar fibromatosis
Differential diagnosis
Board review style question #1

    Which of the following is true about the disease pictured in this photomicrograph of a painful nodule on the sole of the foot?

  1. Beta catenin will show diffuse nuclear reactivity, confirming the diagnosis
  2. Associated with similar processes in other body sites
  3. Most common in the central and lateral portions of the foot
  4. Most common in adolescents
  5. t(x;18) is pathognomonic
Board review answer #1
B. This is plantar fibromatosis. It is associated with similar processes in other body sites.

Reference: Palmar / plantar fibromatosis

Comment Here
Board review style question #2
    Plantar fibromatosis is

  1. Associated with diabetes
  2. Bilateral in 5% of cases
  3. Often cured without recurrence after excision
  4. Synonymous with plantar fasciitis
  5. Usually rapidly growing
Board review answer #2
A. Associated with diabetes

Reference: Palmar / plantar fibromatosis

Comment Here
Board review style question #3
    Which of the following stains may be positive in palmar or plantar fibromatosis although not specific?

  1. CD34
  2. CD56
  3. DOG1
  4. Keratin
  5. Smooth muscle actin
Board review answer #3
E. Smooth muscle actin

Reference: Palmar / plantar fibromatosis

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