Soft tissue

Fibroblastic / myofibroblastic

Fibromatosis

Fibromatosis-palmar / plantar



Last author update: 28 November 2023
Last staff update: 28 November 2023

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PubMed Search: Palmar fibromatosis / plantar fibromatosis

See Review Article: J Pathol Transl Med 2021;55:265

See Also: Peyronie disease

Brian D. Stewart, M.D.
Alessandra F. Nascimento, M.D.
Page views in 2023: 27,229
Page views in 2024 to date: 8,718
Cite this page: Stewart BD, Nascimento AF. Fibromatosis-palmar / plantar. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuefibromatosissuperficial.html. Accessed April 14th, 2024.
Definition / general
  • Palmar and plantar fibromatosis are benign nodular fibroblastic / myofibroblastic proliferations typically arising in the volar aspect of the hands and fingers or involving plantar aponeuroses, respectively
  • Both processes are histologically similar, composed of a bland cellular proliferation of spindle cells with a bluish appearance and with a variable amount of background collagen, depending on the age of the lesion (J Pathol Transl Med 2021;55:265)
Essential features
  • Bland, variably cellular proliferation of spindled fibroblasts / myofibroblasts
  • Collagenous stroma
  • Involvement of aponeurosis and variably subcutis and dermis
  • A subset shows nuclear beta catenin expression, despite the absence of CTNNB1 or APC gene mutations (Histopathology 2007;51:509, Mod Pathol 2001;14:695)
Terminology
  • Palmar fibromatosis: Dupuytren disease or contracture
  • Plantar fibromatosis: Ledderhose disease, morbus Ledderhose
ICD coding
  • ICD-10
    • M72.0 - palmar fascial fibromatosis (Dupuytren)
    • M72.2 - plantar fascial fibromatosis
  • ICD-11
    • FB51.0 - palmar fascial fibromatosis
    • FB51.1 - knuckle pads
    • FB51.Y - other specified fibroblastic disorders (for plantar)
    • 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
  • Palmar fibromatosis
    • Unknown (WHO 5th edition)
    • May be caused by fibrogenic cytokines (J Hand Surg Br 2005;30:557)
    • Associations with diabetes, smoking and repetitive vibrational trauma reported but not validated (WHO 5th edition)
  • Plantar fibromatosis
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 of heel

CT of heel



Images hosted on other servers:

Palmar fibromatosis
Ultrasound

Ultrasound

MRI

MRI

Palmar and plantar fibromatosis
Axial T1 weighted MRI

Axial T1 weighted MRI

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

Postoperative improvement

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: Weiss: Enzinger and Weiss's Soft Tissue Tumors, 7th Edition, 2019
Gross images

Contributed by Brian D. Stewart, M.D.

Palmar fibromatosis

Excised contraction band

Frozen section description
Frozen section images

Contributed by Brian D. Stewart, M.D.

Plantar fibromatosis

Spindle cell proliferation

Microscopic (histologic) description
  • Both processes are histologically similar
  • Typically, tumors involve a thickened palmar / plantar aponeurosis and form single or multiple discontinuous, moderately cellular spindle cell nodules in a collagenous stroma (Am J Surg Pathol 2005;29:1095)
  • Morphologically, there are 3 phases of growth (proliferative, involutional and late stage)
    • Proliferative phase
      • Cellular, parallel fascicles of bland, plump, relatively uniform spindled fibroblasts with tapering nuclei, vesicular chromatic and small or inconspicuous nucleoli, with minimal stromal collagen
      • Some plantar lesions are hypercellular and can mimic spindle cell sarcomas but they lack atypia
      • Scattered chronic inflammation and occasional typical mitotic figures, especially in early lesions
      • Mitotic activity may be prominent in pediatric patients
      • Occasional, particular plantar lesions show interspersed multinucleated cells (Am J Surg Pathol 2002;26:244)
    • Late stage
      • Increase collagenous matrix and decreased cellularity
  • Reference: WHO 5th edition
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 6 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, rare or absent mitotic activity
Cytology images

Contributed by Brian D. Stewart, M.D.

Plantar fibromatosis

Touch prep

Positive stains
Negative stains
Electron microscopy description
  • 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) (see comment)
    • Comment: Sections show a variably cellular proliferation of spindled fibroblasts and myofibroblasts without atypia set within a collagenous stroma, morphologically consistent with palmar fibromatosis (Dupuytren contracture).

  • Soft tissue, foot nodule, excision:
    • Plantar fibromatosis (see comment)
    • Comment: Sections show a variably cellular proliferation of spindled fibroblasts and myofibroblasts without atypia set within a collagenous stroma, morphologically consistent with 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. Associated with similar processes in other body sites
  2. Beta catenin will show diffuse nuclear reactivity, confirming the diagnosis
  3. Most common in adolescents
  4. Most common in the central and lateral portions of the foot
  5. t(x;18) is pathognomonic
Board review style answer #1
A. Associated with similar processes in other body sites. Plantar fibromatosis is associated with similar processes in other body sites. Answer B is incorrect because beta catenin usually shows nuclear reactivity in palmar fibromatosis but not in plantar fibromatosis. Answers C and D are incorrect because plantar fibromatosis is most common in the medial and central bands of the plantar aponeurosis and in middle aged patients. Answer E is incorrect because synovial sarcoma has a pathognomonic t(x;18).

Comment Here

Reference: Palmar / plantar fibromatosis
Board review style question #2
Which statement about plantar fibromatosis is correct?

  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 style answer #2
A. Associated with diabetes. Plantar fibromatosis has been shown to have an association with diabetes. Answer B is incorrect because plantar fibromatosis is bilateral in about 25% of cases. Answer C is incorrect because recurrence is common after excision. Answer D is incorrect as plantar fasciitis is inflammation of the ligament itself and does not form nodules; it is part of the differential diagnosis of plantar fibromatosis. Answer E is incorrect because plantar fibromatosis is usually slow growing.

Comment Here

Reference: Palmar / plantar fibromatosis
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 style answer #3
E. Smooth muscle actin is the correct answer as it is reactive in many spindle cell proliferations and is not specific. Answers A - D are incorrect as all of these markers are negative in palmar and plantar fibromatosis.

Comment Here

Reference: Palmar / plantar fibromatosis
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