Home > Soft tissue > Fibromatosis-palmar / plantar

Soft tissue

Fibroblastic / myofibroblastic

Fibromatosis

Fibromatosis-palmar / plantar

Authors: Brian D. Stewart, M.D., Alessandra F. Nascimento, M.D.

Editorial Board Member: Jose G. Mantilla, M.D.

Deputy Editor-in-Chief: Borislav A. Alexiev, M.D.

Last author update: 28 November 2023

Last staff update: 28 November 2023

Copyright: 2002-2024, PathologyOutlines.com, Inc.

PubMed Search: Palmar fibromatosis / plantar fibromatosis

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

See Also: Peyronie disease

Page views in 2023: 27,229

Page views in 2024 to date: 9,642

Table of Contents

Cite this page: Stewart BD, Nascimento AF. Fibromatosis-palmar / plantar. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuefibromatosissuperficial.html. Accessed April 25th, 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
- Prevalence not currently understood (Foot Ankle Spec 2018;11:168)
- Middle aged patients
  - Several reports of patients < 16 years old to as young as 9 months (J Pediatr Orthop 1997;17:16)
- NIH list of rare diseases (< 200,000 people)
- ~25% bilateral
- M > F (Orthop Res Rev 2018;11:1)
- 2 genetic variants found in genome wide association study suggest a possible genetic predisposition
  - 1 indel (chr5:118704153:D) and 1 SNP (rs62051384) (Int J Sports Med 2018;39:314)

Sites

Palmar fibromatosis
- Metacarpophalangeal joint, the proximal interphalangeal joint or both joints (Hand (N Y) 2011;6:149)
Plantar fibromatosis
- Most often the medial and central bands of plantar aponeurosis

Pathophysiology

Palmar fibromatosis
- Proliferation of fibroblasts, their differentiation into myofibroblasts and the production of extracellular matrix
- Proposed roles of fibroblast growth factor (FGF), wingless / integrated (WNT) and transforming growth factor beta (TGFβ) in disease progression (Exp Cell Res 2010;316:2390, Med Hypotheses 2012;78:385, Cell Physiol Biochem 2012;30:927, N Engl J Med 2011;365:307, Ann Plast Surg 2019;83:594)
Plantar fibromatosis
- Proliferative phase: increased fibroblastic activity and cellular proliferation
- Active phase: nodule formation occurs
- Residual phase: collagen deposition, scar formation and tissue contracture

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
- Unknown (WHO 5th edition) (Foot Ankle Spec 2018;11:168)
- Associations with long term phenobarbital usage for epilepsy, frozen shoulder, smoking, alcohol addiction, diabetes and repeated trauma have not been validated (WHO 5th edition) (Epilepsia 2008;49:1965, Foot Ankle Spec 2018;11:168)

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

Palmar fibromatosis
- Ultrasound
  - Nodules superficial to flexor tendons in superficial fascia
  - Early lesions: hypoechoic with hypervascularity
  - Chronic lesions: hyperechoic without vascularity (J Ultrason 2012;12:381)
- Magnetic resonance imaging (MRI)
  - Subcutaneous nodules
  - Typically uniformly low signal intensity on both T1 and T2 (AJR Am J Roentgenol 1993;160:813)
Plantar fibromatosis
- Radiographs usually normal
- Ultrasound
  - Better than MRI (Semin Musculoskelet Radiol 2010;14:334)
  - Lesion(s) embedded on the plantar fascia
  - Less reflective to the much brighter plantar fascia surrounding it with sharp juxtaposition (Semin Musculoskelet Radiol 2010;14:334)
  - Hypo to mixed echogenicity
  - Discrete, fusiform, multinodular thickening of plantar fascia (AJR Am J Roentgenol 2002;179:1167)
  - Comb sign in 51% of cases (J Ultrasound Med 2018;37:2725)
    - Alternating linear bands of hypoechogenicity and isoechogenicity relative to the plantar fascia
- MRI
  - Nodules appear as focal, oval shaped areas of disorganization embedded in plantar fascia (Semin Musculoskelet Radiol 2010;14:334)
  - T1: iso to low signal (compared to muscle)
  - T2: low to intermediate signal (AJR Am J Roentgenol 2002;179:1167, Radiographics 2000;20:333)

Radiology images

Contributed by Mark R. Wick, M.D.

Plantar fibromatosis

CT of heel

Images hosted on other servers:

Palmar fibromatosis

Ultrasound

MRI

Palmar and plantar fibromatosis

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
- Worse with bilateral involvement, multiple nodules and a positive family history (Foot (Edinb) 2017;31:31)

Case reports

Palmar fibromatosis
- 59 year old woman with bilateral Dupuytren contractures of the thumb interphalangeal joints (J Plast Reconstr Aesthet Surg 2012;65:1738)
- 65 year old man with hand flexion contracture (BMJ 2014;349:g6137)
- 82 year old man with heterotopic ossification in Dupuytren disease (J Bone Joint Surg Br 2011;93:1676)
Plantar fibromatosis
- 28 year old Brazilian woman with 6 year history of painless bilateral plantar nodules (J Clin Aesthet Dermatol 2010;3:45)
- 30 year old man presented with painful bilateral plantar nodules (Surg J (N Y) 2016;2:e102)
- 51 year old woman with giant bilateral plantar fibromatosis (Int J Dermatol 2018;57:365)
- 54 year old man with nodular swelling of the right plantar arch (Diagn Interv Imaging 2014;95:893)
- 64 year old woman with osseous metaplasia in a case of plantar fibromatosis (J Foot Ankle Surg 2004;43:430)

Treatment

Palmar fibromatosis
- Observation, excision or incision of contracture band
- Often recurs
- Surgical treatment is necessary if the contracture results in functional disability and the total flexion deformity is > 30° (Plast Reconstr Surg 2017;139:240e)
- Collagenase (clostridial collagenase histolyticum) (Plast Reconstr Surg 2017;139:240e, Ir J Med Sci 2020;189:529, Foot Ankle Spec 2018;11:168)
Plantar fibromatosis
- Conservative measures should be used prior to recommending surgery (Orthop Res Rev 2018;11:1)
- Observation, surgery if symptomatic; complete fasciectomy has fewer recurrences (0 - 50%) than local excision (57 - 100%) and wide excision (8 - 80%) (Plast Reconstr Surg 2008;122:486, Foot Ankle Spec 2018;11:168, Orthop Res Rev 2018;11:1)
  - Wide excision: 2 - 3 cm margin
- Several nonoperative treatments with variable scientific support (Orthop Res Rev 2018;11:1)
  - Steroid injections
  - Verapamil
  - Radiation therapy
  - Extracorporeal shock wave therapy
  - Tamoxifen
  - Collagenase

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

Same as Microscopic (histologic) description but immaturity of the fibroblasts is less evident (blue staining is less intense)

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:

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

Palmar fibromatosis
- Usually not necessary to perform due to characteristic histology
- Vimentin, variable actin - muscle specific and smooth muscle actin, less frequently desmin
- Aberrant nuclear expression of beta catenin common (Mod Pathol 2001;14:695, Histopathology 2007;51:509)
Plantar fibromatosis
- Vimentin, variable actin - muscle specific and smooth muscle actin, less frequently desmin

Negative stains

Palmar fibromatosis
- Keratin, CD34, EMA, S100
Plantar fibromatosis
- Keratin, CD34, EMA, S100, beta catenin
Reference: Weiss: Enzinger and Weiss's Soft Tissue Tumors, 7th Edition, 2019

Electron microscopy description

Fibroblasts and myofibroblasts

Electron microscopy images

Images hosted on other servers:

Fibroblast

Molecular / cytogenetics description

The tumor lacks the CTNNB1 and APC mutations characteristic of desmoid fibromatosis (Histopathology 2007;51:509, Mod Pathol 2001;14:695)
Palmar fibromatosis
- Near diploid, often +7 or +8, no gene amplifications or deletions (Cancer Genet Cytogenet 2008;183:6)
- Loss of Y chromosome (Mod Pathol 2000;13:1080)
- Usually considered reactive, not neoplastic (J Transl Med 2006;4:21)
- Aberrations in Wnt signaling pathway but no somatic mutations of beta catenin genes, unlike desmoid fibromatosis (N Engl J Med 2011;365:307, Plast Reconstr Surg 2012;129:921, Joint Bone Spine 2012;79:7, Mod Pathol 2001;14:695)
Plantar fibromatosis
- Near diploid, often +7 or +8; no somatic mutations of beta catenin genes, unlike desmoid fibromatosis (Mod Pathol 2001;14:695)
- Trisomy 8 and 14 (Cancer Genet Cytogenet 1999;108:176)
- Reciprocal t(2;7)(p13;p13) (Cancer Genet Cytogenet 2005;158:67)

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

Palmar fibromatosis
- Spindle cell sarcomas (i.e., synovial sarcoma, malignant peripheral nerve sheath tumor, etc.)
- Desmoid type fibromatosis:
  - Rare in hand
  - Dominant mass infiltrates skeletal muscle
- Epithelioid sarcoma:
  - Epithelioid sarcoma commonly presents in the hand but cells will show a distinctive epithelioid appearance with abundant eosinophilic cytoplasm (J Pathol Transl Med 2021;55:265)
  - Central necrosis or hyalinization is often seen
  - Epithelioid sarcoma is characteristically reactive for cytokeratins and CD34 and shows a loss of nuclear staining for SMARCB1 (INI1)
Plantar fibromatosis
- Clinical differential without nodules: calcaneal stress fracture, tarsal tunnel syndrome, plantar fasciitis (Orthop Res Rev 2018;11:1)
- Clinical differential with nodules: melanoma, synovial sarcoma, Kaposi sarcoma
- Calcifying aponeurotic fibroma:
  - Plump or epithelioid fibroblasts palisading around cartilage and spotty calcification
- Desmoid type fibromatosis:
  - Rare in feet
  - Infiltrates skeletal muscle
  - > 3 cm
  - Often beta catenin+
- Spindle cell sarcomas (i.e., synovial sarcoma, malignant peripheral nerve sheath tumor, etc.):
  - Monophasic synovial sarcoma:
    - Uniformly hypercellular
    - Often staghorn vascular pattern or ropy collagen
    - Patchy keratin or EMA
    - Rearrangement of SS18
  - Malignant peripheral nerve sheath tumor:
    - S100 and SOX10 patchy / focal in up to 50%
    - Loss of nuclear H3K27me3 in subset
    - History of NF1 in subset

Additional references

WHO Classification of Tumours Editorial Board: Soft Tissue and Bone Tumours, 5th Edition, 2020, eMedicine: Dupuytren Contracture [Accessed 14 August 2023], Wikipedia: Dupuytren's Contracture [Accessed 14 August 2023], J Am Acad Orthop Surg 2011;19:746, Wikipedia: Plantar Fibromatosis [Accessed 14 August 2023], eMedicine: Plantar Fibromatosis [Accessed 14 August 2023]

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?

Associated with similar processes in other body sites
Beta catenin will show diffuse nuclear reactivity, confirming the diagnosis
Most common in adolescents
Most common in the central and lateral portions of the foot
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).

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Reference: Palmar / plantar fibromatosis

Board review style question #2

Which statement about plantar fibromatosis is correct?

Associated with diabetes
Bilateral in 5% of cases
Often cured without recurrence after excision
Synonymous with plantar fasciitis
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.

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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?

CD34
CD56
DOG1
Keratin
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