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Soft tissue

Fibroblastic / myofibroblastic

Fibromatosis

Fibromatosis-palmar / plantar

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

Editor-in-Chief: Debra L. Zynger, M.D.

Last author update: 16 March 2020

Last staff update: 14 August 2023 (update in progress)

Copyright: 2002-2023, PathologyOutlines.com, Inc.

PubMed Search: Fibromatosis superficial

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

See Also: Peyronie disease

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Table of Contents

Cite this page: Stewart BD, Nascimento AF. Fibromatosis-palmar / plantar. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuefibromatosissuperficial.html. Accessed October 4th, 2023.

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
- 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)
- Two genetic variants found in genome wide association study suggest possible genetic predisposition
  - One indel (chr5:118704153:D) and one 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 the extracellular matrix
- Proposed roles of fibroblast growth factor (FGF), wingless / integrated (WNT) and transforming growth factor beta (TGF-β) 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
- May be caused by fibrogenic cytokines (J Hand Surg Br 2005;30:557)
- Associated with diabetes, smoking, repetitive vibrational trauma
Plantar fibromatosis
- Not currently understood (Foot Ankle Spec 2018;11:168)
- Associated with long term phenobarbital usage for epilepsy, frozen shoulder, smoking, alcohol addiction, diabetes, repeated trauma (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)
- 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, 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
- 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 six 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 a nodular swelling of the right plantar arch (Diagn Interv Imaging2014;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 greater than 30° (Plast Reconstr Surg 2017;139:240e)
- Collagenase (clostridial collagenase histolyticum) (Plast Reconstr Surg 2017;139:240e, Ir J Med Sci 2019 Nov 11 [Epub ahead of print], 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

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:

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

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 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

Electron microscopy description

Palmar fibromatosis
- Fibroblasts and myofibroblasts
Plantar fibromatosis
- Fibroblasts and myofibroblasts

Electron microscopy images

Images hosted on other servers:

Fibroblast

Molecular / cytogenetics description

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)
Soft tissue, foot nodule, excision:
- Plantar fibromatosis

Differential diagnosis

Palmar fibromatosis
- Spindle cell sarcomas (i.e. synovial sarcoma, malignant peripheral nerve sheath tumor, etc.)
- Epithelioid sarcoma: common in hands but some cells have distinctive epithelioid appearance with abundant bright eosinophilic cytoplasm, also necrosis; keratin+, CD34+
- Desmoid type fibromatosis: rare in hand, dominant mass infiltrates skeletal muscle
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

Additional references

Board review style question #1

Beta catenin will show diffuse nuclear reactivity, confirming the diagnosis
Associated with similar processes in other body sites
Most common in the central and lateral portions of the foot
Most common in adolescents
t(x;18) is pathognomonic

Board review style answer #1

B. This is plantar fibromatosis. It is associated with similar processes in other body sites.

Comment Here

Reference: Palmar / plantar fibromatosis

Board review style question #2

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

Comment Here

Reference: Palmar / plantar fibromatosis

Board review style question #3

CD34
CD56
DOG1
Keratin
Smooth muscle actin

Board review style answer #3

E. Smooth muscle actin

Comment Here

Reference: Palmar / plantar fibromatosis

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