Updated: 5/17/2019

Plantar Fibromatosis (Ledderhose Disease)

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Introduction
  • Overview
    • Plantar fibromatosis is a benign tumor of the foot plantar fascia that is characterized by myofibroblast and collagen proliferation
      • also known as Ledderhose disease
      • similar process to Dupuytren's palmar fibromatosis 
  • Epidemiology
    • incidence
      • most common soft tissue neoplasm in the foot
    • demographics
      • more common in males
      • usually seen in middle-aged or elderly individuals
        • can affect individuals of all ages
    • location
      • bilateral in 25-50%
    • risk factors
      • male gender
      • Caucasian
      • epilepsy
      • repeated trauma
      • diabetes
      • long-term alcohol use
      • chronic liver disease
      • other fibrosing conditions (see below)
  • Pathophysiology
    • occurs in 3 phases
      • proliferative phase
        • characterized by increased fibroblast activity and cell proliferation
      • involutional/active phase
        • characterized by nodule formation
      • residual/resting phase
        • characterized by decreased fibroblast activity, collagen maturation, and scar/contracture formation
  • Genetics
    • unlike Dupuytren's disease, the genetic basis of plantar fibromatosis is unclear
  • Associated conditions
    • Dupuytren's disease
      • occurs in 25% with Dupuytren's
    • Peyronie's disase
  • Prognosis
    • malignant transformation is rare
    • recurrence is common after local excision
      • usually recurs as a more aggressive lesion
Anatomy
  • Plantar fascia
    • comprised of 3 bands: central, medial, lateral
      • medial band most commonly affected
    • originates from the medial and anterior aspects of the calcaneus
    • divides into 5 digital slips at the MTP joints
    • inserts on the base of the proximal phalanges
Presentation
  • Symptoms
    • usually asymptomatic
      • may become painful
  • Physical exam
    • inspection
      • subcutaneous thickening or nodules
        • usually on medial aspect of plantar foot
      • digital contractures
        • rare
      • evaluate for the presence of other fibrosing conditions (Dupuytren's, Peyronie's)
    • motion
      • document ankle and hindfoot motion
      • evaluate for the presense of Achilles tendon or gastrocnemius contractures
Imaging
  • Radiographs
    • recommended views
      • AP
      • lateral
    • findings
      • usually normal
  • MRI
    • imaging study of choice
    • findings
      • nodular thickenings on the medial aspect of the plantar aponeurosis
        • low signal intensity on T1-weighted images
          • due to the relative acellularity and high collagen content of lesions
        • low or medium signal intensity on T2-weighted images
          • can have areas of high signal intensity if aggressive type
  • Ultrasound
    • findings
      • multiple lesions embedded on the plantar fascia with sharp juxtaposition between the less reflective fibroma and the much brighter plantar fascia surrounding it
      • comb sign
        • alternating linear bands of hypoechogenicity and isoechogenicity relative to the plantar fascia
        • represents the hyperechoic, fibrous regions of the fibroma on a background of hypoechogenic cellular matrix
Studies
  • Histology
    • gross anatomy
      • lobulated firm irregular mass
    • histology
      • dense fibrocellular tissue with mature collagen and fibrocytes in various stages of maturation
      • no atypical features or abnormal mitotic activity
Differential
  • Post-traumatic neuroma
  • Fibrosarcoma
  • Clear cell sarcoma
Treatment
  • Nonoperative
    • observation and supportive therapy
      • indications
        • first line of treatment
        • lesions that are small or minimally painful
      • modalities
        • NSAIDs
        • orthotics
        • physical therapy
        • corticosteroid injections
  • Operative
    • excision +/- radiation
      • indications
        • lesions that are large, painful, or activity-limiting
Techniques
  • Excision +/- Radiation
    • technique
      • total fasciectomy is preferred over local or wide excision due to risk of recurrence
        • 57-100% recurrence with local excision
        • 8-80% recurence with wide excision (2-3cm margins)
        • 0-50% recurrence with total fasciectomy
      • avoid incisions directly over nodules
        • may lead to local recurrence due to intimate association of the nodule and skin
      • if performing a partial fasciectomy, it is important to resect the nodule and the overlying skin
        • may require skin grafting
    • complications specific to this treatment
      • skin necrosis
      • nerve entrapment
      • loss of arch height
    • outcomes
      • high rate of local recurrence with high-grade lesion
        • consider postoperative radiation to minimize recurrence
Complications
  • Recurrence
    • incidence
      • occurs in 60% of excised lesions
        • usually recurs as a more aggressive lesion
    • risk factors
      • bilateral disease
      • multiple nodules
      • family history of plantar fibromatosis
    • prevention
      • can perform adjuvant radiation
 

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