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Updated: Jun 22 2021

Plantar Fibromatosis (Ledderhose Disease)

  • summary
    • Plantar Fibromatosis, also known as Ledderhose disease, is a benign tumor of the foot plantar fascia that is characterized by myofibroblast and collagen proliferation.
    • The condition usually presents in elderly patients with subcutaneous thickening or nodules in the foot. 
    • Diagnosis is made with a combination of physical examination and MRI studies. 
    • Treatment is observation for minimally symptomatic lesions. Surgical excision is recommended for lesions that are large, painful, or activity-limiting.
  • 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
    • Anatomic 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)
  • Etiology
    • 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 disease
  • 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
  • Prognosis
    • Malignant transformation is rare
    • Recurrence is common after local excision
      • usually recurs as a more aggressive lesion
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