Neuroma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.B.B.S.[2] Maria Fernanda Villarreal, M.D. [3]

Synonyms and Keywords: Traumatic neuroma; Morton neuroma; Joplin neuroma; Amputation neuroma; Pseudoneuroma; Morton’s metatarsalgia; Plantar interdigital neuroma

Overview

Neuroma (Neuro- is from the Greek for nerve) is defined as a benign tumor of a nerve. However, neuroma commonly refers to any tumor of cells of the nervous system. Neuromas form part of the peripheral nerve sheath tumors. Neuroma was first described by Thomas Morton in 1876. Neuromas may be classified according to histopathological features into 3 groups: Morton's neuroma, traumatic neuroma, and neoplasic neuromas. The pathogenesis of neuroma is characterized by neural degeneration with epineural and endoneural vascular hyalinization, and perineural fibrosis. Neuroma is more commonly observed among patients aged between 15 to 50 years old. Neuroma is more commonly observed among middle aged adults. Females are more commonly affected with neuroma than males. The female to male ratio is approximately 5:1. Common risk factors in the development of neuroma include improper footwear and high impact sports (e.g., rock-climbing, ballet dancing). A major complication of neuroma is chronic neuropathic pain. On ultrasound, neuroma is characterized as a well-defined, hypoechoic lesion located in the intermetatarsal space proximal to the metatarsal head. Patients with neuroma usually appear with antalgic posture. Physical examination may be remarkable for tenderness to palpation and dysesthetic pain. Surgical excision is the treatment of choice for patients with neuroma; this surgical intervention is associated with a relatively favorable success rate, approximately 80%. The recurrence rate after surgery is as high as 50%.

Historical Perspective

  • In 1876, Neuroma was first described by Thomas Morton

Classification

  • Neuroma may be classified according to histopathological features into following groups:[1][2][3]
    • Morton neuroma[4][5][6]
      • Symptomatic perineural fibrosis around a plantar digital nerve of the foot due to chronic nerve compression
      • Also known as:
        • Morton’s metatarsalgia
        • Plantar interdigital neuroma
    • Traumatic neuroma[7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]
      • Arises from nerve injury (often as a result of surgery)
      • They occur at the end of injured nerve fibers as a form of unregulated nerve regeneration
      • Subtype of traumatic neuroma, called "Joplin neuroma" (a compression traumatic neuroma)
      • Occurs most commonly near a scar
      • Often very painful
      • Also known as:
        • Amputation neuroma
        • Pseudoneuroma
    • Neoplasic neuroma
      • Solid nodular mass
      • Usually, separate from nerve fibers
    • Acoustic neuroma - this is misnomer; it is actually a Schwannoma
    • Palisaded encapsulated neuroma

Pathophysiology

  • The pathogenesis of neuroma is characterized by neural degeneration with epineural and endoneural vascular hyalinization, and perineural fibrosis[1]
  • The pathogenesis of traumatic neuroma is characterized by:[24][14][25]
    • Tangle of neural fibers and connective tissue that develops following a peripheral nerve injury
    • Interruption in continuity of nerve causing wallerian degeneration (loss of axons in proximal stump and retraction of axons in distal segment), followed by exuberant regeneration of nerve and formation of mass of Schwann cells, axons and fibrous cells
    • Chronic reactive fibroinflammatory disorganized regeneration around a nerve after an injury (such as traction injury or chronic repetitive stress)
  • Morton neuroma is characterized by being located in the 3rd web-space, between 3rd and 4th metatarsal heads[26]
  • Another subtype of traumatic neuroma is terminal neuroma (also known as "stump neuroma") which can occur after transection of the nerve (e.g. limb amputation)
  • There are no genetic mutations associated with the development of neuroma
  • On gross pathology, characteristic findings of neuroma, include:[27][28]
  • On microscopic histopathological analysis, characteristic findings of neuroma, include:[27][28]
    • Extensive fibrosis around and within the nerve
    • Digital artery
    • Thrombosis
    • Arterial thickening

Histopathology of traumatic neuroma

  • Numerous well formed small nerve twigs
  • Limited soft tissue infiltration
  • Contains axons in haphazardly arranged nerves within mature collagenous scar with entrapped smooth muscle
Traumatic neuroma Source: Libre Pathology

Common sites of involvement by traumatic neuroma

  • Most common oral locations are:
    • Tongue
    • Near mental foramen of mouth
  • Rarely involves:
    • Head
    • Neck

Causes

  • Common causes of neuroma include:[27][28]
  • Indirect nerve trauma
  • Traction injury
  • Chronic repetitive stress

Differentiating Neuroma from other Diseases

  • Neuroma must be differentiated from other diseases that cause forefoot pain and numbness such as:[1][28]

Epidemiology and Demographics

  • Neuroma is a uncommon disease[28]

Age

  • Neuroma is more commonly observed among patients aged between 15 to 50 years old[28]
  • Neuroma is more commonly observed among middle aged adults

Gender

  • Females are more commonly affected with neuroma than males
  • The female to male ratio is approximately 5:1[28]

Race

  • There is no racial predilection for neuroma

Risk Factors

  • Common risk factors in the development of neuroma include:[27]
  • Improper footwear
  • High-impact sports (e.g., rock-climbing, ballet dancing)

Natural History, Complications and Prognosis

  • The majority of patients with neuroma are symptomatic at the time of diagnosis[1]
  • Early clinical features include neuropathic pain or local tenderness
  • If left untreated, the majority of patients with neuroma may progress to develop difficulty walking and limping
  • A significant complication of neuroma is chronic neuropathic pain
  • Prognosis is generally good, and the survival rate of patients with neuroma is 99%[29][30]

Diagnosis

Symptoms

Physical Examination

  • Patients with neuroma usually appear with antalgic posture[1]
  • Physical examination may be remarkable for:

Laboratory Findings

  • There are no specific laboratory findings associated with neuroma[27]

Imaging Findings

MRI

  • On MRI, characteristic findings of neuroma include:[31][32][33][34][35][36][37][38][6][39]
    • Dumbbell/ovoid-shaped lesion at a similar position to that described on ultrasound
    • T1: typically low-to-iso signal
    • T2: typically low signal but can sometimes be intermediate in signal
    • T1 C+ (Gd): tends to show intense enhancement
  • On MRI, characteristic findings of traumatic neuroma include:[1]
    • Fusiform swelling of a nerve or a bulbous mass at a nerve end
    • The parent nerve of some small nerve may difficult or impossible to discern
    • T2/STIR:inhomogeneous hyperintensity (may have a hypointense rim)
    • T1 C+ (Gd): variable contrast enhancement
Morton neuroma MRI Source: Radiopedia

Ultrasound

  • On ultrasound, neuroma is characterized by the following findings:[1][40][41][42][43][44][45][46][47][5][48]
    • Round to ovoid
    • Well-defined, hypoechoic lesion
    • Located in the intermetatarsal space proximal to the metatarsal head
  • On ultrasound, traumatic neuroma is characterized by the following findings:[1]
    • Swollen nerve (mass-like)
    • Hypoechoic
    • Loss of normal fibrillar pattern
    • Usually small, but may be as large as 5 cm

Treatment

Medical Therapy

Surgery

  • Surgery is the mainstay of therapy for neuroma[63][64][65][66][67][68][69][70][71][72][73][74][75][29]
  • Surgical excision is the treatment of choice for patients with neuroma; this surgical intervention is associated with a relatively favorable success rate, approximately 80%
  • The recurrence rate after surgery is as high as 50%

Prevention

  • There are no primary preventive measures available for neuroma[1]
  • Secondary prevention measures include: personal hygiene measures, such as wearing ergonomic shoes

References

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