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Updated: Mar 12 2024

Neurilemmoma

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https://upload.orthobullets.com/topic/8053/images/string sign.jpg
  •  summary
    • Neurilemmoma, also known as Schwannoma, are benign, encapsulated nerve sheath tumors composed of Schwann cells that occur on the surface of peripheral nerves.
    • The condition usually presents in patients in the 3rd to 6th decades of life with paresthesias in the distribution of the peripheral nerve.
    • Diagnosis is made with biopsy showing a lesion that is well encapsulated in a nerve sheath with histology showing verocay bodies. Immunostaining is positive for S100.
    • Treatment is usually observation for asymptomatic lesions. Marginal resection is recommended in cases of progressive symptoms.
  • Epidemiology
    • Demographics
      • peak incidence is in the 3rd to 6th decades
        • previous test question ages: 40, 45
      • affect males and females equally
    • Anatomic location
      • tumor is well encapsulated on the surface of a peripheral nerve
      • often occurs on flexor surfaces of extremities and head and neck
      • larger lesions may occur in the pelvis
  • Etiology
    • Pathoanatomy
      • can affect motor or sensory nerves
    • Genetics
      • often associated with mutations affecting NF2 gene
    • Malignant transformation
      • extremely rare
  • Presentation
    • Symptoms
      • usually asymptomatic
      • may have paresthesia in the distribution of the peripheral nerve
    • Physical exam
      • may have positive Tinel’s sign in the distribution of the nerve affected may be present
  • Imaging
      • sequences
        • low intensity (dark) T1
        • high intensity (bright) on T2
        • diffuse enhancement with gadolinium
      • findings
        • may show “string sign”
        • difficult to differentiate from neurofibroma
  • Histology
    • Gross pathology
      • the lesion is well encapsulated in a nerve sheath
      • gray in color
    • Histology
      • Antoni A structure
        • a pattern of spindle cells arranged in intersecting bundles (spindle cell battle formation)
      • Antoni B
        • areas with less cellularity with loosely arranged cells
      • Verocay bodies
        • pathognomonic
        • composed of two rows of aligned nuclei in a palisading formation
    • Immunochemistry
      • strongly uniform S100 antibody staining
  • Treatment
    • Nonoperative
      • observation
        • indications
          • asymptomatic lesions
    • Operative
      • marginal excision
        • indications
          • symptoms or interfering with quality of life
        • technique
          • nerve function may be preserved by careful dissection, excising the lesion parallel to the nerve fascicles so the lesion may be extruded.
        • complications
          • small risk of sensory deficits
        • outcomes
          • recurrence is rare
Card
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What are the pathognomonic histological structures?
    • Histology
      • Verocay bodies
        • pathognomonic
        • composed of two rows of aligned nuclei in a palisading formation

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Question
1 of 9
In scope icon L 2
QID 219342 (Type "219342" in App Search)
A 51-year-old male presents to the clinic after his masseuse pointed out a palpable mass along his right volar forearm. He notes that it is not particularly painful when palpated, but he has had progressively worsening paresthesias about the thumb, index, and middle fingers over the last year. On exam, there is a positive Tinel's sign along the same distribution. A representative slice from the patient's MRI is shown in Figure A, and he undergoes operative treatment with findings from the intra-operative frozen section shown in Figure B. Which of the following represents the best subsequent approach to his definitive treatment?
  • A
  • B

Chemotherapy alone given degree of nerve involvement

1%

4/691

Wide resection with reconstruction of the affected nerve

13%

93/691

Marginal resection with excision parallel to the nerve fascicles

78%

536/691

Marginal resection with adjuvant radiation

6%

42/691

Marginal resection with adjuvant chemotherapy

2%

11/691

  • A
  • B

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