Neurilemmomas (neurilemomas) are benign, encapsulated tumors of the

nerve sheath. Their cells of origin are thought to be Schwann cells derived
from the neural crest (see the image below); accordingly, they are often
referred to as schwannomas. These masses usually arise from the side of
a nerve, are well encapsulated, and have a unique histologic pattern.

The cell of origin for a

neurilemmoma is the Schwann cell, which is derived from the neural crest.
These cells line the peripheral nerve processes.
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This benign lesion essentially manifests itself with cosmetic deformity, a
palpable mass, symptoms similar to a compressive neuropathy, or some
combination of these. Neurologic symptoms tend to present late.
Symptoms can be vague, and there is an average interval of up to 5 years
before the diagnosis is established.
Etiology
The cause of these neoplasms is unknown. [1] Neurilemmoma can be
associated with von Recklinghausen disease; when this is the case,
multiple tumors often are present.
Epidemiology
Neurilemmoma is the most common neurogenic tumor, but precise
prevalence figures are not available. They affect persons aged 20-50
years. No racial or sex predilection is recognized. Common locations for
the tumors are, in order of decreasing frequency, the head and flexor
surfaces of the upper and lower extremities and the trunk.
Prognosis
Recurrence is unlikely after complete resection. Patients usually have rapid
and complete relief of pain, with excellent long-term results. [2]
Rare descriptions exist of malignant change in long-standing
neurilemmomas, usually in patients with an underlying diagnosis of
neurofibromatosis. Malignant change is extremely rare in isolated lesions.
 Kano et al evaluated tumor control and hearing preservation relating to
tumor volume, imaging characteristics, and nerve and cochlear radiation
dose after stereotactic radiosurgery with a Gamma Knife (Elekta,
Stockholm, Sweden) in patients with acoustic neuroma. [3]
At a median of 20 months after surgery, none of the patients required
further treatment. [3] Serviceable hearing was preserved in 71% of all
patients and in 89% of patients with Gardner-Robertson (GR) class I
hearing. Patients who received a radiation dose lower than 4.2 Gy to the
central cochlea had significantly better hearing preservation of the same
GR class, and all 12 patients younger than 60 years who received a
cochlear radiation dose lower than 4.2 Gy retained serviceable hearing at 2
years after surgery

1.
2.

History and Physical Examination

Because neurilemmomas (neurilemomas; also frequently referred to as
schwannomas) can present in many locations, the clinical presentation can
be varied. [4] Some may involve the spinal nerve roots and present with
symptoms that mimic those of herniated disk disease of the spine. [5, 6]
Tenderness to palpation is often present; secondary neurologic symptoms
may occur if the tumor is large. When involving the C7 nerve root,
neurilemmoma has been described as a cause of thoracic outlet syndrome.
Lesions in the sciatic nerve can mimic diskogenic low-back pain.
In the extremities, neurilemmomas can present either as an asymptomatic
mass (usually mobile in the transverse plane and tethered along the axis of
the nerve from which it arises) or as mild, localized pain and paresthesia
resulting from pressure on the nerve of origin. Masses are slow-growing
and can exist for months to years without producing symptoms. The
average time from onset of symptoms to diagnosis is 5.5 years.
Lesions in proximal nerves may cause distal symptoms. If these masses
occur in well-defined compartments (eg, wrist or ankle), they can present
as either carpal tunnel or tarsal tunnel syndrome.

Diagnostic Considerations
Differential diagnoses include the following:
3. Fibroma
4. Neurofibroma
5. Neurosarcoma
6. Ganglion cyst
7. Giant cell tumor of tendon sheath
8. Lipoma
Medical Therapy
Neurilemmomas (neurilemomas; also frequently referred to as
schwannomas) can cause a functional deficit because of local pressure on
the nerve of origin. Malignant degeneration, which is extremely rare, was
described by Yousem et al in 1985. [10] Primary malignant tumors of this cell
type do exist, but they are histologically distinct from neurilemmomas. In an
unusual case in which resection would lead to a significant functional
deficit, these benign lesions can be merely observed.
In a study assessing trends in management of unilateral vestibular
schwannoma at Johns Hopkins University from 1997 through 2007, Tan et
al noted an increase in cases observed with follow-up scanning (from
10.5% to 28.0%) and recommended for radiation (from 0% to 4.0%), along
with a decrease in surgical cases (from 89.5% to 68.0%). [11] The increased
frequency of observation was significant even after age, hearing status,
and tumor size were controlled for. Tan et al concluded that this trend
implied changes in both provider philosophy and patient expectations

Surgical Therapy
Like most benign tumors, neurilemmomas respond well to local resection.
On inspection, the nerve is usually splayed out over the lesion. The lesion
is excised marginally, and the nerve fibers are spared. Interlesional
resection is warranted when complete resection would result in permanent
neurologic deficit. Local control is usually excellent.
Effective use of sterotactic radiosurgery (SRS)

Complications
The most common complication of surgical treatment of neurilemmoma is
initial neurapraxia; however, this neurologic deficit can be permanent,
depending on the resection of neural tissue. Generally, patients tolerate
resection well, with complete and rapid relief of symptoms.
 Imaging Studies
Findings on plain radiography generally are not specific. The rare
intraosseous lesion presents as a benign-appearing, well-circumscribed
lesion. Differential diagnoses for these lesions include giant cell tumors,
chordomas (when involving the spine), and chondroblastomas. Massive
bony destruction may be present, especially when the lesion involves the
sacrum.
Special studies to consider include computed tomography (CT) and
magnetic resonance imaging (MRI). [7] MRI is particularly useful (see the
image below); it shows a usually round or oval mass with a moderately
bright signal on T1-weighted images and a bright, heterogeneous signal on
T2-weighted images. [8] The mass is usually less than 2.5 cm in size. The
lesion enhances uniformly with gadolinium contrast.

Wu et al conducted a study to determine the value of ultrasonography (US)

in the diagnosis of neurilemmoma (neurilemoma; also often referred to as
schwannoma) and to determine whether the presence of a round or
roundlike shape on the ultrasound image is diagnostically useful. [9] They
concluded that US is capable of diagnosing neurilemmomas with a high
degree of accuracy but that round or roundlike shape and the ratio of
longitudinal maximal diameter to short maximal diameter (L/S) are of little
value in identifying neurilemmomas of the limbs.
Biopsy
Biopsy may be needed to clearly define the tissue type. For bone lesions
and for particularly large soft-tissue lesions, biopsy is prudent to ensure
correct diagnosis and management. Whenever a biopsy is considered,
strict biopsy guideline principles must be followed.
Histologic Findings
Lesions of the spinal cord often have a dumbbell shape; otherwise, they
are fusiform in shape. They have an epineurium encapsulation, frequently
with overlying vessels. The cut surface is pink or white. In very large
masses, degenerative cysts, hemorrhage, or dystrophic calcification may
be present.
Neurilemmomas have a well-defined, fibrous capsule. Histologically, there
are two distinct regions, as follows:
9. Antoni A areas - These are cellular regions with predominantly benign
spindle cells in many intersecting bundles; they may palisade around
eosinophilic regions that are called Verocay bodies, and they are
positive for S100 staining
10. Antoni B areas - These are much less cellular and have a background
of loose connective tissue that is myxomatous in appearance
 Occasionally, a more aggressive histologic appearance may predominate,
but such forms usually lack mitotic figures.
Staging
Neurilemmomas are commonly classified according to the Enneking
system for benign lesions, as follows:
1. Grade 1 - Inactive lesions
2. Grade 2 - Lesions that deform the surrounding tissues but are not
destructive or locally aggressive
3. Grade 3 - Lesions that are locally aggressive and may invade local
tissues but do not have metastatic potential