chapter 26

Benign Nerve Sheath Tumors

Schwannoma (Neurilemmoma) and Neurofibroma

CLINICAL SUMMARY
Schwannomas and neurofibromas are slow-growing, benign nerve sheath tumors that arise either eccentri-
cally from peripheral myelin-producing cells or centrally from intraneural support cells, respectively. When
located in deep tissues, they often have a distinctive MRI appearance. Small, asymptomatic lesions can be
observed. Excision of symptomatic tumors is easier for schwannomas, due to their eccentric origin.

DIAGNOSTIC FEATURES

History Broad age range, majority present in the third to fifth decades
Often incidental finding
Once discovered, demonstrate slow growth
Larger lesions may produce mild, intermittent pain, sometimes for years

Location Schwannomas more commonly intermuscular, in the lower extremity

Neurofibromas have a more diffuse distribution

MRI Fusiform shape with rim of surrounding fat

Entering and exiting nerve fiber
Deep lesions are more distinctive

IM AGIN G

Schwannomas (neurilem-
momas) often have a
distinctive appearance on
imaging, as seen on these thigh
(left) and ankle (right) T1 MRI
images.

Imaging characteristics include

intermuscular location, small
amount of surrounding fat,
ovoid shape, and, when in
large nerves, tubular structures
entering and exiting the mass
eccentrically.

153

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T2 MRI of a thigh
schwannoma shows high
signal, reflecting the high water
content of the myxoid tissue
(left).

Benign nerve sheath tumors

enhance to a variable degree
with gadolinium
administration (arrow), as seen
on this axial thigh image
(right).

Neurofibromas, in contrast to
schwannomas, appear to arise
centrally from the afferent and
efferent nerves, as shown in
this subcutaneous lesion (left).
The “target sign” on T2 MRI is
very suggestive of a benign
nerve sheath tumor, as seen on
this thigh neurofibroma (right).
The peripheral high signal
represents the myxoid
portions, and the lower central
signal represents the inner,
collagenized tissue.

A large neurofibroma arising

from a spinal nerve root and
looking like a “dumbbell” is
seen on this coronal T2 MRI
(left). These can be associated
with bone deformity (not seen).

A dramatic “plexiform”
neurofibroma is seen on this
coronal T2 MRI, involving both
the left pelvis and thigh (right).
The finding of a plexiform
neurofibroma is diagnostic of
type 1 neurofibromatosis.

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 Benign Nerve Sheath Tumors 155

HISTOLOGY

While schwannomas and

neurofibromas are both
benign nerve sheath tumors,
schwannomas arise between
the nerve and the sheath
(left), while neurofibromas
arise directly from the nerve
(right).

Schwannomas arise
eccentrically, relative to the
nerve (left).
In contrast, a neurofibroma
cannot easily be separated
from the afferent nerve
(right). The arrow pairs
demonstrate the
relationship between the
tumor and the nerve in each
case.

Schwannomas demonstrate
two types of histologic
features. On the left is a
region of palisading spindle
cells (Antoni A).
In contrast, neurofibromas
demonstrate a more
disorganized pattern (right).

Schwannomas (left) also

have regions with a myxoid
background (Antoni B).
Neurofibroma, on higher-
power view, shows bundles
of pink matrix with
abundant myxoid
extracellular matrix (right).
Nerve sheath tumors stain
positively for S100 (not
shown).

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156 VISUAL GUIDE TO MUSCULOSKELETAL TUMORS: A CLINICAL - RADIOLOGIC - HISTOLOGIC APPROACH

TREATMENT
Small, asymptomatic lesions can be observed.
Symptomatic schwannomas (neurilemmomas) can be excised; neurofibromas typically cannot easily
be separated from the affected nerve.

More Information

Type 1 neurofibromatosis (NF-1) presents with numerous cutaneous and deep neurofibromas,
café-au-lait spots, freckling, and cognitive disabilities as well as other signs.
NF-1 is the most common single-gene disorder of the nervous system. The defect is on chromosome
17q11.2 in a gene encoding for neurofibromin, a tumor suppressor protein, and inheritance is autosomal
dominant, although 50% of patients have new mutations.
Patients are at risk for transformation to malignant nerve sheath tumors, with a lifetime risk of
8% to 13%.

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