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Schwannoma 
Last revised by Maulik S Patel (/users/maulikspatel?lang=us) on 6 Aug 2023

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Schwannomas, less commonly called neurinomas or neurilemmomas, are benign

tumors of Schwann cell origin and are the most common tumor of peripheral nerves,
including cranial nerves.

This article provides a general overview of schwannomas. For a discussion of

schwannomas located at specific sites, please refer to the relevant articles listed below.

On this page:
:
 Article:
Epidemiology
Clinical presentation
Pathology
Radiographic features
Treatment and prognosis
References

Images:
Cases and figures

Epidemiology
The vast majority of schwannomas are sporadic, with a peak presentation in the 5th to
6th decades. There is no sex predilection 9.

When they occur in patients with neurofibromatosis type 2 (NF2)

(/articles/neurofibromatosis-type-2-3?lang=us), schwannomas usually present by the 3rd
decade 4.

Associations
Most schwannomas are solitary (90%) 2,9 and sporadic, however, there is an association
with neurofibromatosis type 2 (NF2) (/articles/neurofibromatosis-type-2-3?lang=us)
(abnormality of chromosome 22). Multiple schwannomas are characteristic of
neurofibromatosis type 2. Approximately 18% of solitary schwannomas occur in
patients with neurofibromatosis type 2 4.

There is also schwannomatosis (/articles/schwannomatosis?lang=us), which consists

of multiple schwannomas without the concomitant involvement of cranial nerve VIII.

Clinical presentation
:
 Presentation depends on the location of the tumor (see below) but generally, symptoms
are due to local mass e"ect or dysfunction of the nerve they arise from.

Pathology

Location
intracranial schwannomas (/articles/intracranial-schwannoma?lang=us)
cranial nerves: although almost any cranial nerve may be involved, except
olfactory nerves and optic nerves which lack sheaths composed of Schwann cells,
by far the most commonly involved nerve is the vestibulocochlear nerve (CN VIII)
(/articles/vestibulocochlear-nerve?lang=us)
non-cranial nerve or intracerebral (very rare)
intraorbital schwannoma: commonly arise from supraorbital and supratrochlear
nerves in the upper anterior orbital cavity 10
spinal schwannoma (/articles/spinal-schwannoma?lang=us)
arising from spinal nerve roots (/articles/spinal-schwannoma?lang=us)
trunk
intercostal nerves (/articles/intercostal-nerve?lang=us): see intercostal nerve
schwannoma (/articles/intercostal-nerve-neurilemmoma?lang=us)
posterior mediastinum
retroperitoneum
gastrointestinal schwannoma (/articles/gastrointestinal-schwannoma?lang=us)
limbs
especially flexor surfaces (specifically ulnar and peroneal nerves)

Macroscopic appearance
Schwannomas are benign encapsulated neoplasms of Schwann cells (WHO grade 1 9).
They arise eccentrically from their parent nerve, with the nerve fibers splayed along
their surface (as distinct to neurofibromas (/articles/neurofibroma?lang=us) which arise
within the nerve).

Microscopic appearance
:
 Conventional schwannomas are composed of spindle cells that demonstrate two
growth patterns: Antoni type A and Antoni type B 7-9.

Antoni type A pattern: elongated cells are thickly bundled and organized in fascicles.
Palisades are occasionally observed; when they are prominent they constitute Verocay
bodies (/articles/verocay-bodies?lang=us). The cells also have fusiform nuclei and
eosinophilic cytoplasm. Antoni type A is a strongly Periodic acid-Schi" (PAS) positive and
immunoperoxidase assay for laminin 10.

Antoni type B pattern cells are less compact and are prone to cystic degeneration, and
haphazardly distributed cells with distinct cytoplasmic margins in a loose myxoid matrix
10
.

Subtypes
Several schwannoma subtypes are recognized 6,8,9:

ancient schwannoma (/articles/ancient-schwannoma?lang=us)

degenerative changes with hyalinization, ischemic change, and atypical appearing
nuclei
cellular schwannoma
predominantly composed of Antoni type A tissue
no Verocay bodies
most commonly found arising from large nerves, brachial and lumbosacral plexus
and thus often in a paravertebral location
epithelioid schwannoma
usually sporadic but may be associated with schwannomatosis
microcystic/reticular schwannoma
rarest subtype
typically arise from viscera, especially the gastrointestinal tract 9
plexiform schwannoma (/articles/missing?article%5Btitle%5D=plexiform-
schwannoma&lang=us)
usually arise from skin or subcutaneous tissues
usually diagnosed at birth or childhood
:
 usually sporadic, but rarely associated with neurofibromatosis type 2
(/articles/neurofibromatosis-type-2-3?lang=us)
should not be confused with plexiform neurofibromas (/articles/plexiform-
neurofibroma?lang=us) (/?lang=us)
MENU SEARCH
associated with neurofibromatosis type 1 (/articles/neurofibromatosis-type-1?
lang=us)
may undergo malignant change
neuroblastoma type 10
schwannoma with neuromelanin accumulation
not a distinct subtype
not to be confused with PRKAR1A-associated malignant melanotic nerve sheath
tumor (/articles/missing?article%5Btitle%5D=PRKAR1A-
associated+malignant+melanotic+nerve+sheath+tumor&lang=us) (a.k.a. melanotic
schwannoma) seen in patients with Carney complex (/articles/carney-complex?
lang=us) 9

Radiographic features
General imaging features of schwannomas include:

well-circumscribed masses which displace adjacent structures without direct invasion

cystic and fatty degeneration is common 4
the larger a schwannoma, the more likely it is to show heterogeneity because of
cystic degeneration or hemorrhage 3
hemorrhage occurs in 5% of cases 3
calcification is rare

CT
CT is not as sensitive or specific for the diagnosis of schwannoma as MRI but is often
the first investigation obtained. It is particularly useful in assessing bony changes
adjacent to the tumor.

Imaging features include:

:
 low to intermediate attenuation
intense contrast enhancement
small tumors typically demonstrate homogeneous enhancement
larger tumors may show heterogeneous enhancement
adjacent bone remodeling with smooth corticated edges

MRI
Schwannomas have fairly predictable signal characteristics 7:

T1: isointense or hypointense

T1 C+ (Gd): intense enhancement
T2: heterogeneously hyperintense (Antoni type A: relatively low; Antoni type B: high)
cystic degenerative areas may be present, especially in larger tumors
T2*: larger tumors often have areas of hemosiderin

Several signs can also be useful:

split-fat sign (/articles/split-fat-sign?lang=us): the thin peripheral rim of fat best seen
on planes along the long axis of the lesion in non-fat-suppressed sequences
target sign (/articles/target-sign-disambiguation?lang=us)
peripheral high T2 signal
central low signal
rarely seen intracranially 7
fascicular sign (/articles/fascicular-sign?lang=us): multiple small ring-like structures

Treatment and prognosis

Schwannomas are slow-growing lesions. Surgery is the treatment of choice. As
schwannomas do not infiltrate the parent nerve, they can usually be separated from it.
Recurrence is unusual after complete resection. They rarely undergo malignant change.

+ Quiz questions
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 + References

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Cases and figures

(/cases/spinal-schwannoma-2)
:
 Case 1: spinal schwannoma (/cases/24163/studies/24420?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_4351629)
(/cases/neurofibromatosis-type-2-16)

Case 2: bilateral vestibular schwannomas - NF2 (/cases/25389/studies/25637?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_4895022)
(/cases/schwannoma-brachial-plexus-3)

Case 3: brachial plexus (/cases/4707/studies/6736?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_12598)
(/cases/oculomotor-nerve-schwannoma)

Case 4: oculomotor nerve (/cases/19778/studies/19820?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_2437912)
(/cases/jugular-schwannoma)
:
 Case 5: jugular foramen (/cases/5352/studies/7114?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_20439)
(/cases/glossopharyngeal-nerve-schwannoma)

Case 6: glossopharyngeal nerve schwannoma (/cases/29972/studies/30516?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_7516471)
(/cases/schwannoma-brachial-plexus-4)

Case 7: T2 (/cases/30213/studies/30844?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_7773242)
(/cases/acoustic-schwannoma-intracanalicular-5)

Case 8: vestibular schwannoma (/cases/37247/studies/39024?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_13223378)
(/cases/pelvic-schwannoma-1)
:
 Case 9: pelvic schwannoma (/cases/37399/studies/39227?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_13616833)
(/cases/posterior-tibial-nerve-schwannoma-2)

Case 10: posterior tibial nerve schwannoma (/cases/85433/studies/101066?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_54356274)
(/cases/spinal-schwannoma-9)

Case 11: spinal schwannoma (/cases/53779/studies/59860?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_30729092)
(/cases/schwannoma-of-the-neck)

Case 12: Schwannoma of the neck (/cases/25154/studies/25422?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_4796045)
(/cases/abdominal-schwannomas)
:
 Case 13: abdominal schwannomas (/cases/54368/studies/60583?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_31291779)
(/cases/vagal-schwannoma-4)

Case 14: vagus nerve (/cases/57373/studies/64313?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_34720490)
(/cases/schwannoma-of-median-nerve-1)

Case 15: schwannoma of median nerve (/cases/69926/studies/79943?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_50846336)
(/cases/cerebellopontine-angle-schwannoma)

Case 16: cerebellopontine angle schwannoma (/cases/71378/studies/81706?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_51618312)
(/cases/jugular-foramen-schwannoma-6)
:
 Case 17: jugular foramen schwannoma (/cases/46026/studies/50369?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_23532760)
(/cases/intercostal-nerve-schwannoma-1)

Case 18: intercostal schwannoma (/cases/77799/studies/90062?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_52682868)
(/cases/sciatic-nerve-schwannoma-1)

Case 19: sciatic nerve (/cases/80156/studies/93465?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_53138171)
(/cases/intraosseous-schwannoma-humerus)

Case 20: intraosseous (/cases/92656/studies/110715?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_55819960)
(/cases/chest-wall-schwannoma-2)
:
 Case 21: schwannoma (/cases/95467/studies/114744?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_56536798)
(/cases/schwannoma-of-the-orbital-cavity)

Case 22: Schwannoma of the orbital cavity (/cases/146820/studies/122359?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_58781817)
(/cases/schwannoma-hand-1)

Case 23: schwannoma - hand (/cases/172015/studies/138986?

lang=us&referrer=%2Farticles%2Fschwannoma%3Flang%3Dus%23image_list_item_62871820)

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