CASE REPORT

A. IDENTITY
Name

: An. A

Age

: 11 y.o

Sex

: Boy

RM

: 025754

Date of admittance

: 18/8/2014

Weight

: 15 kg

B. ANAMNESIS
Chief complaint: weakness at left and right lower limb
Suffered since 15 days ago, the patient cant move both of his lower limb.
Fever (-), cough (-), pain (-). History of debridement paravertebral mass on
25/8-2014
History of back pain that suffered since 1 year ago, the pain is worsen on the
night, the pain radiates to right thigh, followed by weakness on right lower
limb.
History weight loss (+)
History of long cough (-)
History of trauma (-), history of same disease before (-)

C. PHYSICAL EXAMINATION
General Status
Poor nourist/concious
Vital Sign:
Blood pressure

:110/70 mmHg

Heart frequency

: 92x/i

Breath frequency

: 20x/i;

 Temperature

: 37,1oC

Local Status
VERTEBRA REGION
Inspection

: Deformity (+), hyperkifosis (+), swelling (-), hematome (-)

Palpation

: Tenderness (-), step off (-)

Motoric Examination
Level of Spinal Cord

Muscle Strenght

C4

5/5

C5

5/5

C6

5/5

C7

5/5

C8

5/5

T1

5/5

L2

0/5

L3

0/5

L4

0/5

L5

0/5

S1

0/5

Physiology Reflex
R

Biceps

Triceps

APR

KPR

Pathology Reflex
R

Biceps

Triceps

APR

KPR

Sensoric Examination
Parasthesia as level as dermatome L1
Anasthesia as level as L2-L5, S1-S4

D. CLINICAL PICTURE

E. LABORATORY EXAMINATION
CBC
September 5th, 2013
WBC

:14,4 x 103 /uL

RBC

:4.09 x 106 /uL

HGB

:11,4 g/dL

HCT

:33,3 %

PLT

:264 x 103 /uL

MCV :63
MCH :27,5
MCHC :33,5
CT

:600

BT

:300

F. RADIOLOGY FINDING
1.

Chest AP X-Ray

2.

Thoracolumbosacral AP/Lateral X-Ray

3.

MSCT Scan Spine

4.

MRI Spine

G. HISTOPATHOLOGY FINDING
Pemeriksaan makroskopis
Diterima 17 potong jaringan berukuran antara 5x3x2 cm sampai
1,5x1x0,5 cm dan jaringan compang-camping 10 cc dalam 1 botol
jaringan padat, kenyal, warna kuning kecoklatan
Pemeriksaan mikroskopis
Sediaan jaringan diproses beberapa kali, seluruh sediaan menunjukkan
proliferasi sel-sel inti spindel kedua ujung tumpul, membentuk area yang
selular dan hiposelular dengan banyak verocay bodies diantaranya,
tampak beberapa fokus perivaskular inflamasi yang terdiri dari sel-sel
radang kronik (limfositik) serta fokus-fokus perdarahan dan 1-2
pembuluh darah dengan hyalinasasi pada dinding pembuluh darah.
Kesimpulan: Schwannoma

H. RESUME
A boy 11 years old came to hospital with chief complain weakness at left and
right lower limb that suffered since 15 days ago, the patient cant move both
of his lower limb. Fever (-), cough (-), pain (-). History of open biopsy lumbal
vertebra on 25/8-2014
On general examination: vital sign normal, localis status at regio vertebra on
inspection deformity (+), kifosis (+), on palpation: normal. Motoric power:
level L3-S1:0/5 sensoric parasthesia as level as L1 and anasthesia as level as
L2-L5, S1-S4, physiology reflex KPR and APR decrease. Pathology reflex:
Babinsky -/From laboratory examination:
CBC
WBC

:14,4 x 103 /uL

 RBC

:4.09 x 106 /uL

HGB

:11,4 g/dL

HCT

:33,3 %

PLT

:264 x 103 /uL

From radiological finding:

Plain X-Ray
Chest PA: normal
Thoracolumbosacral AP/Lateral: paravertebral mass dd/ paravertebral abses as
level as L1-L5
From histopathology examination: conclusion: scwannoma

I. DIAGNOSIS
Paraplegia due to paravertebra mass dd/ paravertebra abses

J. TREATMENT
IVFD Ringer Laktat
Analgetic
Antibiotic

DISCUSSION
NEURILEMMOMA

A. ANATOMY

Figure 1. Anatomy of Lumbal Spine1

Figure 2. Anatomy of Peripheral

Nerve1

Figure 3. Anatomy of Spinal Cord1

B. DEFINITION
Neurilemmoma is a benign tumour of the nerve sheath. It is seen in the
peripheral nerves and in the spinal nerve roots. The patient complains of pain or
paraesthesiae; sometimes there is a small palpable swelling along the course of
the nerve. (Apley)

C. ETIOLOGY
The cause of these neoplasms is unknown. Neurilemmoma can be
associated with von Recklinghausen disease; when this is the case, multiple
tumors often are present. (case studies)

D. EPIDEMIOLOGY
Schwannomas are common intradural extramedullary neoplasms that
make up 85% of nerve sheath tumors. They occur equally in men and women
with a peak age of presentation between the fourth and sixth decades. Most
appear as solitary tumors and occur equally throughout the spinal
canal.(rothman)
Neurilemmomas generally affect persons between the age of 20-50 years.
There is no racial or sex predilection. Common locations for the tumors are the
head and flexor surfaces of the upper and lower extremities, and the trunk. The
mass is usually mobile within the center of the nerve. (case studies)

E. DIAGNOSE
Schwannomas are derived from the nerve root sleeve and are more likely
to present as unilateral radiculopathy, whereas meningiomas, derived from the
dura, are more likely to present with diffuse pain or symptoms of cord
compression. (rothman)
Tenderness to palpation is often present; secondary neurologic symptoms
may occur if the tumor is large. Lesions in the sciatic nerve can mimic a

herniated-disc within the lower-back. Lesions within the tibial nerve of the foot
can mimic tarsal tunnel syndrome.
Because these tumors can present in many locations, the clinical
presentation can be varied. Some may involve the spinal nerve roots and present
with symptoms that mimic those of herniated disk disease of the spine. In the
foot and ankle, neurilemmomas can present either as an asymptomatic mass or as
localized pain and paresthesia resulting from pressure on the nerve. The 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.
In an unusual case in which resection would lead to a significant functional
deficit, these benign lesions can be observed. (CASE STUDIES)
Neurilemmomas are generally graded using the Enneking system, which
grades benign lesions ranging from 1 to 3. Grade 1 lesions are inactive, grade 2
lesions deform the surrounding tissues but are not destructive or locally
aggressive, and grade 3 lesions are locally aggressive andmay invade local
tissues but do not have a metastatic potential.Generally, neurilemmomas are
either grade 2 or 3. (case studies)
Neurilemmomas are radiolucent, therefore plain radiographs will
generally be nonspecific. The rare intraosseous lesion presents as a benign
appearing, well-circumscribed lesion. Laboratory studies are generally not
beneficial. Computer imaging, computed tomography (CT) and magnetic
resonance imaging (MRI), are useful when evaluating neurilemmomas.6 When
there is no bony destruction evident on plain radiographs, an MRI is more useful
than CT when evaluating a neurilemmoma. It is generally recommended to
performa gadoliniumcontrast, which will help differentiate the neurilemmoma
from fluid-filled cysts. On MRI, a neurilemmoma is usually round or oval with a
moderately bright signal on T1-weighted images and a bright, heterogeneous
signal on T2-weighted images. (case studies)

F.

DIFFERENSIAL DIAGNOSIS
The

differential

diagnoses

include:

fibroma,

neurofibroma,

neurosarcoma, ganglion cyst, giant cell tumor of tendon sheath, and lipoma.

G. COMPLICATION
The most common complication is initial neuropraxia; however, this
neurologic deficit can be permanent, depending on the resection of
neurilemmoma. Generally, patients tolerate resection well, with complete and
rapid relief of symptoms. Recurrence is unlikely following complete resection.
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.

H. TREATMENT
Tumor removal is dependent on tumor location and characteristics. Most
schwannomas are dorsolateral and easily accessed, but more anteriorly located or
dumbbell tumors with paraspinal extension may require extended posterolateral
exposures. Most nerve sheath tumor capsules are adherent to the arachnoid of the

nerve root, and this layer must be incised to reach the lesion. The capsule is
generally cauterized to shrink and decrease vascular input to the tumor, and then
the tumornerve root attachment can be visualized. Piecemeal tumor removal or
internal debulking with an ultrasonic aspirator should be used with large tumors.
Nerve root sacrifice may be required due to tumor inside the root of large tumors
encasing it. However, the incidence of permanent neurologic deficit from
transection of a tumor-enveloped root is lower than might be expected, usually
due to poor preoperative function of the root secondary to the tumor. Care must
be taken to carefully assess root function and weigh the benefit of complete
tumor removal against loss of function before and during surgery.
As with most benign tumors, neurilemmomas respond well to local
resection.On inspection, usually the nerve is 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.
Surgical excision is first-line therapy for extramedullary intradural
tumors, with radiation and chemotherapy being rarely indicated for benign
pathologies. Adjunctive radiation therapy and chemotherapy is typically reserved
for World Health Organization grade III and IV extramedullary tumors.
Fractionated radiation is not routinely used for extramedullary low-grade lesions,
but some authors advocate radiation for residual or recurrent large filum
terminale ependymomas. Radiosurgery, however, has recently been proposed as
an alternative to surgery for poor surgical candidates harboring low-grade
lesions. The studies are small, and follow-up is brief. Delayed radiation-induced
spinal cord toxicity is a feared complication of spinal cord radiotherapy, and
more follow-up data are necessary to determine its safety, which is of vital
importance when treating benign pathologies. (ROTHMAN)

I.

PROGNOSIS
The surgical morbidity and mortality in the removal of extramedullary
tumors is low while the neurologic prognosis is generally good. Postoperative
neurologic function is generally predicted by the preoperative neurologic status,
patient age, and duration of symptoms. In one large series of intradural
extramedullary tumors, there was a 12.3% rate of postoperative neurologic
worsening, but most deficits were transient and only 2.3% of the patients had a
permanent postoperative loss of function. Overall, intradural extramedullary
tumors have a recurrence rate of 8.9% to 32% at 5 years with the strongest
predictors of recurrence being histologic grade and previous surgical resection.