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 can’t 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 0/5Physiology Reflex

R L

Biceps N N

Triceps N N

APR ↓ ↓

KPR ↓ ↓

Pathology Reflex

R L

Biceps N N

Triceps N N

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 :6’00”

• BT :3’00”

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 can’t 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 tumor–nerve 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.