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DUMBBELL-SHAPED CHONDROSARCOMA

OF THE

THORACIC SPINE

A Rare Case
ABSTRACT

This is a case of a 37 year old female who presented with a 6

months history of gradual onset of lower extremity weakness, followed

by numbness of both lower extremities, which occurred 4 months later

with associated constipation. Chest x-ray revealed a right hilar mass,

which on lateral view x-ray of the thoracolumbar spine showed that it

was a paravertebral mass. MRI of the thoracic spine was also done

which revealed a dumbbell-shaped tumor, which was hypointense on

T1W images, hyperintense on T2W images, and heterogenous

enhancement on contrast study. Differential considerations for this

kind of tumor presentation are meningiomas and nerve sheath tumors.

Patient underwent laminectomy and thoracotomy and final

histopathologic examination, surprisingly, were read as well-

differentiated chondrosarcoma, a rare case of malignant spinal tumor,

which can arise de novo or from a pre-existing benign cartilaginous

tumor. Retrospective analysis of the case revealed that MRI was not

adequate to derive to the diagnosis, but also CT scan was necessary.

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INTRODUCTION

One of the commonly involved diseased organ system is the

nervous system, and is considered to be the “computer system” of our

body. It is divided mainly into two, the central nervous system and the

peripheral nervous system . The brain constitutes the central nervous

system, while the spinal cord and its nerve roots constitute the

peripheral nervous system.

Peripheral nerves serve as intricate conduction system which

serve as mediator of neural impulses traveling in both directions

between the CNS and other tissues of the body through which many

important body functions are regulated. Therefore, any lesions

involving the CNS and PNS, be it due to trauma, infection, metabolic

derangements, and tumors will always cause malfunctioning of at least

one part of the body or at most paralysis of the entire body.

Tumors of the spinal cord are much less frequent than

intracranial tumors with overall prevalence approximating one spinal

tumor for every four intracranial lesions. However, although rare, it is

an important inclusion in the differential diagnosis for any patient

experiencing myelopathy, radiculopathy, neck or back pain. These

tumors may either be intramedullary, intradural extramedullary,

extradural extramedullary or both.

For its imaging, magnetic resonance imaging is the most

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important diagnostic modality for patients with spinal cord tumors. It

provides spatial and contrast resolution of neural structures that is

unattainable by any other imaging

modality. And plain x-rays have minimal role in the modern diagnosis

of spinal cord tumors as they do not image soft tissue adequately.

However, the effects of intraspinal tumors on the vertebral elements

are sometimes evident. Computed Tomography (CT) on the otherhand,

is more useful in assessing the body structures of the spine.

This paper will present a rare case of spinal cord tumor

diagnosed pre-operatively as a benign tumor using the patient’s

presenting signs and symptoms, and by MRI. Which, however, post-

operative histopathological result revealed a more rare case of spinal

cord tumor, chondrosarcoma, and wherein complementary CT scan of

the spinal cord may have altered the MRI diagnosis.

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CASE REPORT

Clinical Presentation

This is a case of a 37 year old female who presented with a six

(6) months history of gradual onset of weakness of both lower

extremities, which she initially ignored. This was followed by numbness

of both lower extremities, which occurred four (4 ) months later. Three

(3) months prior to admission, patient started complaining of right

back pain, which the patient claimed to be aggravated by movement.

And (1) one month prior to admission, she noted feeling of heaviness

of both lower extremities while walking, more in the right, causing her

to drag her right leg. She also complained of constipation for three

months. No cough, urinary incontinence nor bladder retention were

noted. Past

medical history revealed that she was admitted last 2005 and had an

operation due to ruptured appendicitis. Family history is

unremarkable.

Pertinent physical examination on admission were as follows:

MSE : awake, alert, oriented to time place and person, ambulatory

with assist.

Cranial Nerves: intact


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Motor : R L Sensory: R

Upper 5/5 5/5 Upper 100% 100%

Lower 3/5 4/5 Lower 70%

80%

Relexes : intact

Work-ups and Diagnosis

With the above presenting signs and symptoms, the initial

working impressions were demyelinating disease and spinal cord

compression. To confirm these, initial work-ups made were chest x-ray

and x-ray of the thoracolumbar spine. Chest x-ray revealed a soft

tissue mass in the right hilar area. Fibrocalcific opacities in the left

upper lung were also noted, wherein PTB was considered. The

optimally visualized osseous structures were unremarkable. (Figure

1) .

Figure 1. PA chest x-ray

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With the x-ray findings, a demyelinating disease, which is more

of a white matter disease was readily ruled out. Soft tissue mass in the

right hilar area on PA chest x-ray could be anywhere in the

mediastinum, and the differential diagnosis will greatly vary. Such that,

if it is in the anterior mediastinum, teratomas, thymomas, and

lymphomas could be the differential considerations. However, if it is in

the posterior mediastinum, neurogenic tumor could be the major

differential consideration. Atlhough a lateral view x-ray of the chest is

of help in arriving a more specific diagnosis, a chest computed

tomography scan was suggested for further evaluation. In computed

tomography of the chest, the location of the right hilar mass could

clearly be identified as well as other lung parenchymal and bony

lesions. However, because of the presenting neurologic symptoms of

lower extremity weakness and numbness with associated constipation,

in a relatively young patient, the working impression was more inclined

to a spinal cord compression secondary to the soft tissue mass. And

for suspicions of spinal tumors, the imaging modality of choice is spinal

MRI scan with and without intravenous gadolinium. 1


These scans

adequately image the spinal cord and its associated nerves. Detail of

the bony vertebral column is not as good as CT scan, but is usually

adequate. Other radiographic studies such as CT-myelography or spine

x-rays may also be necessary depending on the type and location of

spinal tumor and its effect on the spine.

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Thoracolumbar spine x-ray was done on this patient, and the soft

tissue paravertebral mass at the level of T4 – T6 was the only

significant finding (Figure 2). The optimally visualized bony structures

were intact.

CBC, electrolytes, BUN, and creatinine were also requested to

rule out any metabolic abnormalities that could cause weakness and

numbness of the lower extremities. However, all the laboratory results

were normal.

Figure 2. AP and lateral view of the thoracolumbar spine shows a soft


tissue mass in the right hilar area at the level of T4-T6 with lucency in the central
area. No calcifications were noted. Osseous structures were unremarkable.

MRI of the thoracic spine was then done which revealed a 4.7 x 3.2 cm

right paravertebral, extramedullary, and combined intra-and extradural


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lobulated, dumbbell-shaped soft tissue mass at the level of T4-T6.

The mass is isointense with the adjacent muscle on T1WI, hyperintense

on T2WI and shows heterogenous strong enhancement on contrast

administration, with the periphery of the mass enhancing more

intensely than the center. The mass widens the right neural foramina

at the level of T4-T5 and compresses and displaces the spinal cord

anterolaterally to the left. Impression was a right paravertebral mass,

causing compression of the adjacent spinal cord.

A B

C D

Figure 3. MRI of the thoracic spine showing a dumbbell-shaped tumor at the level of
T4-T6, displacing the cord to the left. a) sagitttal contrast enhanced scan showing
heterogenous enhancement , with the periphery of the mass enhancing more
intensely than the center, b) sagittal T2W images showing hyperintense signal of
the tumor, c) axial T1W images showing hypointense signal to the spinal cord, and
d) axial contrast enhanced scan showing heterogenous enhancement.

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Figure 4. Sagittal MRI
of the thoracic spine showing the hypointense lesion in T1W images with
heterogenous enhancement on T2W images. The cord is displaced
anteriorly.

Primary considerations for this kind of tumor are meningioma, and

nerve sheath tumors. Spinal meningiomas are the most common

benign intradural spinal tumor, which arise from arachnoid cluster cells

and thus can be seen from the nerve roots. It may occur in any age

group, but mostly in the 5th and 7th decade of life with female

preponderance. Nerve sheath tumor, on the otherhand, arise from the

dorsal roots, relatively avascular and shows no calcification. Based on

the incidence of a combined intra- and extradural tumor (dumbbell-

shaped tumor), the most common are nerve sheath tumors which

comprises 15% of intraspinal masses. 2,3

Management

Hence, patient was scheduled for operation with pre-operative

diagnosis of a nerve sheath tumor. Medications were Decilone forte,

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vitamin B-complex, ranitidine and mefenamic acid for symptomatic

relief. A two-step operation, laminectomy and thoracotomy, were done

on the patient excising the right paravertebral and spinal mass.

Specimens were sent to the laboratory for histologic examination and

were labeled as follows: a) paravertebral mass, b) spinal mass, and c)

bone from spine.

Outcome

The histopathologic report surprisingly revealed not any of the

benign intra-extradural extramedullary spinal tumors mentioned

above. It showed that all the tissues submitted were secondary to a

well-differentiated chondrosarcoma, which is a malignant tumor of

chondrogenic origin that arises from chondoblasts, cells that form the

cartilage.

Her post-operative course was unremarkable, such that on her

3rd post-operative day, she was transferred from the intensive care

unit to a regular room. Intravenous antibiotics and pain relievers were

given as medications. On her 5th post-operative day, she was shifted

to oral medications. She was discharged improved on her 6 th post-

operative day, with the following physical examination findings:

Motor : R L Sensory: R

Upper 5/5 5/5 Upper 100% 100%

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Lower 3/5 3/5 Lower 100%

100%

Relexes : intact

The patient went for follow-up check-up and for physical rehabilitation

only two times during her 1st post-operative month, and afterwhich,

due to financial constraints, was lost to follow-up. On her 10th post-

operative month, patient was again seen. No complaints of numbness

of both lower extremities nor paresis were noted. Patient now can do

all kinds of household chores, except for complaints of occasional mid

back pain especially while doing her laundry.

Knowing the final histopathologic report of the excised spinal

tumor, patient was advised for follow-up CT scan of the thoracic spine

to re-assess the bony structures around the tumor site. As expected,

the CT scan revealed a bone mass which is of low attenuation,

multiloculated soft tissue mass causing bony expansion of the

transverse process of the 6th thoracic vertebra extending to the right

pedicle, as well as in the costotransverse end of the right 6 th posterior

rib (Figure 5).

Figure 5. Bone reconstruction of the axial and oblique sagittal views of the
thoracic spine show the laminectomy defect from the level of T5 to T7, with an
expansile bone lesion in the right transverse process of the 6th thoracic
vertebra, and costotransverse segment of the 6th right posterior rib.

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Correlating it with the histologic findings, this could be a residual

chondrosarcoma, or one of the expansile benign bone or cartilage

tumors such enchondromas or osteoblastomas, since according to

literatures, these tumors could give rise to chrondrosarcomas .

DISCUSSION

Sarcomas of the bone are rare and represent about 0.2% of all

new cancer cases each year. The two most common forms of bone

cancer are osteosarcoma and Ewing’s sarcoma, followed by the less

common chondrosarcoma, which constitutes 20% - 27% of all primary

malignant tumors.4,5

Chondrosarcoma is a malignant tumor of chondrogenic origin

that remains essentially cartilaginous throughout its evolution. It arises

from the chondroblasts and collagenoblasts. Therefore, it is a type of

sarcoma that is predominantly found in the area around bones. It

occurs most frequently in the pelvis and femur (50%), and less

frequent in the ribs (15%) and spine (less than 7%). 7 It usually occurs

in the 5th and 6th decade of life with a male to female ratio of 1.5-2.0:

1.6 Our case belongs to the less than 7% of all chondrosarcomas (or

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about 1.4% of the primary malignant osseous neoplasms), occurring in

the 3rd decade of life of a female, which is rarely found in literature

reviews.

Murphy et al described the patterns of chondrosarcomas as

primary or secondary. Chondrosarcomas that arise de novo are called

primary chondrosarcomas. Conversely, chondrosarcomas

superimposed on preexisting benign cartilaginous neoplasms such as

enchondroma or osteochondroma are referred to as secondary

chondrosarcomas. They are also categorized as central, peripheral, or

juxtacortical (periosteal) lesions depending on their osseous location.

Central chondrosarcomas are intramedullary in origin, although large

tumors may erode the cortex and invade the surrounding soft tissue.

Peripheral chondrosarcomas are subdivided into those secondary to a

preexisting osteochondroma or enchondroma and those developing

from the bone surface (juxtacortical). Restrospective analysis of the

patient’s case shows that the excised tumor could either be a primary

tumor of the transverse process of the 6th thoracic vertebra with soft

tissue mass extension, or a secondary tumor that arose from the

benign tumor of the transverse process and head of the 6 th posterior

rib.

Moreover, he also said that the soft tissue component of

chondrosarcomas frequently reveal typical punctate or ring-and-arc

matrix mineralization or calcification and that the non-mineralized

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component have high water content due to hyaline cartilage. These

explains the MR imaging findings of the patient, wherein the low signal

intensity of the lesion on T1 is attributed to the calcifications or

mineralized component of the tumor and its water content, while the

heterogenous signal intensity on T2 is attributed to the intense signal

of water on T2 and with the calcified component remaining low in

signal. Reviewing the osseous structures on MRI, since the follow-up

CT scan showed of an expansile bone tumor in, it was really difficult to

assess the aforementioned bone lesions since they were obscured by

the large soft tissue mass. Likewise, although the bony structures can

already be assessed on x-rays alone, especially if the lesion is on the

long bones, because of the location of the tumor wherein structures

overlap, complicated by the soft tissue mass, the presence of the

lesion was not detected. Calcifications were not present on radiograph

study, perhaps due to the tumors higher non-mineralized component.

Hence, although most likely it was already there, its presence prior to

the operation could not really be ascertained and that CT scan could

have been done to derive to a more accurate diagnosis.

The treatment for intradural-extramedullary spinal tumors is

surgical excision. The goal is total removal of the tumor with maximal

preservation of neurological function. These type of tumor are

amenable to total resection with minimal or no neurological problems

post-operatively. Same is true for chondrosarcomas , and

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chemotherapy as well as radiation have very limited role since they

generally are not sensitive to these treatments and maybe employed

for high-grade types due to high metastatic tendencies 7,8. Our case is

of low-grade type, hence surgical excision is adequate enough.

Rehabilitation or physical therapy is also necessary for the affected

area to regain strength and use after surgery.

Because of the rarity of this type of spinal tumor, only few cases

have been reported in the literatures, presenting in different histologic

types and location. Some of which, were not identified pre-operatively

but only after histological examination.

A similar case was reported by Sakayama et al (2004). 9 He

reported a case of a 58-year-old male who complained of a right

cervical pain with difficulty of swallowing without apparent cause. MRI

and CT suggested a diagnosis of a dumbbell tumor. Bone scintigraphy

was also done which was negative. Surgery was then performed

assuming the presence of a neurogenic tumor, however, postoperative

histopathological diagnosis revealed a grade II chondrosarcoma.

And in 2005 at the Haseki Eductional and Research Hospital, in

Turkey, a type of chondrosarcoma, myxoid chondrosarcoma in a 40

year old man who experienced a 3 month history of back pain was

reported. 10
MRI was done and pre-operatively, the lesion was not

identified as chondrosarcoma. However, histological examination

revealed a primary myxoid chondrosarcoma. The differential

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consideration of the intradural mass at that time was meningioma,

plasmacytoma and non-neoplastic intradural spinal cord lesion.

Another type was reported by the Christian Medical Colleges and

Hospital in India, wherein a 52-year-old man presented with symptoms

of progressive cervical radiculomyelopathy. Histologic examination

after surgery showed a mesenchymal type of chondrosarcoma.11

Another case occurring in the cervical spine, histologic type not

specified, was reported in a female by another journal.12

Only one literature was found showing the most common

location of spinal chondrosarcomas. Lloret and Server (2006) made a

retrospective review of 5 patients with histopathologically confirmed

primary spinal chondrosarcoma which showed that 3 of them was

thoracic (60%), 1 was cervical (20%) and 1 was lumbar (20%) in

distribution. 13

RECOMMENDATION

Spinal tumors can give us a lot of differential diagnoses

depending on their location (i.e. intramedullary, extramedullary,

intradural, extradural or both). And an accurate pre-operative

diagnosis is so important because the type of management and

surgical procedure will depend on it. At present, clinicians, even

radiologists, when thinking of a spinal cord tumor, magnetic resonance

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imaging is primarily requested after x-ray of the spine, since it directly

image the spinal cord and extent of soft tissue involvement. However,

as in this case, not all paravertebral soft tissue masses that cause

spinal cord compression do readily signify a pure spinal cord lesion, but

it may also arise from the osseous and cartilaginous structures (e.g.

chondrosarcoma). Hence, although MRI can already give us a line up

of differential considerations, correlation with other imaging

modalities is needed, carefully scrutinizing at least plain radiographs

for osseous abnormalities, and if there is something suspicious, then

CT scan and/or other examinations such as bone scintigraphy should

be done.

However, because of the very limited experience on

chondrosarcomas, especially that it has different histological features

with corresponding radiographic patterns, and wherein prognosis vary,

further reviews as to its radiographic, MRI and CT scan appearances is

recommended. In effect, if the radiologist becomes aware of the

imaging features of these tumors, improvement on diagnostic

accuracy, treatment, planning and prognosis is attained.

Accurate description of the imaging features of tumors should

also be given to pathologists to assist them in their histologic

diagnosis.

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And, specifically for this case, since the patient’s thoracic spine

CT scan revealed an expansile bone tumor in the spine and rib, biopsy

is recommended, and whether it is benign or malignant is yet to be

determined. This would aid proper management of the patient and at

the same time, conclude the origin of the excised tumor.

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