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 were read and as final well-

histopathologic

examination,

surprisingly,

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 3

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, postoperative 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 5

Motor : L Upper Lower 80% Relexes : intact

R

L

Sensory:

R

5/5 3/5

5/5 4/5

Upper Lower

100%

100% 70%

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. tissue mass in the right hilar area. Chest x-ray revealed a soft

Fibrocalcific opacities in the left The

upper lung were also noted, wherein PTB was considered. optimally visualized osseous structures were unremarkable. 1) .

(Figure

Figure 1. PA chest x-ray

6

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 8

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, nerve sheath tumors.

and

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 (dumbbellshaped 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, 10

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 above. intra-extradural extramedullary spinal tumors mentioned

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 postoperative day, with the following physical examination findings: Motor : L Upper 5/5 5/5 11 Upper 100% 100% R L Sensory: R

Lower 100% Relexes : intact

3/5

3/5

Lower

100%

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 postoperative 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 13

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 14

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 15 for chondrosarcomas , and

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. 16 The differential

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 after surgery showed a radiculomyelopathy. Histologic examination

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.
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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 17

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 diagnosis. pathologists to assist them in their histologic

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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.

REFERENCES 1. Atlas, Scott W.
Neoplastic Disease of the Spine and Spinal Cord: Magnetic Resonance Imaging of the Brain and Spine. 2nd ed. 1996. p. 1339. Rowe. ed. Volume 1. 411-412. Essentials of Skeletal

2. Yochum, Terry R. and Lindsay J.
Radiology. 2
nd

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3. Jallo, George I. Intradural Spine Tumors. Spine Universe.Com c
1999-2007. 4. Mc-Farlane-Parrott, Sally C. 2005-2007. Healthline Networks, Inc. Chondrosarcoma Health Article. C

5. Murphy, Mark D., MD and Eric A. Walker, MD et al. Imaging of
primary chondrosarcoma: radiologic-pathologic Radiographics. 2003; 23: 1245-1278. correlation.

6. Degroot, Henry III, MD. Chondrosarcoma. Bone Tumor.Org.
7. Prevedello DM and Cordeiro JG, et al. Management of primary spinal chondrosarcoma : report of two cases causing cord compression. Arq Neuropsiquiatr. 2004; 62(3B) : 875-878. 8. Mandelli C and Bernucci C, et al. Chondrosarcoma of the thoracic spine : total en bloc sagittal resection. A case report. J Neurosurg Sci, 2001; 45(2): 114-119. 9. Sakayama K and Kawatani Y, et al. Dumbbell-shaped chondrosarcoma that primarily developed in the cervical spine: a case report. J Orthop Sci 2004; 9(2) : 166-170. 10. Kotil K, Bilge T., and Olagac V. Primary intradural myxoid chondrosarcoma: a case report and review in the literature. J Neurooncol. 2005; 75(2): 169-172. 11. Intraspinal mesenchymal chondrosarcoma. Case report. Department of Neurological Sciences, Christian Medical College and Hospital, Vellore, India. 12. Dominguez CJ, Martin-Ferrer S; Rimbau J, and Joly C. Upper cervical chondrosarcoma. Neurocirugia (Astur). 2005; 16 (3): 261-265. 13. Lloret, I., Server, A. and Bjerkehagen, B. chondrosarcoma: radiologic findings with pathologic correlation. 2006 Feb; 47(1) : 77-84. 20 Primary spinal Acta Radiologica

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